SOUTHERN REGIONAL MEETING ABSTRACTS
Southern Regional Meeting Abstracts
Cardiovascular Club I
11:00 AM
Thursday, February 20, 2014
1
INCREASED RHO-KINASE ACTIVITY IN RENAL AFFERENT
ARTERIOLES OF ANGIOTENSIN II INFUSED
HYPERTENSIVE RATS
Shao W, Bivona BJ, Kobori H, Navar L. Tulane University School of Medicine, New Orleans, LA.
Purpose of Study: It is known that Ang II activates Rho-kinase activity and
Rho-kinase plays an important role in regulating afferent arteriolar tone.
However, the role of Rho-kinase on afferent arterioles in chronic Ang II infused hypertensive rats has not been examined. Experiments were performed
to determine if Rho-kinase inhibition with fasudil has greater vasodilatory
effects on afferent arterioles in Ang II infused rats.
Methods Used: Kidneys from Sprague-Dawley rats were studied in vitro using the blood-perfused juxtamedullary nephron technique.
Summary of Results: Juxtamedullary nephrons were superfused with
fasudil (1μM to 100μM) and Ang II (1 nM) in normotensive control rats
(n = 6; systolic arterial pressure, 115 ± 1 mmHg) and hypertensive rats infused with Ang II (80ng/min) for 11 to 13 days (n= 5; systolic arterial pressure, 187 ± 6 mmHg). Fasudil alone markedly dilated afferent arterioles in
both normotensive and hypertensive rats. During Ang II superfusion, fasudil
caused afferent arteriolar vasodilation in both control and Ang II infused rats;
however, the afferent arteriolar diameter response to 100 μM of fasudil in
Ang II infused rats was significantly greater than in control rats (23 ± 2 vs.
18 ± 1 μm; p < 0.05). Compared with the Ang II superfusion only period,
fasudil dilated afferent arterioles similarly in both control and Ang II infused
rats (46 ± 6% and 50 ± 12%; p < 0.05). However, the afferent arteriolar diameter responses to 100 μM of fasudil in Ang II infused rats were significantly
greater compared with those in control rats (97 ± 18 vs. 68 ± 10%; p < 0.05).
Conclusions: In conclusion, the afferent arteriolar activity of Rho-kinase in
Ang II infused hypertensive rats was enhanced compared with normotensive
rats. This augmented Rho-kinase activity may play an important role in mediating the enhanced afferent arteriolar sensitivity in chronic Ang II dependent hypertension.
2
INTERFERON ALPHA REDUCES NITRIC OXIDE
PRODUCTION IN ENDOTHELIAL CELLS
Buie JJ1, Muise-Helmericks R2, Oates J1,3. 1Medical University of South
Carolina, Charleston, SC; 2Medical University of South Carolina, Charleston, SC and; 3Medical University of South Carolina, Charleston, SC.
Purpose of Study: Systemic lupus erythematosus (SLE) patients have accelerated atherosclerosis not fully explained by conventional risk factors. Type I
interferon strongly associates with vascular endothelial dysfunction (VED)
in SLE patients. VED results from diminished expression of endothelial
nitric oxide synthase (eNOS) and nitric oxide (NO) bioavailability and serves
as the first step in the development of atherosclerosis. Herein, we examined
the impact of interferon alpha (IFN-alpha) on eNOS gene and protein expression and on NO production in primary human umbilical vein endothelial
cells (HUVECs).
Methods Used: HUVECs were treated with of IFN-alpha at 6hrs in the presence or absence of bradykinin. Cells were examined for eNOS gene expression using RT2PCR. mRNA stability was analyzed using an Actinomycin D
based assay while changes in heteronuclear RNA (hnRNA) were examined
using PCR. Protein abundance and post-translational modifications of eNOS
(peNOS Ser1177 and Thr495) were evaluated using Western blot analyses.
NO production was detected by flow cytometry using the NO sensitive dye
DAF-FM (4-Amino-5-Methylamino-2′,7′-Difluorofluorescein) Diacetate.
Summary of Results: HUVECs treated with IFN-alpha exhibited a 50% reduction in eNOS gene expression after 6hrs (p<0.005). Changes in eNOS
mRNA stability were insignificant, however, a 48% reduction in hnRNA
expression was observed (p<0.05) suggesting that IFN alpha may impair
eNOS transcription. IFN-alpha also reduced total eNOS expression and increased post-translational phosphorylation of eNOS at the threonine 495 site
1.7 fold. These changes were reversed by treatment with bradykinin. Lastly,
pre-incubation with IFN-alpha lead to a 40% reduction in NO production
(p<0.05). However, NO production was restored more than 50% with addition of bradykinin treatment.
Conclusions: These data further support a role for IFN-alpha in the development of endothelial dysfunction that may lead to atherosclerosis in SLE
populations.
3
CHEMOTHERAPIES: CATION DYSHOMEOSTASIS AND
DELAYED MYOCARDIAL REPOLARIZATION WITH
CARDIAC ARRHYTHMIAS
Khan MU, Khalid H, Weber KT. University of Tennessee Health Science
Center, Memphis, TN.
Purpose of Study: Chemotherapeutic agents, individually or in combination, can be cardiotoxic. Responsible mechanisms for their adverse effects
are poorly understood. They can alter the absorption and/or excretion of
cations which take place along epithelial cells of kidneys and intestine. We
hypothesized resultant cation dyshomeostasis would be indirectly cardiotoxic
accompanied by delayed myocardial repolarization and QTc interval prolongation on the ECG to increase the propensity for arrhythmias. We therefore
studied serum electrolyte concentrations, QTc interval duration, and the appearance of arrhythmias in patients receiving chemotherapeutic agents
reported to be associated with this pathophysiologic scenario.
Methods Used: A retrospective chart review of 30 consecutive patients
(59±2 yrs; 19 females) who were started on chemotherapy, between January
to December 2012, for various cancers. Patients with previously documented
arrhythmia, chronic renal failure (serum creatinine >2 mg/dL), or who were
receiving medications known to prolong repolarization were excluded. Lowest
total serum [Ca2+], ionized [Ca2+]o, K+ and Mg2+, obtained within first 8 wks
of initiation of chemotherapy, together with QTc interval and cardiac rhythm
from standard ECG or rhythm records obtained within 3 wks of these laboratory
values were noted.
Summary of Results: Hypocalcemia was present in 70% and ionized hypocalcemia in 67%; hypokalemia (K+ <4.0) in 90% and in 80% <3.5; hypomagnesemia (Mg2+ <2.0) in 100% and <1.8 in 87%. Prolonged QTc
(>440 ms) was found in 73% when K+ was either <4.0, Mg2+ <2.0, and/or
hypocalcemia was present. New-onset arrhythmias were seen in 67% when
QTc was prolonged: supraventricular, 17; ventricular, 2; and both ventricular and atrial in 1.
Conclusions: Chemotherapeutic agents are accompanied by a dyshomeostasis of K+, Mg2+ and Ca2+ with QTc prolongation and a predisposition to
supra- and ventricular arrhythmias. Serial surveillance and maintenance
of cations within strictly defined limits (i.e., K+ ≥4.0 and Mg2+ ≥2.0) is
recommended to avoid indirect cardiotoxicity with arrhythmias in response
to chemotherapy.
MEAN±SEM (AND NORMAL RANGE):
4
DECREASING OXIDATIVE/NITROSATIVE STRESS IN RENAL
INJURY FOLLOWING ISCHEMIA-REPERFUSION
Pacurari M1, Hage F2, Guo Y2, Ndebele K1, Kafoury R1. 1Jackson State University, Jackson, MS and 2University of Alabama at Birmingham,
Birmingham, AL.
Purpose of Study: Acute kidney injury (AKI) is a highly prevalent condition
among hospitalized patients. It is associated with high cardiovascular morbidity and mortality. A major process in AKI pathogenesis is oxidative/
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
nitrosative stress (OS/NS). Due to high AKI prevalence and limited treatment options, finding novel interventions to prevent ischemia/reperfusion
(I/R)-induced OS/NS is urgently needed. In the present study we tested the
hypothesis that systemic transfusion of EC prevents OS/NS-induced vascular
injury in I/R AKI model.
Methods Used: Male Sprague-Dawley rats were subjected to bilateral I/R injury to the kidneys by clamping the renal pedicle for 45 min under anesthesia
(ketamine and pentobarbital). Immediately after renal pedicle de-clamping,
rats were IV transfused with saline (I/R+Veh) or rat endothelial cells (0.5 ×
105 cells/500 μl, I/R + EC). After 3 day, rats were euthanized and kidneys
were collected, snap frozen in liquid nitrogen for gene expression or paraffin
embedded for immunohistochemistry. A control negative group was
subjected to sham bilateral surgery.
Summary of Results: A severe renal injury was found 3 days post-I/R determined by H&E staining. At 3days post-I/R, an increased myeloperoxidase
(MPO) immunostaining was found in the kidney cortex of I/R + Veh compared to sham, whereas a diminished MPO immunostaining was found in
I/R+EC. I/R significantly decreased vonWillebrand (vWF) immunoreactivity
in kidney cortex and renal artery compared to sham, whereas EC transfusion
minimally improved vWF immunoreactivity. Transfusion of EC robustly reduced nitrotyrosine immunostaining in kidney cortex of I/R+EC vs I/R+Veh.
In summary, I/R in rats leads to increased OS/NS which is worsening kidney
vascular/microvasculature and tubular injury. Transfusion of EC markedly
decreases I/R-induced OS/NS, thus ameliorating renal injury and therefore
may represent a new therapeutic strategy for attenuating I/R AKI. Presently,
we are exploring alternative approaches to decrease OS/NS following I/R-induced AKI that could become clinically translated.
Conclusions: I/R causes a severe OS/NS in the kidney, therefore decreasing
the production of OS/NS products following I/R may represent a novel therapeutic strategy to attenuate acute kidney injury.
Adult Clinical Case Symposium
12:00 PM
Thursday, February 20, 2014
5
HYPOTHALAMIC SARCOIDOSIS WITH VISION LOSS AND
HYPOPITUITARISM—CASE SERIES AND REVIEW OF
LITERATURE
Anthony J1, Esper GJ2, Ioachimescu A3,1. 1Emory University, Atlanta, GA;
2
Emory University, Atlanta, GA and 3Emory University, Atlanta, GA.
Case Report: Hypothalamic-pituitary (HP) neurosarcoidosis (NS) is extremely rare representing 0.5% of sarcoidosis and <1% of sellar region
lesions. The prognosis is worse compared with sarcoidosis without HP involvement. We present a case series of NS with optic chiasm involvement
and pituitary hormonal abnormalities (PHA).
Patients include four men (three African American and one white) with
both PHA and optic chiasm involvement. Mean follow-up was 6.7 years
(range 0.3-16). Mean age at diagnosis was 45.5 (range 34-59). All patients
presented with anterior hypopituitarism (FSH, LH and TSH in 4/4, ACTH
in 2/4) and diabetes insipidus (DI) in 2/4. Prolactin was normal in 4/4. All
patients developed vision loss within a year. Systemic sarcoidosis was present in 3/4 (lung, mediastinal lymph nodes, sinuses). Diagnosis was made by
histology in three and Zajicek clinical criteria in one. MRI showed a hypothalamic lesion in three cases and nodular thickening of the proximal infundibulum in one. The leptomeninges and geniculate ganglion were involved in
one case.
Treatment with high dose glucocorticoids (GC) was begun in all cases.
Vision stabilized and PHA persisted in all patients despite MRI improvement
in 2/4. Steroid sparing agents were employed in 3/4. Hyperglycemia was
common and weaning of therapy resulted in disease reactivation in 2/4
patients. Hormonal replacement with thyroid hormone and testosterone is
currently administered 4/4, GC in 2/4 (while the other two patients are still
taking high dose GC) and DDAVP in 2/4.
Review of four published series on HP NS (4, 5, 9 and 24 cases) indicates
anterior hypopituitarism as a landmark of the disease, and DI in 50% cases.
Of 42 total cases, only six showed improvement in PHA as a result of treatment. Symptomatic optic chiasm involvement occurred in 9/42 patients, but
visual outcomes were not documented.
410
Presentation with PHA and vision loss in context of a suprasellar lesion
should raise the suspicion for HP NS. Although hormone deficiencies are
likely permanent, early treatment may improve the neurological outcomes
and maintenance therapy is important to prevent relapse. Long term, multidisciplinary care is essential for patients with this serious condition.
6
ATYPICAL HEMOLYTIC UREMIC SYNDROME TREATED
WITH ECULIZUMAB
Khawar MU1, Bhutta U2, Haragsim L2. 1OUHSC, Oklahoma city, OK and
2
OUHSC, Oklahoma city, OK.
Case Report: Atypical hemolytic uremic syndrome (aHUS) is a rare, life
threatening illness characterized by widespread thrombotic microangiopathy
(TMA). We report a patient with acquired aHUS treated with Eculizumab
resulting in resolution of her renal failure without the need for dialysis.
We were consulted for oliguric acute renal failure in a 36-years-old Hispanic
female, admitted to the OB/GYN service with severe pre-eclampsia in her 31st
week of pregnancy; had undergone emergent cesarean section, complicated by
post operative bleeding from incision site requiring multiple blood transfusions.
Physical examination was significant for elevated blood pressure, tenderness
around the incision site & lower extremity edema. Pertinent laboratory abnormalities included an increase in creatinine from baseline of 0.6 to 6.8 mg/dl,
anemia, thrombocytopenia, elevated LFTs, schistocytes on peripheral blood
smear, elevated LDH level, low fibrinogen level & high fibrin degradation
products (FDPs). She was initially thought to have HELLP syndrome & conservatively managed in the hope that her condition would improve after delivery of
the baby, but her condition continued to worsen. Her ADAMTS13 activity was
61% of normal. She was diagnosed to have aHUS & started on Eculizumab for
treatment. Her renal function and platelet count started improving the very next
day. All other laboratory abnormalities trended back to normal. She did well
over the next few days and was discharged home with renal function slightly
above her baseline.
aHUS refers to the constellation of acute renal failure, thrombocytopenia,
microangiopathic hemolysis without antecedent diarrhea & is caused by
chronic uncontrolled activation of complement system. Because of its overlap with thrombotic thrombocytopenic purpura (TTP), it has been considered
a variant of TTP and treated with plasma exchange (PE) in the past. Often PE
does not reverse the renal process or stop the TMA and patients end up on
dialysis. A major achievement in the treatment of aHUS is the recent availability of Eculizumab, a humanized monoclonal anti-C5 antibody that targets
the underlying cause of TMA in aHUS. This case highlights the importance
of distinguishing aHUS from TTP and starting definitive treatment to prevent
irreversible loss of kidney function.
7
ACQUIRED METHEMOGLOBINEMIA BENZOCAIN
INDUCED METHEMOGLOBINEMIA
Zedan A, Omar S, Suarez J. Texas tech university health science center,
Lubbock, TX.
Purpose of Study: Methemoglobimemia is a rare cause of central cyanosis
that could be a life threatening condition.Normally methemoglobin level in
blood should be less than 1%. Methemoglobinemia could be acquired or
congenital, with the acquired form being more common. Acquired methemoglobinemia occurs when the rate of methemoglobin formation exceeds
the rate of its reduction. In clinical practice, Pharmacologic agents are the
most frequent cause. Of these medications Benzocaine is one of the most
commonly used medication as a topical anesthetic causing this type of hemoglobinopathy. In this abstract we will discuss a rare case of acquired drug induced Methemoglobimemia.
Methods Used: Case analysis and literature review
Summary of Results: We report a case of benzocaine-induced methemoglobinemia in a 42-year old male with past medical history of type 2 diabetes
mellitus, hypertension and drug abuse who presented to the ER with right
leg dragging and altered speech For which brain MRI was ordered and
showed acute left anterior cerebral artery infarction. During the complete
work up for stroke transesophageal echocardiography was done and 2 hours
after the procedure during which benzocain was used as a local anesthetic,
the patient developed central cyanosis and altered mental status with low oxygen saturation that was not improved with oxygenation. Arterial blood
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
gases showed PH of 7.45, PCO2 42, and PO2 82. Methemoglobin level measured by co-oxymetry was found to be 36 percent, intravenous methelyne
blue was started, 8 hours later methemoglobin level was measured again
and found to be less than 1 percent. The patient’s cyanosis resolved and his
mental status came back to norml with normal oxygen saturation.
Conclusions: Methemoglobinaemia is one of the uncommon causes of central cyanosis that could be a life threatening condition if not considered in the
differential diagnosis and work up for centrally cyanosed patients who are refractory to oxygen administration especially after administration of topical
anesthetics. Benzocain is one of those medications commonly used as a topical anesthetic in many procedures including TEE and bronchoscopy and can
cause methemoglobinemia as a rare life threatening side effect. Methylene
blue should be available in all areas where these procedures are performed
to ensure rapid and prompt management of this condition.
8
FAR MORE THAN RED EYES
Fenire M, Mulkey Z, Ali E, Mazek H, Mohamed A. Texas Tech University
Health Science Center, Lubbock, Lubbock, TX.
Purpose of Study: 1-To report a case of relapsing polychondritis (RP) with
images. 2- Highlight that misdiagnosis is common and can lead to serious
consequences.
Methods Used: Case analysis and literature review
Summary of Results: A 45-year-old man presented to the emergency center
after one week of redness of his left eye and ear. He was diagnosed with possible cellulitis and discharged with antibiotics but returned three days later
after his symptoms worsened and involved both eyes (Panel A and B). Of
note, one year prior, similar symptoms resulted in him losing his hearing
on the left side. Ophthalmological exam revealed iritis, acute diffuse scleritis
and proptosis bilaterally. His cardiopulmonary exam was normal as was the
electrocardiogram. A diagnosis of relapsing polychondritis was made and a
course of high-dose corticosteroids was initiated to which he responded very
well. The patient declined maintenance immunosuppressive treatment,
electing to treat flare ups only and has remained symptom free for 8 months.
Conclusions: RP is rare. More than 600 cases have been reported worldwide. It is a multisystem disease that can be life threating, debilitating, and
difficult to diagnose. In a series of 66 patients, the mean delay from time
of medical attention was sought for symptom onset until diagnosis was
2.9 years, one third of those patients needed to see five or more physicians
before diagnosis. The etiology is unknown, but the pathogenesis seems to be
an immunologic reaction to type II collagen. The clinical features are the key
in diagnosis of the RP. The onset is usually sudden. In the Isaak et al series auricular chondritis was the initial presenting symptom in 39% of the patients.
Permanent hearing loss can occur as a consequence of sensorineural involvement believed to be secondary to vasculitis in the vestibular or cochlear
branch of the internal auditory artery. Ocular manifestations occur in 60%
of patients, the most common are scleritis, episcleritis, and conjunctivitis.
Prednisone had been the main form of treatment, and methotrexate may help
lessen corticosteroid requirement.
9
ELECTROCARDIOGRAM CHANGES IN COMMON
VARIABLE IMMUNODEFICIENCY RELATED ADRENAL
INSUFFICIENCY
Parekh N1, Modi F2, Patel B1, Byrd RP2,1, Roy T2,1. 1ETSU, Johnson City,
TN and 2ETSU, Johnson City, TN.
Purpose of Study: We present an adult patient with known history of common variable immunodeficiency (CVID) who was identified to have adrenal
insufficiency-associated QTc interval prolongation and t-wave inversion.
Treatment with hydrocortisone resolved these endocrinopathy-associated
myocardial electrical abnormalities.
Methods Used: A 70 year old male with past medical history of CVID was
admitted for hypotension refractory to fluid challenge. He reported fatigue,
loss of appetite and unintentional weight loss 40pounds in last year. His
blood pressure was 82/42 mmHg. His hemoglobin was 11.9 g/dl and had
an eosinophilia of 13.9%. He was hyponatremic with normokalemia. The
electrocardiogram (ECG) showed a QTc prolongation of 600ms with nonspecific anterolateral T wave changes. A thorough history ruled out any prior
use of glucocorticoids or other medications which could be the cause of
Southern Regional Meeting Abstracts
adrenal insufficiency or QT interval prolongation prior to this hospitalization.
Left heart catheterization ruled out acute ischemic process.In the1950s, ECG
abnormalities in adrenal insufficiency were described. Studies of rat heart
muscle show that glucocorticoid is essential for the calcium transport in sarcoplasmic reticulum and microsomal phosphorylase activity. Additional
reports suggest adrenal insufficiency-related reversible cardiomyopathy and
T wave changes in adult patient. The link between adrenal insufficiency
and CVID was suggested when a literature review identified a pediatric case
of autoimmune lymphocytic hypophysitis in association with CVID who
presented in acute adrenal crisis.
Summary of Results: Adrenal insufficiency was confirmed by very low
morning cortisol levels -1.3 ug/dl. Improvement in patient’s symptoms, blood
pressure and EKG changes resolved in parallel with hydrocortisone supplementation. He was later diagnosed with low serum ACTH and a partially
empty sella on magnetic imaging studies supporting secondary adrenal
insufficiency.
Conclusions: To our knowledge, this is the first case report of an adult where
CVID, adrenal insufficiency and QTc abnormality were coexistent. Consideration of adrenal insufficiency early in hospitalization lead to prompt diagnosis and treatment which prevented potential catastrophic consequences of
ventricular arrhythmia or death.
10
FAMILIAL THORACIC AORTIC ANEURYSM AND
DISSECTION PRESENTING WITH FLASH PULMONARY
EDEMA IN A 26-YEAR-OLD MAN
Omar S, Moore T, Mulkey Z, Nugent K. Texas Tech University Health Science center, Lubbock, TX.
Purpose of Study: Familial thoracic aortic aneurysm (TAAD) is an autosomal dominant disease that usually involves the ascending aorta and presents
with dissection of the involved segments. The genetic basis of familial
TAAD, not associated with other genetic syndromes, has only been recently
described. Several associated loci with relevantgenes mutations have been
identified. This disease typically presents at a mean age of 56, which is ten
years younger than non-familial cases.
Methods Used: Case analysis and literature review
Summary of Results: We are reporting one case of familial thoracic aortic
aneurysm with a dissection in a 26-year old male with no significant past
medical history and a family history of dissecting aortic aneurysm in his
mother at age 40. The patient has normal physical appearance and does not
have any features that suggest Marfan’s syndrome, Ehlers-Danlos syndrome,
Loeys-Dietz syndrome, or ANCA-positive vasculitis. He presented with
cough, shortness of breath, and chest pain for 10 days. Chest x-ray at the time
of presentation showed bilateral pulmonary infiltrates. He was treated outside
the hospital as bronchopneumonia but did not improve. When CT scan of the
chest showed dissecting ascending thoracic aortic aneurysm, the patient was
transferred to our hospital and successfully underwent aortic aneurysm repair. Three months after aortic dissection repair, the patient returned to our
hospital with new complaints of sharp back pain. CT angiography showed
a new aortic aneurysm and dissection extending from the carotid arteries
through the bifurcation and into the right iliac artery. The patient underwent
aortic, carotid, and iliac repair
Conclusions: This case report focuses on a patient with familial TAAD and
discusses the associated genetic loci and available screening methods.
Screening begins with CT angiogram, MRI,or TEE. Genetic screening
includestesting for the ACTA2 gene mutation and may involve sequencing
of the seven other genes associated with familial TAAD. It is important to
recognize potential cases of familial TAAD and understand the available
screening methods because early diagnosis allows appropriate management
of risk factors and treatment when necessary
11
CHANGES IN LIPIDS AND BLOOD SUGAR POST BARIATRIC
SURGERY IN OBESE HISPANIC PATIENTS- POSSIBLE
MECHANISMS
Hernández-Gil de Lamadrid JJ1, Altieri PI1,2, Mora-Lemus L1, Corretjer L1,
Nieves JJ1, Banchs HL1,2, Suárez A1, Muñiz J1, Soto M1, Escobales N1,
Crespo M1. 1University of Puerto Rico, San Juan and 2Cardiovascular Center of Puerto Rico and the Caribbean, San Juan.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
411
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: Bariatric surgery is the most frequent used procedure to
control obesity (o) and the metabolic syndrome (Mets). The purpose is to describe the changes after this procedure and possible mechanisms.
Methods Used: A retrospective analysis of 102 patients records who had
bariatric surgery analysis was done preoperative and 12 months post-surgery.
Summary of Results: 97% had Roux-N-Y surgery with a mean age of
39 years. 79.4 were females and 20.6 were males. 44% were diabetics.
Metabolic Reductions:
1) BMI (Kg/m2) - 47 ± 9–33 ± 7) P < 0.0001
2) Weight (Lbs.)- 285 ± 62–198 ± 54) P < 0.0001.
3) Cholesterol (mg/dl) - 169 ± 30–151 ± 30 P < 0.0001.
4) LDL (mg/dl)- 101 ± 27–86 ± 25 P. < 0.0001.
5) Triglycerides (mg/dl)- 112 ± 47–84 ± 22 P < 0.0001.
6) FBS (mg/dl) - 98 ± 25–87 ± 20 P < 0.0001.
Metabolic Increase:
1) *HDL mg/dl) - 44 ± 10 to 49 ± 13 P < 0.0006. *Not significant
The correlation factor between weight loss and changes in FBS, Cholesterol, HDL and LDL fluctuated between .1 - .5.
Conclusions: These results shows that the metabolic changes and lipids, predominantly are due to weight reduction, but the poor correlation factor suggest other mechanisms:
1. Up regulation of Glu) -1 protein producing and increase in reabsorption
of glucose from Jejunum.
2. Inflammation of the Jejunal mucosa producing adaptation (atrophyproliferation) reducing the absorption of cholesterol (diabetics >
non-diabetics).
3. More degradation of LDL in Jejunum (more in diabetics).
4. No degradation of HDL.
In summary the changes in blood sugar and lipids are complex and
should be studied further as well as changes in gut microbiota. These
changes are related to a further reduction in cardiovascular risks, especially
in the Hispanic population who has a lower effect of risk factors in the development of ischemic heart disease.
12
AN INTERESTING CASE OF HYPOKALEMIC PERIODIC
PARALYSIS
Sotello D1, Rivas M1, Castaneda J2, Lado-Abeal J1. 1Texas Tech University
Health Sciences Center, Lubbock, TX and 2AtlantiCare Regional Medical
Center, Atlantic City, NJ.
Case Report: A 31 year old Asian male without past medical history presented
to the emergency for 1 day of multiple episodes of severe bilateral thigh pain,
lasting 1-2 seconds each, occurring every time he tried to move the proximal
lower extremity muscles, associated with 3 episodes of sudden lower extremity
muscular weakness, the most significant one lasting >30 minutes. He had a
similar episode 8 months before. Vital signs: BP: 132/84 mmHg, HR: 116 beats
per minute, RR: 18 per minute and Temperature: 99.7° F. Physical examination:
tachycardic, no murmurs, diminished lower extremity strength 2/4, with diffuse
tenderness, and brisk patellar reflexes, the rest was normal. CBC, creatinine and
liver enzymes were within normal limits; Na: 144 mEq/L, K: 1.7 mEq/L, Glucose: 140 mg/dl, Ca: 10.2 mg/dl, Magnesium: 1.9 mg/dl, Phosphorus 2.1 mg/dl,
TSH: <0.01 ng/ml, free T3: 17.65 pg/ml, free T4: 5.01 ng/dl, diagnosing
thyrotoxic periodic paralysis (TPP). Thyroid Stimulating and Thyrotropin binding inhibiting immunoglobulins, antithyroid peroxidase and antithyroglobulin
antibodies were positive, suggesting Graves’ disease. The patient was started
on IV potassium replacement, as well was treated with propylthiouracil, propranolol, iodine and dexamethasone, which resolved patient’s symptoms.
Hypokalemic periodic paralysis (HPP) is an heterogeneous group characterized clinically by hypokalemia and acute systemic weakness due to multiple
mechanisms. The most common cause of HPP is an autosomal dominant defect
in the Cav1.1 skeletal muscle voltage-gated Ca channel or the Nav1.4 Na channel which occurs more commonly in non-hispanic Caucasians. Nonfamilial
causes of HPP includes TPP which is more common in Asian population and
in males. The pathogenesis of TPP has long been thought related to increased
Na-K ATPase activity stimulated by thyroid hormone and/or hyperadrenergic
activity and hyperinsulinemia. Recent findings show that loss of function
mutations of the skeletal muscle-specific inward rectifying K (Kir) channel,
Kir2.6, which cause an inadequate outward K efflux in skeletal muscle can lead
to a vicious cycle of hypokalemia and paralysis. Treatment is with potassium replacement as well as restoration of euthyroidism.
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13
MYSTERIOUS ORIGINS OF ANDROGEN EXCESS
Cosentino G, Friday K. LSUHSC-NO, New Orleans, LA.
Case Report: Androgen secreting tumors are notoriously hard to find when
they are secreting small amounts of hormones. However, when testosterone
secretion is ample enough to cause virilization, a neoplasm is usually evident
on imaging. This case highlights the use of a laboratory evaluation for localization of a virilizing, androgen-secreting tumor.
This is the case of a 37 year old woman with a past medical history of
lupus and obesity, status-post gastric bypass, who was referred to the endocrinology clinic with complaints of increasing facial hair and male pattern
baldness for one year. She had been treated by another physician for nonclassic congenital adrenal hyperplasia with steroids, but she had failed to improve. Other complaints included deepening of her voice and acne. Her
medications included dexamethasone 0.5 mg twice daily and spironolactone
200 mg daily. Blood pressure was 116/78 and BMI was 38.4. Physical exam
revealed male pattern hair distribution with terminal hair on cheeks and sideburns, neck, chest, and back. She had modest clitoral enlargement. Previous
labs included a total testosterone of 1271 ng/dL and free testosterone of 301
(upper limits of normal for women are 45 and 6.4 respectively), a low
DHEA-S of 22, hematocrit of 48.5%, estradiol 46, suppressed LH and
FSH. Pelvic ultrasound, CT, and MRI failed to identify any definitive abnormalities of the adrenal glands nor of the ovaries. The patient was also sent for
adrenal and ovarian vein sampling which did not identify the source of excess testosterone. The patient did not desire children, and she was so burdened by her symptoms that her gynecologist agreed to do a hysterectomy
and oophorectomy. Although in the surgery there were no palpable abnormalities of the ovaries, pathology revealed a 2.5 cm Leydig tumor of the left
ovary. Blood work done following the surgery confirmed that the patient’s
testosterone levels had decreased to 3 ng/dL.
Neoplasm must be suspected when patients present with virilization that
occurs over a short time. When the adrenal glands are the source of androgen
excess, it is accompanied by elevated levels of DHEA-S. In our patient these
levels were low. Often when serially imaged, these tumors are seen as the
hormone levels rise. However, when the source remains elusive, hormone
precursors can be used to identify the likely organ of secretion.
14
DIABETIC MUSCLE INFARCTION OR PYOMYOSITIS?
Pena C1, Argueta E1, Nantsupawat T1,2, Nugent K1,3. 1Texas Tech University,
Lubbock, TX; 2Texas Tech University, Lubbock, TX and 3Texas Tech University, Lubbock, TX.
Case Report Introduction: DMI is a rare complication of poorly controlled
diabetes mellitus (DM) that occurs during spontaneous skeletal muscle necrosis. Muscle swelling and tenderness characterize it. The differential diagnosis
includes PM, in which a muscle abscess develops. PM can present in three
stages; muscle swelling and tenderness, then abscess formation, and finally sepsis. PM diagnosed in the first stage is challenging and can be delayed. We present a patient with PM, whose presentation suggested DMI with concurrent
diabetic ketoacidosis (DKA), but developed PM in six weeks.
Case: 44-year-old woman with DM type II presented with left lower back pain
of two weeks duration. She had tachycardia, no fever, left paraspinal swelling with
no erythema or ecchymosis. Blood analysis revealed leucocytosis, 20.4 cells/mm3
(84% neutrophils), glucose 261 mg/dL, HbA1C 11.7%, normal CK, ESR 70 mm/hr,
increased anion gap metabolic acidosis with acetone in serum. Abdomen CT
imaging revealed isodense left paraspinal muscle enlargement. Ultrasound
was negative for fluid or cystic collections. A non-contrast MRI showed paraspinal
muscle T2 hyperintensity consistent with muscle infarction. She was discharged
three days after DKA management with mild back pain. The patient presented
six weeks later with increased back pain. A repeat CT showed left paraspinal muscle multiloculated fluid collections. An abscess was confirmed after aspiration,
and culture was positive for oxacillin resistant Staphylococcus aureus.
Discussion: PM diagnosis depends on clinical infromation; the MRI is considered a sensitive method to demarcate inflamed muscle during infection. In early
stages, the muscle may show preserved or increased signal intensity on T1
weighted images and abnormally high intensity in T2 weighted images. These
findings have also been associated with DMI. Both conditions, DMI and PM,
may have elevated ESR and CK levels, and usually affect the thigh muscles.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Conclusions: This case represents an early PM presentation without clear
signs of infection. A confounding factor was the concurrent DKA with
leucocytosis attributed to it, and absence of fever. Muscle pain and swelling
in patients with poorly controlled DM needs close follow up.
15
WHEN YOU CAN NOT SEE, EAT, AND SPEAK, YOU COULD
HAVE MILLER FISHER SYNDROME!
Bahaa Aldeen M, Talibmamury NB, Nadhem O, Al-alusi S, Smalligan RD.
Texas Tech University, Amarillo, TX.
Case Report: A 56yo male presented with dysphagia, diplopia, dysarthria,
saliva dribbling and facial droop that had worsened over 3 days. A week before he was admitted for gastroenteritis. He denied headache, fever, extremity
weakness or sensory deficits and he denied fluctuations of symptoms during
the day. He had no chemical exposure or sick contacts. PMH: neg except the
recent admission; P/S: smokes, no ETOH or drugs; PE: BP 121/85, HR 97,
RR 22, T 98.5, O2 sats 98% on RA. Heart, lungs, abd, extrem, skin normal;
neuro: A&O with bilateral facial palsy, no nystagmus, weak abduction of left
eye, absent gag reflex, weak tongue. Motor and sensory exams of extremities
were normal and reflexes were decreased but present, gait nl. Labs: WBC
19k, electrolytes nl except AG 16, Cr 0.9, gluc 103, Ca 10, Alb 4.4, TP
7.5, LFTs and UA nl. LP nl with neg cmv/enterovirus. Botulism neg. Negative actin (smooth muscle) AB; CSF electrophoresis showed increased beta
globulin; AChR blocking ABs, Asialo-GM1, GM1, GM2 and GD1b all
neg, GD1a and GQ1b Abs positive. Images: CXR nl, CT head: nl except
mild encephalomalacia due to remote infarction. Myasthenia tests were neg
(Curtain sign and edrophonium). EMG was normal.
Discussion: Acute, progressive weakness is frightening to patients and if ascending pattern is followed it is usually Guillain-Barre Syndrome (GBS).
Our patient’s weakness began in the face with the limbs preserved raising
suspicion of Miller Fisher Syndrome (MFS). MFS is an acute immune-mediated demyelinating polyneuropathy, variant of GBS, which presents as a paralyzing illness provoked by a preceding infection. Signs and symptoms include
ophthalmoplegia with ataxia and areflexia. 25% of MFS pts have extremity
weakness but incomplete forms ophthalmoplegia without ataxia, and ataxic neuropathy without ophthalmoplegia. A brainstem stroke or Wernicke’s can
mimic MFS except symptoms are more abrupt with stroke and Wernicke’s
usually has nystagmus. Although Miller Fisher syndrome typically presents
with ophthalmoplegia, ataxia, and areflexia, variant forms exist as in our
case. 80+% of patients with MFS have a positive anti-GQ1b antibody in
their CSF. Physicians should keep MFS in their differential of any patient
with unexplained facial palsy or bulbar weakness.
16
DIABETES INSIPIDUS UNMASKED BY NAUSEA AND
VOMITING OF PREGNANCY
Orellana-Barrios MA, Rivas M, Sotello D, Lado-Abeal J. Texas Tech University Health Science Center, Lubbock, TX.
Case Report: A 33-year-old pregnant woman, G2P1, in her eleventh week
of pregnancy was admitted for worsening nausea and vomiting of pregnancy.
Her symptoms started at 5 weeks gestation and were associated with polyuria
and polydipsia. She had a history of migraine headaches diagnosed 5 months
prior to presentation, resolved gestational DM in her last pregnancy, and a
positive family history for Graves’disease in her mother and aunt. On admission, her vital signs were within normal limits, and her physical exam was
remarkable for dehydration. Her serum sodium was 137 mmol/L, potassium
3.4 mmol/L, calcium 10.4 mg/dL, BUN 2 mg/dL, glucose 94 mg/dL, and
urine specific gravity 1.005. She was started on intravenous (IV) fluids
(D5WLR, 150 ml/h) and IV thiamine, esomeprazole and antiemetics. Her
urinary output in the first 12 hours after admission was 3750 ml. Her
symptoms improved but her serum sodium increased to 148 mmol/L ; further
testing at that time revealed a serum osmolality of 300 mOsm, urinary osmolality of 103 mOsm, and urinary sodium of 40 mmol/L. Nasal desmopressin
(dDAVP) was started at a dose of 10 mcg twice daily and IV fluids were
changed to 1/2NS (45ml/h). The patient’s symptoms improved over 3 days
with normalization of her urine output and serum sodium. Further work up
revealed a low TSH and normal TSI, anti-TPO, anti-Thyroglobulin, SS-A
and SS-b antibodies. Her head MRI was normal.
Southern Regional Meeting Abstracts
During pregnancy the metabolic clearance of ADH increases due to placental derived vassopressinase. In most pregnant women, this augmented
clearance is compensated by an increase in vasopressin production. Insufficient vasopressin secretion or reserve can be unmasked by pregnancy, and inadequate oral intake secondary to NVP can exacerbate this clinical scenario.
This case illustrates how this mechanism can be present even in very early
pregnancy (1 month after the estimated date of conception) and how prompt
diagnosis is imperative to avoid the reported maternal and fetal complications of hypernatremia, profound free water loss, and sometime inadvertent sudden changes in serum osmolality.
17
HOARSENESS, DYSPHAGIA - SUBTLE MANIFESTATIONS OF
VERY RARE AND UNUSUAL TUMOR
Suvorava N1, Hosiriluck N1, Aulakh A1,2, Konala V1,2, Nguyen D1,2,
Warraich I3. 1TTUHSC, Lubbock, TX; 2TTUHSC, Lubbock, TX and
3
TTUHSC, Lubbock, TX.
Case Report: Introduction: Alveolar Soft- Part Sarcoma (ASPS) is a rare
malignant tumor which accounts to less than 1% of soft tissue sarcomas
and 0.1% of all head and neck sarcomas. It has an indolent clinical course
and a high metastatic potential with early metastases to lungs, bone and
brain. Late metastases can occur years after resection of the primary tumor,
even without local recurrence of the disease.
Case Presentation: A 58 year old male heavy smoker presented with progressive hoarseness, dysphagia and significant weight loss for over a year.
The patient underwent flexible laryngoscopy, which revealed a pedunculated
obstructing mass at the left supraglottic region. CT scan of neck revealed a
1.3cm soft tissue mass in the glottis. PET scan revealed a hyper-metabolic focus in the left posterolateral aspect superior to the vocal cords. Patient underwent resection of the left supraglottic mass and left neck radical lymph node
dissection. Pathology reported poorly differentiated alveolar soft part sarcoma with lymph node metastases and perivascular invasion.
Patient was staged as IVA and completed 35 post operative radiation
treatments. He was started on adjuvant treatment with Sunitinib. He developed a skin reaction to the drug that required dose adjustment, but he tolerated it well afterwards. Five months after starting treatment, PET scan
showed increased hypermetabolic activity in left vocal cord region and suspicious left cervical lymph node on physical exam. Subsequent work up
was negative for any recurrence. Patient later developed worsening fatigue
and generalized weakness, and further treatment with Sunitinb was discontinued. He had a disease free survival of nine months with Sunitinib.
Discussion: Treatment of choice for ASPS is wide surgical resection with
lymph node dissection for clinically suspicious lymph node involvement. Radiation therapy can be used in high risk patients to prevent local recurrence.
No standard chemotherapy regimen is effective in ASPS. However, newer studies show promising results using targeted therapies such as Sunitinib and
Sorafenib (multi-tyrosine kinase inhibitors), Cediranib and Bevacizumab.
18
ORGANIZING PNEUMONIA ASSOCIATED WITH HERPES
SIMPLEX VIRUS INFECTION PROGRESSING TO ACUTE
RESPIRATORY DISTRESS SYNDROME IN AN
IMMUNOCMPETENT PATIENT
El-Bakush A, Kambali S, Nugent K. Texas Tech University Health Sciences
Center, Lubbock, TX.
Case Report: Introduction: Organizing pneumonia (OP) an uncommon
diffuse interstitial lung disease that affects the terminal and respiratory
bronchioles, alveolar ducts, and alveoli. A definitive diagnosis requires
adequate tissue biopsy showing the characteristic intra-alveolar buds of
granulation tissue associated with fibroblasts, myofibroblasts, and loose
connective tissue. Most cases are idiopathic; some are associated with
infections, drugs, radiation, connective tissue disorders, transplantation,
and environment agents. We present an uncommon case of OP associated
with Herpes simplex virus (HSV).
Case: 40-year-old man with hypertension presented with dyspnea, fever and
productive cough for 2 weeks. He was treated 5 days for acute bronchitis as
an outpatient with no improvement. Exam revealed mild distress with an oxygen saturation of 92% on room air and right sided crackles. Labs included a
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
413
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
WBC of 19.3. CXR showed bilateral infiltrates greater on the right. He was
diagnosed with community-acquired pneumonia and started on broad spectrum antibiotics. Sputum cultures were negative. His respiratory status progressively deteriorated, and he was intubated for mechanical ventilation.
Bronchoalveolar lavage was positive for HSV; transbronchial biopsies showed
focal organizing pneumonitis with plentiful intra-alveolar macrophages. He received 10 days of IV antibiotics and 14 days of IV acyclovir. After being
discharged home, he was readmitted10 days later with worsening symptoms
and infiltrates on the CXR. He was intubated for respiratory failure. CT chest
showed diffuse, patchy consolidation of both lungs, right more than left. Open
lung biopsy showed extensive organizing pneumonia, diffuse alveolar damage,
intra-alveolar macrophages, and pleural fibrosis. The diagnosis of OP was
made, and he was treated with IV steroids. He was extubated after 10 days;
within 2 weeks the CXR was markedly improved.
Discussion: Organizing pneumonia is usually idiopathic; infection is one of
the secondary causes. To our knowledge only one case associated with HSV
has been reported, and it was in a transplant patient on tacrolimus. Early diagnosis and treatment is crucial as it usually responds to steroid treatment.
19
WELLENS’ SIGN - A RELIABLE ECG SIGN OF CORONARY
(WIDOW MAKER) ARTERY DISEASE FOR THE INTERNIST
Panikkath R, Panikkath D, Meyyerose G, Jenkins L. Texas Tech University
Health Sciences Center, Lubbock, Lubbock, TX.
Case Report: Introduction: Although Wellens’ sign is reliable sign of significant proximal left anterior descending artery (LAD) disease, awareness
of this among primary care doctors, hospitalists and emergency room
physicians is very low.
Methods: This is a case report and review of literature
Case Report: A 56 year old man with history of coronary stenting and coronary artery bypass surgery (CABG) in 2011, presented to his primary care
physician (PCP) with short episodes of chest pain. The cardiac biomarkers
were negative. Although the PCP obtained an ECG, which showed T
inversions in leads V2 and V3, suggestive of Wellens’ sign, it was
disregarded due to lack of awareness regarding this sign. No urgent referral
to a cardiologist occurred. Six months later, when the patient followed up
with a cardiologist, myocardial perfusion study showed a large amount of anterior ischemia. Echocardiogram showed normal left ventricular function and
no wall motion abnormalities. A coronary angiogram was performed, which
showed near total occlusion of the stent in the proximal left anterior
descending coronary artery. However, since the CABG graft to the mid
LAD was patent, no intervention was performed.
Conclusion: Wellens’ sign is a reliable sign of proximal LAD disease with a
sensitivity of 69%, specificity of 89% for significant (≥ 70% diameter) stenosis of the LAD with positive predictive value 86%. The objective of this abstract is to create better awareness among primary providers about this sign.
It has been reported in 14%-18% of patients with unstable angina. This finding is usually signifies a pre-infarction stage of coronary artery disease. Its
progression to anterior wall myocardial infarction is so likely that medical
management alone is not considered enough to stop the natural course.The
evolution to anterior wall MI is often rapid with a mean time of 8.5 days from
onset of this sign to infarction. This sign often appears when the patient is
pain free, signifying the importance of a follow up ECG in such patients. Infarction due to occlusion of the proximal LAD places the patient at risk for
left ventricular dysfunction, heart failure, and death. Awareness of this sign
among physicians helps in early detection of proximal LAD lesions.
Pediatric Clinical Case Symposium
12:00 PM
Thursday, February 20, 2014
20
THE BENZOCAINE BLUES; A CASE OF SEVERE
METHEMOGLOBINEMIA INDUCED BY TOPICAL 20%
BENZOCAINE IN A PEDIATRIC PATIENT
Broge T, Patel S, Neemuchwala F, Gorman S, Haggett L. Florida State University Pediatric Residency Program at Sacred Heart Hospital, Pensacola, FL.
414
Case Report: A three year-old male was admitted to the pediatric intensive
care unit for altered mental status and hypoglycemia. Initial work-up was
unremarkable. He was placed on intravenous fluids with dextrose and
cardio-respiratory monitoring. He responded to therapy and exhibited gradual
improvement of his mental status. However, on hospital day three, he experienced acute onset of cyanosis and altered mental status. He was
tachycardic with a heart rate of 230 beats per minute and saturation by pulse
oximetry of 86%. He was placed on 100% oxygen, with subsequent improvement of oxygen saturation to only 90%. A venous blood gas was obtained and yielded a pH of 7.35, pCO2 of 38.6mmHg, HCO3 of 21.2 mEq/l,
hemoglobin of 7.6g/dL, lactate of 5.2 mmol/L and methemoglobin concentration was critically elevated at 66.3%. The blood sample was noted to be
chocolate-colored. Methylene blue was administered at 1.85 mg/kg/dose.
Subsequent blood gases showed a methemoglobin concentration of 16%
and then 1.4%. A bottle of 20% benzocaine oral gel was found on the floor
in patient’s room, which was originally in purse of the patient’s mother. The
patient’s acute presentation was attributed to the ingestion of this topical
over-the-counter agent.
Methemoglobin (MetHb) is a nonfunctional state of hemoglobin in
which iron atoms from hemoglobin are oxidized from their ferrous form to
a ferric state, rendering the heme incapable of carrying oxygen. MetHb is
normally present in small amounts in the blood; however, at increased
concentrations methemoglobinemia can be life threatening. All the commonly used benzocaine preparations have been reported to cause acquired
methemoglobinemia including over-the-counter teething pain products. Although perceived to be relatively benign agents, topical benzocaine preparations have demonstrated potential to cause life-threatening methemoglobinemia.
Primary care providers must be aware of the side effects of benzocaine products
and ensure that parents are educated regarding the proper dosing and safe storage
of topical benzocaine preparations.
21
A PRESENTATION OF KAWASAKI DISEASE SHOCK
SYNDROME
Strachan S, Williard C, Wall T, Borasino S. The University of Alabama at
Birmingham, Birmingham, AL.
Case Report: A 5 year old female presents in shock and respiratory failure
after 6 days of fever, malaise, headache, rash, conjunctivitis and cervical
lymphadenopathy. She was seen by her pediatrician who prescribed
Amoxicillin and then an oral cephalosporin which had to be held for a
rash reaction. In 48 hours she developed painful neck swelling and was
admitted to a regional hospital. There she developed tachycardia and an
increased work of breathing requiring transfer to a PICU. On arrival: T
38.9°C, HR 124 bpm, BP 66/38 (after a 40cc/kg normal saline bolus),
and SpO2 92% (on 5L facemask). PE: bilateral erythematous conjunctiva
with subconjunctival hemorrhages, a 4 x 4 cm firm right cervical lymph
node, coarse breath sounds, a I/VI systolic murmur, delayed cap refill,
and an erythematous maculopapular rash on her legs. She was started on
antibiotics, required inotropic support, and was rapidly intubated for respiratory failure. Kawasaki Disease (KD) was suspected and IVIG was administered. After much improvement she was transferred to a general
inpatient ward service. However, she remained febrile to 38.8 - 39.4°C.
Physical exam showed injected conjunctiva, a diffuse erythematous
maculopapular rash, and edema of her hands and feet with desquamation
of her palms. Blood cultures finalized negative, and antibiotics were
discontinued after 7 days. High dose methylprednisolone and a one-time
dose of Infliximab were given which finally led to defervescence. KD is
the leading cause of acquired heart disease among children in North
America and Japan, and although the etiology is not well understood, recognition and treatment have been well defined. KDSS was first described
by Kanegaye et al. in 2009 as signs of KD with hypotension (≥ 20% decrease from baseline). Recently there have been several case reports of
KDSS; however, overall it has proven to be an uncommon presentation.
It has been associated with higher markers of inflammation, low grade
consumptive coagulopathy, and higher rates of coronary artery dilatation
and aneurysm formation than KD without shock. Most cases showed improvement in cardiac function after IVIG; however, some remained refractory. Although the cause of this severe presentation of KD is unknown,
clinicians need to be alerted to the possible increase in prevalence of this
severe complication.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
22
24
GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS
ORBITAL PSEUDOTUMOR IN A PEDIATRIC PATIENT: A
CASE DEMONSTRATING THE NEED FOR CONTINUED
SUSPICION OF ANCA-ASSOCIATED VASCULITIS IN CASES
OF ISOLATED ORBITAL PSEUDOTUMOR
HSV ENCEPHALITIS IN A PATIENT AFTER RADIOTHERAPY
AND ADJUVANT CHEMOTHERAPY
Carlson AP1,2, Santucci K2,1, Church A2,1, Subtirelu M2,1. 1University of
Tennessee College of Medicine at Chattanooga, Chattanooga, TN and
2
The Children’s Hospital at Erlanger, Chattanooga, TN.
Case Report: Granulomatosis with polyangiitis (GPA), formerly called
Wegener’s granulomatosis, is an uncommon adult disease with a prevalence
of 8.4-11.3 per million per year among those greater than 15 years of age.
In the adult population, GPA presents with ocular manifestations in 3.530% of cases, orbital disease being the most common form, composing
22% of all eye manifestations of GPA. GPA is less common in children,
occurring 2.75 cases per million children per year. Children most commonly
present initially with GPA with constitutional symptoms (90%), pulmonary
manifestations (80%), upper respiratory disease (80%), and renal disease
(75%). Only 19 % of children present initially with ocular manifestations,
and the documentation of children with GPA who present with symptoms
suggestive of orbital pseudotumor is limited to case reports and case series.
While GPA infrequently presents with orbital inflammation, the renal and
pulmonary manifestations of GPA are life-threatening if untreated. Early diagnosis of the rare childhood case of GPA is critical. This report documents
the case of a 10-year-old female who presented with proptosis and eye pain.
Initial evaluation was negative for a definite cause, and patient was diagnosed
with orbital pseudotumor. After an interval of more than a year, the patient
developed renal and respiratory involvement which led to a diagnosis of
GPA. This case suggests the benefit of ongoing surveillance and high suspicion for GPA in children diagnosed with isolated orbital pseudotumor.
23
FOREIGN BODY INGESTION PRESENTING AS A
MEDIASTINAL MASS
Thiagarajan A, Bradley J, Lawson K, Macariola D. East Tennessee State University, Johnson City, TN.
Case Report: A 4-year-old male presented with a 4-day history of fever &
abdominal pain. Associated symptoms included poor oral intake, decreased
activity & anorexia. Past medical history was significant for recurrent upper
respiratory tract infections & pneumonia. On physical exam, he was afebrile,
in no apparent distress with the rest of the exam findings being unremarkable. Pertinent laboratory tests include elevated ESR while chest x-ray demonstrated a right mediastinal mass. Chest CT confirmed the mediastinal mass
of the upper third of the esophagus while barium swallow demonstrated a
filling defect on the same site. Laboratory work up was unremarkable. He
was treated initially with IV ceftriaxone for suspected infected bronchogenic
cyst. On discharge he received a two-week course of cefdinir. Repeat chest
CT after 2 weeks showed slight resolution of the mass. He underwent a right
lateral thoracotomy. During surgery, this cystic mass was found to be an
esophageal pseudodiverticulum resulting from a piece of plastic imbedded
in the esophageal wall. The esophagus was repaired after removal of the plastic material. A gastrograffin swallow study demonstrated esophageal
narrowing at the operative site without extravasation. He made a full recovery on follow up.
Foreign body ingestion is a common pediatric condition. Often, this is
preceded by specific clinical history revealing the time of ingestion and the
material ingested. However, occasionally patients may present with nonspecific symptoms such as fever, pain, cough, anorexia, and even respiratory
distress necessitating an extensive evaluation. At times these patients can
be misdiagnosed as having bronchitis, asthma or pneumonia & mistakenly
treated for these conditions. Suspicion for foreign body aspiration should increase when symptoms persist or recur despite optimal therapy. Diagnosis is
often obtained by bronchoscopy but may require surgical exploration. Delay
in diagnosis of foreign body aspiration can lead to poor outcomes & therefore should be considered early in the course of the illness so that appropriate
interventions can be initiated. Our case highlights a foreign body masquerading
as a mediastinal mass or bronchogenic cyst. Clinicians should therefore think of
foreign body ingestion in similar situations as depicted in our case.
Wisner E1, Steinhardt M1, Raulji C1, Morrison C1, Zakris E2, Velez M1.
1
Children’s Hospital, New Orleans, LA and 2Touro Infirmary, New Orleans, LA.
Case Report: Herpes simplex encephalitis (HSE) is the most common identifiable cause of viral encephalitis. This infection leaves the majority of
survivors with significant neurologic morbidities. Multiple case reports have
demonstrated the relationship of HSE among cancer patients treated with
CNS radiotherapy. This is the case of a nineteen-year-old female with a
brainstem tumor who was diagnosed with HSE while being treated concurrently with radiotherapy, adjuvant oral chemotherapy, and high-dose steroids.
It is with hope that this report will heighten the index of suspicion for HSE
among cancer patients with altered mental status.
A 19-year-old female with a known brainstem tumor who had already
begun treatment with radiotherapy (3960cGY-22fractions), Temozolamide
(90mg/m2), and dexamethasone, presented to our emergency department
with altered mental status. She developed new-onset seizure activity and
fevers. Broad spectrum antibiotics were initiated and an infectious disease
evaluation began. Her mental status remained depressed which was attributed to anti-epileptics versus sequelae from her brain tumor. An EEG at that
time revealed global slowing and possible offending medications were
discontinued. A MRI revealed a few non-specific, small high T2 hyperintensities within the deep white matter of the bilateral frontal lobes and a
slightly smaller medullary tumor. HSV-I DNA PCR was found to be positive
in the cerebrospinal fluid. The patient was started on Acyclovir which was
escalated to Foscarnet after no improvement in her mental status. A repeat
MRI one week after treatment revealed widespread disease. Despite aggressive treatment, the patient died nine months after the initial diagnosis.
In adjunct with supporting literature, this case demonstrates an association with radiation therapy and steroids with HSE. Our case is unique in that
it presents a pediatric patient, of which there have only been three such cases
reported. It is important to consider HSE in the differential diagnosis among
oncology patients with altered mental status. This report highlights the need
for a prospective study confirming the association of chemo-radiation and
high dose steroids among patients with brain malignancies and HSE.
25
A NOVEL PRESENTATION OF POSTURING AND
HEMIPLEGIA IN AN INFANT FEMALE
Hart R, Skeens R, Puri V. University of Louisville, Louisville, KY.
Case Report: We are reporting a case of a 6 month old female who
presented over several months with progressive episodes of apparent neurologic events: paroxysmal dystonia, nystagmus, alternating hemiparalysis, and
regression of milestones during attacks with restoration of normal development between episodes. After an extensive workup at multiple institutions,
including multiple normal EEGs and MRIs, electrolytes, and genetic testing;
the patient was diagnosed with Alternating Hemiplegia of Childhood (AHC).
This disorder is exceedingly rare with an incidence of approximately 1 in
1,000,000 births but is often mistaken for seizures, a common pediatric diagnosis. These non-epileptiform episodes present unique diagnostic and management challenges. In this case presentation we will explore diagnostic
criteria, etiologies, and treatments of AHC as well as a brief overview of neurologic channelopathies, the current proposed etiology of this disorder. We
will provide an approach to evaluating infants with seizure-like activity and
highlight features that distinguish AHC from epilepsy.
Patient’s Progression of Symptoms and Neurologic Workup
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
415
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Symptoms at presentation of patients with AHC
Leon Dure, MD - University of Alabama Birmingham Department of Pediatrics Division of Neurology; Birmingham, Alabama
Joseph Pressey, MD - University of Alabama Birmingham Department of
Pediatrics Division of Hematology Oncology; Birmingham, Alabama
Abstract: In this report, we present a case of opsoclonus occurring coincidently with hypoglycemia in the absence of malignancy. The patient is a 5
month old term female infant who presented with a 3 month history of
episodes concerning for seizure. The predominant type of episode was characterized by sustained, rapid, darting conjugate eye movements, and they often occurred when awakening from sleep (video available for display). These
episodes were felt to be consistent with opsoclonus-myoclonus, so a neoplastic workup was initiated that was concerning for adrenal mass. During her
workup, the patient was found to have multiple episodes of profound hypoglycemia. This prompted a thorough work up for neonatal hypoglycemia.
Ultimately, work up was consistent with benign adrenocortical adenoma
rather than neuroblastoma, but she was found to have congenital hyperinsulinism secondary to ABCC8 gene mutation. 18-F-DOPA PET/CT scan showed focal disease and partial pancreatic resection was performed. After treatment of
focal hyperinsulinism, hypoglycemia resolved as did opsoclonus. This case
presented numerous diagnostic dilemmas and a fascinating course with multispecialty and inter-institutional collaboration to arrive at the ultimate diagnosis.
26
PILONIDAL CYST LEADS TO DIAGNOSIS OF AML IN
15 YEAR OLD MALE
Roebuck BM, Meyer WH, Dingeldein LM. University of Oklahoma Health
Sciences Center, Oklahoma City, OK.
Case Report: Introduction: Acute myeloid leukemia (AML) is a malignant
clonal proliferation of myeloid precursor cells; it is the second most common
leukemia in children. While 80-90% of children obtain remission after induction, 30-40% of patients will suffer a recurrence with a poor 5 year survival
rate. Most often children present with signs of bone marrow replacement by
malignant cells. We report a case of a 15 year old male treated for a draining
pilonidal cyst and subsequently diagnosed with AML.
Case presentation: A 15 year-old Hispanic male presented to the Emergency Department complaining of a lumbar lesion present for the past month
that for the past three weeks had been draining brown fluid. Prior to admission, he was treated by an outside clinic with incision and drainage, ibuprofen, two different antibiotics, and a course of oral prednisolone. Upon arrival,
the patient also complained of 3 months of fatigue, 6 weeks of subjective
fevers, chills, and night sweats, as well as a two week history of bilateral neck
swelling with tenderness to palpation and new onset snoring with intermittent difficulty breathing that improved with steroids. Physical exam was significant for diffuse lymphadenopathy, hepatosplenomegaly, and an open
non-draining 7.8 cm gluteal cleft abscess. The initial CBC showed marked
leukocytosis (231,000), with blasts (81%), anemia (9.2), and thrombocytopenia (29, 000); both uric acid (9.4), and LDH were elevated (606).
Flow cytometry was consistent with AML, cytogenetics showed inv(16)
(p13.1q22). Histology of the cyst showed extensive myeloblastic infiltration with rod-shaped bacilli. He was treated with combination chemotherapy
(AML0531) and antimicrobials, and the pilonidal cyst slowly resolved.
Discussion: Our patient presented with a simple pilonidal cyst that failed
I&D and standard oral antibiotic therapy. Further investigation resulted in a
diagnosis of AML. Despite presentation of AML with high WBC, this patient had favorable cytogenetics, responded well to induction therapy, and
currently remains in remission. A literature review revealed no cases with a
similar presentation.
28
BREAST MILK AS A SOURCE OF METHICILLIN-RESISTANT
STAPHYLOCOCCUS AUREUS INFECTION IN
PREMATURE NEONATES
Keefe R. Tulane University School of Medicine, New Orleans, LA.
Case Report: Methicillin-resistant Staphyloccus aureus (MRSA) infection
is a well-known cause of severe life-threatening infections in patients in the
Neonatal Intensive Care Unit (NICU). Transmission of MRSA to infants
through their mother’s breast milk has previously been reported as a source
of infection in premature neonates, typically in the setting of maternal mastitis or breast abscess. This is a case report of premature twin infants in our
NICU who developed MRSA sepsis within days of initiating enteral feeds
with MRSA positive expressed breast milk from their asymptomatic mother.
Twin A and Twin B were born at 27 weeks gestational age via emergent
cesarean section for possible pericardial effusion in twin A. Both twins
remained in critical, but stable, condition throughout the first week of life.
Enteral feeds were begun on day of life (DOL) 8. The next day the condition
of twin A rapidly deteriorated. On DOL 10, twin A died of overwhelming
multi-system organ failure secondary to suspected sepsis. Two days following her death, Twin B’s condition worsened. Blood, urine and tracheal aspirate cultures obtained from twin B grew MRSA. On this same day, Twin
A’s tracheal culture obtained on the day of her death grew MRSA. Although
the mother did not have any clinical evidence of mastitis or other breast infection, given the temporal relationship to the initiation of enteral expressed
breast milk feeds and clinical deterioration of both patients, consent was
obtained from the twins’ mother to send a sample of her breast milk for culture, which resulted positive for MRSA. On DOL 20, despite aggressive antibiotic treatment, twin B also died of overwhelming multi-system organ
failure secondary to MRSA sepsis.
Although there are many well documented benefits for early initiation of
enteral feeds and breast milk for patients in the NICU, this case suggests that
premature infants may be at higher risk of severe systemic infection secondary to gut exposure to MRSA positive breast milk. Given this tragic and
likely preventable outcome, we recommend culturing the breast milk of
mothers of premature infants in the NICU prior to initiating enteral feeds.
27
A CASE OF CONGENITAL HYPERINSULINISM
PRESENTING WITH OPSOCLONUS-MYOCLONUS
29
Hoops K, Anderson T, Dure L, Pressey J. University of Alabama
Birmingham, Homewood, AL.
Case Report: Katherine Hoops, MD MPH - University of Alabama
Birmingham Department of Pediatrics; Birmingham, Alabama
Tori Anderson, MD - University of Alabama Birmingham Department of
Pediatrics; Birmingham, Alabama
METASTATIC NEUROBLASTOMA WITHOUT
PRIMARY SOURCE
416
Van Buren JW, Imran H, Wilson F, Siddiqui A. University of South Alabama,
Mobile, AL.
Case Report: Neuroblastoma is an embryonic tumor arising from the sympathetic nervous system. Patients with bone metastases are considered high
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
risk. Histological evaluation of the primary mass is important in establishing
diagnosis and prognosis.Our objective is to report a case of neuroblastoma
with unknown primary mass diagnosed through bone and bone marrow
metastatic sites. A 5 year old male presented with persistent fevers, bone pain
and refusal to walk for 3 weeks. Plain radiographs showed single lytic lesion
in proximal humerus, but magnetic resonance imaging showed increased
fluid signals in multiple vertebrae and long bones. Bone marrow biopsy
showed extensive involvement with small round pleomorphic blue cells suggestive of neuroblastoma. Bone biopsies from the humeral and femoral
lesions revealed similar lesions. The cells stained positive for neuron specific
enolase but the diagnosis of neuroblastoma was confirmed after tyrosine hydroxylase stains were performed by Dr Shimada (second opinion). The
MYCN gene was not amplified. Urine catecholamines were elevated.
Metaiodobenzylguanidine (MIBG) scintiscan showed diffuse increased uptake in most bones but no primary mass was found. Since there are no systematic published data for treatment and outcome of such cases, it was
decided to treat him per the Children’s Oncology Group protocol ANBL0532
for high risk neuroblastoma. He had a remarkable clinical response and
post-induction chemotherapy evaluation revealed no histological evidence
of cancer in the bone marrow. A small MIBG avid lesion in proximal femur
remains that will require radiation. Upon parents’ request, he will now proceed to an immunotherapy trial instead of an autologous stem cell transplantation. To our knowledge there are 13 reported instances of metastatic
neuroblastoma without a primary source. Only 5 had reported outcomes.
At the conclusion of the respective studies, one patient had local recurrence, and two had died and 2 patients were disease free. Studies are
needed to establish a treatment protocol for such unusual presentations
of neuroblastoma. It is difficult to assess an accurate prognosis, but our
patient has had an excellent response, so far, to chemotherapy per high
risk protocol.
30
BUCCAL BIOPSY TO DIAGNOSE NEONATAL
LIVER FAILURE
Ravisankar S1, Misra S2. 1East Carolina University, Greenville, NC and
2
East Carolina University, Greenville, NC.
Case Report: A term male baby who was the product of an uncomplicated
pregnancy presented with lethargy and hypoglycemia at 24 hours of life.
Though hypoglycemia corrected easily with IV fluids, he clinically deteriorated to develop seizures and became encephalopathic. Concerned of an
acute liver failure, work up was broadened from an infectious and metabolic
standpoint including TORCH titers, Viral Hepatitis panel, genetic testing for
PKU, Tyrosinemia pending newborn screen results. With no conclusive
yield, results from an iron panel incited a working diagnosis of Neonatal Hemochromatosis which was eventually confirmed by salivary gland biopsy.
The baby was transferred to a transplant center to be listed, but passed away
shortly later secondary to worsening of his underlying disease process. Autopsy was denied by the family.
Neonatal Hemochromatosis is a rare condition resulting in severe liver
disease with extrahepatic siderosis. About 100 cases have been reported in
the literature. An alloimmune etiology has been proposed, but the exact
etiopathogenesis remains unknown and no gene locus has been identified
so far. Presentations vary from an uncomplicated pregnancy to history of
IUGR, oligohydramnios, decreased fetal movement, with the birth of a baby
who quickly develops signs of acute liver failure. Initiation of specific work
up starts with an iron panel which shows an elevated serum ferritin, low
transferrin with a high TIBC. Biopsy of the minor salivary glands is the gold
standard and a positive iron stain confirms the diagnosis reflecting the extensive extrahepatic siderosis. Management strategies have evolved from using
exclusive iron chelation therapy in the past to now, a combined approach of
using an antioxidant cocktail of drugs, double volume exchange transfusion,
IV Immunoglobulin and eventually listing for liver transplantation. Fulminant hepatic failure is rare in neonates. Data from the Pediatric Acute Liver
Failure Registry reveals that Neonatal Hemochromatosis is a common cause
of liver failure in this group. Hence a high index of suspicion is needed for
early diagnosis, as timely initiation of the newer management strategies have
shown promising outcomes with better prognosis for this otherwise devastating disease.
Southern Regional Meeting Abstracts
31
SUCCESSFUL MANAGEMENT OF INVASIVE FUNGAL
SINUSITIS IN A PEDIATRIC LEUKEMIA PATIENT WITH
PROFOUND NEUTROPENIA
LeBlanc D1, Fletcher M1, Prasad P1, Simon L3, Nuss D3, Seybolt L2,
Velez M1. 1LSU Health Sciences Center/Children’s Hospital New Orleans,
New Orleans, LA; 2LSU Health Sciences Center/Children’s Hospital New
Orleans, New Orleans, LA and 3LSU Health Sciences Center/Children’s
Hospital New Orleans, New Orleans, LA.
Case Report: Invasive fungal sinusitis is a potential complication of
prolonged neutropenia associated with significant morbidity and mortality
(up to 50%). Treatment often necessitates extensive, potentially disfiguring,
debridement. We describe a case of invasive fungal sinusitis in a child with
acute lymphocytic leukemia (ALL) successfully managed with topical and
systemic antifungals and minimally invasive surgical treatment.
A 3-year-old male with ALL in consolidation phase developed febrile
neutropenia. Despite broad-spectrum antimicrobials he remained febrile with
no identifiable source. Absolute neutrophil count (ANC) remained 0. On hospital day 6 right sided paranasal and infraorbital erythema and edema were
noted. He underwent emergent nasal endoscopy and biopsy, revealing extensive black discoloration of mucosal surfaces. The biopsy was consistent with
fungal infection with angioinvasion; culture grew Lichtheimia species.
The patient was started on granulocyte-colony stimulating factor (GCSF) and aggressive antifungal treatment with IV liposomal amphotericin
(5mg/kg/day), oral posaconazole (10 mg/kg BID) and intranasal amphotericin spray (5 mg/mL, 1 spray TID). Emergent surgical debridement demonstrated necrosis of mucosa and bone requiring extensive resection of his
left paranasal sinuses and septum. Over the next month he underwent serial
nasal endoscopies with debridement. The patient completed a 6-week course
of daily liposomal amphotericin and an additional 6 weeks of 3 times per
week dosing. Topical amphotericin and posaconazole were discontinued after 6 weeks. Subsequent surveillance endoscopies showed healing mucosa
with no signs of recurrence.
There is limited literature regarding management of fungal sinusitis in
neutropenic children. With aggressive medical management along with minimally invasive surgical debridement, this invasive fungal infection was
contained and resolution achieved without the need for disfiguring or functionally compromising debridement.
32
GROUP C STREPTOCOCCUS SEPSIS IN A PEDIATRIC
PATIENT WITH TUBEROUS SCLEROSIS
Schmit EO, Diaz F. University of Alabama at Birmingham, Birmingham, AL.
Case Report: Group C streptococci (GCS), a frequent cause of veterinary
disease, are uncommon among human patients but most frequently cause illness in elderly or immunocompromised patients. We report a 2-year-old African American female with tuberous sclerosis and congenital lymphedema
who presented with severe sepsis due to GCS. Initial symptoms were fever,
abdominal pain, and pain in the right thigh. Labs revealed a neutropenia,
lymphopenia, metabolic acidosis, and coagulopathy (DIC). She developed
fluid-refractory shock requiring vasoactive drug support of dopamine at 10
mcg/kg/min and epinephrine at 0.08 mcg/kg/min and mechanical ventilation.
Wide spectrum antibiotics (vancomycin, cefepime, nafcillin, clindamycin,
and fluconazole) were started at admission. Blood cultures grew GCS 11
hours from admission, and antibiotic therapy was adjusted to vancomycin
and gentamicin. No definite source for her GCS sepsis was found. The most
likely primary infections in our patient included endocarditis related to underlying cardiac rhabdomyomas or cellulitis related to underlying lymphedema, but neither source could be proven despite echocardiograms,
abdominal ultrasounds, abdominal CT, and Doppler ultrasound of her lower
extremities. She had a prolonged hospital course including acute kidney injury with resultant hypertension and acute heart failure. She was extubated
at day 12 after admission. She was treated with a 14-day course of antibiotics
from her first negative blood culture. She clinically improved and was
discharged home in her prior state of health after a 21 day admission.
GCS infections are often linked to exposure to domestic animals or unpasteurized dairy products, and can cause a variety of illnesses including upper
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
417
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
and lower respiratory infections, meningitis, bacteremia, osteomyelitis, and
soft tissue infections. GCS are a rare pathogen causing bacteremia, as they
are only isolated from the blood in less than 1% of all positive blood cultures.
Although uncommon, GCS are a clinically important pathogen as associated
infections have a mortality rate of up to 25-30%. GCS are generally highly
sensitive to penicillin. It has been suggested that a synergistic effect may
be achieved with aminoglycosides, but this has not been consistently proven
in studies.
33
CUTANEOUS LEISHMANIASIS ACQUIRED IN THE US IN
A PEDIATRIC PATIENT -THERAPY WITH TOPICAL
KETOCONAZOLE
Singh M1, Balan A2, Parham D3, Weingartner J4, Allen P4. 1University of
Oklahoma, Oklahoma City, OK; 2University of Oklahoma, Oklahoma City,
OK; 3University of Oklahoma, Oklahoma City, OK and 4University of
Oklahoma, Oklahoma City, OK.
Case Report: Background: Leishmaniasis is a parasitic infection primarily
affecting people in the tropics, subtropics and southern Europe. It is rare in
the United States; occasional cases of cutaneous leishmaniasis have been acquired in Texas and Oklahoma.
To our knowledge, only one pediatric patient with this infection has been
reported, making this the first detailed description of a child with cutaneous
leishmaniasis acquired in the United States.
Methods: A 5-year-old boy presented with a one-year history of a 1.5 cm
chronic ulcerative lesion, lateral to the right nasal alar crease. He lived in rural southern Oklahoma, and his only travel history included a visit to Corpus
Christi, Texas, two years prior to presentation. He played in the yard and periodically got insect bites. The lesion remained stable in size, and he was otherwise asymptomatic. Treatment with topical antihistamines and cryotherapy
did not help. Initial pathology was suggestive of leishmaniasis, so that he underwent a second biopsy for confirmation. CDC testing by Leishmania culture and PCR were negative but a definitive diagnosis was made by
electron microscopy.
Results: The decision was made to treat with 2% topical ketoconazole cream
twice daily. After six weeks, the lesion demonstrated significant improvement, so that topical ketoconazole was discontinued; however, the area soon
developed erythema and edema. Topical ketoconazole treatment was
restarted continued for 2 months until the lesion had healed completely, with
only a small biopsy scar remaining.
Conclusion: We present the first detailed description of a pediatric patient
with cutaneous leishmaniasis acquired in the United States, likely in
Oklahoma. Tissue culture and biopsy were negative, and a definitive diagnosis was made by electron microscopy. Given his otherwise benign clinical
course, the most likely species involved was L. mexicana. While illness
caused by this species can be self-limited, our patient seemed to respond to
topical ketoconazole treatment with no side effects, making it an attractive
treatment option.
34
TWO CASES OF MYCOPLASMA ENCEPHALITIS: EARLY
SUSPICION REQUIRED FOR THIS UNDERESTIMATED
PATHOGEN
Onuegbu P1, Taylor S2, Lee A1. 1Florida State University College of Medicine, Pensacola, FL and 2Harbor-UCLA Medical Center, Torrance, CA.
Case Report: A 5 year old male presented with left eye twitching, left hand
shaking and transient lethargy. EEG, neuroimaging and CSF viral and bacterial cultures were negative. The patient developed fever and choreiform
movements, with negative workup for rheumatic fever. Neurologic deterioration continued with progressive dysphagia, dysarthria and self-mutilation. Finally, CSF Mycoplasma pneumoniae IgM resulted positive and azithromycin
was initiated. Anti-NMDA receptor antibody was positive and despite a short
course of steroids and two infusions of IVIG, dysarthria persisted, intermittent twitches remained and he required G-tube placement due to oromotor
dysfunction.
Subsequently, a 7 year old male was admitted for concerns of meningitis.
Physical exam revealed medial deviation of the left eye, photophobia and
418
altered mental status. Empiric antibiotics were initiated; MRI brain was negative. CSF Mycoplasma titers were sent and empiric intravenous
azithromycin initiated. CSF studies then resulted positive for Mycoplasma
IgM , but negative for anti-NMDAR antibodies. Three weeks later the patient
was appropriately responsive, but speech remained slow and ambulation difficult. Five months elapsed before he had returned to baseline health.
We have described two patients with Mycoplasma encephalitis and variable outcomes. We encourage clinicians to maintain a high index of suspicion for Mycoplasma in the acutely encephalopathic pediatric patient and
recommend consideration of early intravenous azithromycin. Once infection
is confirmed or neurologic condition deteriorates, testing for anti-NMDA receptor antibodies should be performed. Anti-NMDA receptor encephalitis is
a debilitating illness manifested by psychiatric symptoms and seizures, presumably autoimmune in nature. Its incidence and possible association with
Mycoplasma infection may be underestimated. Finally, our experience is
consistent with literature describing prolonged hospitalization for these
patients. We recommend such children receive care at a facility with rehabilitation services available.
Case Reports in Cardiovascular Medicine
2:00 PM
Thursday, February 20, 2014
35
HYPERTENSIVE URGENCY AS THE INITIAL
MANIFESTATION OF RENAL CELL CARCINOMA
McShurley T, Jain N. LSUHSC, New Orleans, LA.
Case Report: Renal Cell Carcinoma (RCC) is often associated with
paraneoplastic syndromes due to ectopic hormone production of renin, erythropoietin, insulin, glucagon and parathyroid hormone. Two cases exist of
norepinephrine secreting RCC presenting as hypertensive urgency. We report
a case of a 48 year-old male with history of CABG 6 years prior with subsequent PCI and well controlled hypertension until two months prior presented
to the emergency department with sharp, 9/10, left sided chest pain exacerbated by exertion, relieved with rest and two sublingual nitroglycerin tablets.
Medications included aspirin 81mg, clopidogrel 75mg, amlodipine 10mg
and lisinopril 20mg. Temperature was 98.5, pulse 68, respirations 16, blood
pressure 220/120, SpO2 98% on room air. Exam revealed a regular rate and
rhythm, no murmurs or rub . There was no jugular venous distension. Pulses
were bounding, equal and symmetric. Lungs were clear to auscultation. Abdomen was soft, non-tender, no masses or bruits appreciated. ECG revealed
an old inferior MI and nonspecific ST changes. Renal function was normal
and cardiac enzymes remained negative. Nitroglycerin infusion was initiated
and carvedilol 6.25mg bid was added to his previous regimen. He continued
to have chest pain associated with elevated blood pressure which was relieved with nitroglycerin. Despite increasing carvedilol to 25mg bid and
the addition of nifedipine 60mg, hydrochlorothiazide 25mg and minoxidil
5mg to lisinopril 40mg daily, his blood pressure remained in the range of
160-200/90-110. Renal artery Doppler ultrasound showed no significant stenoses. MRI of the adrenal glands revealed normal appearing adrenals, however a 3.5cm complex cyst with a prominent mural nodule in the right
kidney was found, highly suspicious for renal cell carcinoma. The patient underwent robotic-assisted right partial nephrectomy. Pathology revealed clear
cell renal carcinoma confined within the capsulated cyst. Blood samples prior
to surgical resection were as follows; renin activity level of 0.2ng/mL/hr [0.24.0 ng/mL/hr], TSH: 3.40 mIU/L [0.35-5.50mIU/L], serum aldosterone:
<1.6 ng/dL (4.0-31.0ng/dL], ESR: 5 mm/hr [0-10mm/hr], erythropoietin level:
7.0 [4.0-27.0mU/mL] and D-Dimer: <150ng/mL. At one month follow up,
his blood pressure was 120/80 on long acting nifedipine 60mg daily.
36
URINARY MAGNESIUM WASTING WITH
HYPOMAGNESEMIA AND VENTRICULAR ARRHYTHMIAS
Askari R, Farah V, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Case Report: Purpose: Patients referred for the evaluation of their cardiac
arrhythmias frequently have a disturbance in extracellular cations as the
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
etiologic origin of their dysrhythmia. Marked hypokalemia and hypomagnesemia, alone or in combination, can be associated with life-threatening
arrhythmias. Herein we present the case of an elderly woman with profound
disturbances in serum K+ and Mg2+ that led to symptomatic ventricular
tachycardia (VT).
Case Report: A 64-year-old female without past medical history of hypertension or diuretic use presented to the ER with shoulder pain. During the
course of her evaluation she had a witnessed seizure with lost consciousness
and was noted to have polymorphic VT (Torsades de pointes) requiring DC
cardioversion. Electrolytes: K+ 1.6 mmol/L; Mg2+ 1.3 mg/dL; Ca2+ 8.7 mg/
dL; and creatinine 1.1 mg/dL. Aggressive electrolyte replacement followed.
During hospitalization, a self-limited, asymptomatic episode of VT was
noted. UDS and alcohol level were negative. Baseline ECG: sinus rhythm
with markedly prolonged QTc of 627 ms. Echocardiogram: normal ejection
fraction without atrial or ventricular chamber enlargement. She was
discharged home on K+ and Mg2+ supplements. Six months later, she again
presented to the ER with VT and reported she had not taken her supplemental
Mg2+ for 4 wks. Electrolytes: K+ 2.1 mmol/L; and Mg2+ 1.3 mg/dL with replacement therapy begun and the patient hospitalized. Chest X-ray, CT scan
and abdominal ultrasound were negative. ECG: sinus rhythm, premature ventricular contractions in a bigeminal pattern and prolonged QTc (588 ms).
Urinary Mg2+ excretion of 11.94% indicated Mg2+ wasting. Primary aldosteronism was excluded. Magnesium oxide supplement together with
spironolactone (50 mg po daily), an aldosterone receptor antagonist, attenuated urinary Mg2+ wasting and stabilized serum Mg2+.
Conclusions: Marked reductions in serum K+ and Mg2+ can have profound
adverse effects on the heart, including delayed myocardial repolarization and
prolonged QTc interval, predisposing to life-threatening VT. Serial surveillance of these cations is recommended together with a medical regimen that
preserves them within narrowly defined limits (i.e., K+ ≥4 and Mg2+ ≥2).
37
WARFARIN-INDUCED SKIN NECROSIS IN A PATIENT WITH
HEPARIN-INDUCED THROMBOCYTOPENIA
Jimenez L1, Marcial JM1, Candelario N2, Mesa M3, Osterman A3,
Rochet N3, Altieri PI1. 1University of Puerto Rico School of Medicine, San
Juan; 2Department of Dermatology, University of Puerto Rico School of
Medicine, San Juan and 3Department of Medicine, University of Puerto Rico
School of Medicine, San Juan.
Case Report: A 64-year-old obese woman with history of hypertension, diabetes mellitus, chronic renal insufficiency and coronary artery disease was
admitted to the hospital due to a myocardial infarction for which she was
treated with dual anti-platelet therapy and full dose low-molecular weight
heparin. Four days after discharge, the patient returned to our institution with
worsening shortness of breath, chest discomfort and bilateral lower extremity
cyanosis associated with diminished lower extremities pulses. Platelet counts
of 200,000/mm3 in previous admission had decreased to 121,000/mm3. Subsequent Heparin platelet factor 4 (PF4) antibody testing and 14C-serotonin
release assay confirmed Heparin Induced Thrombocytopenia (HIT). The patient was started on argatroban and bridged to warfarin as therapy for HIT.
Four days after starting warfarin, the patient developed ecchymosis of the entire right breast associated with hemorrhagic bullae. Biopsy of the skin lesion
was performed and showed epidermal necrosis, vessel thrombosis with fibrin
deposition in the superficial and deep dermis, and extravasation of erythrocytes;
Dermatologic manifestations of WISN (left) and HIT (right)
Southern Regional Meeting Abstracts
all suggestive of warfarin-induced skin necrosis (WISN). Anticoagulation therapy is commonly used in the hospital setting. These medications may result in
serious complications, such as thrombocytopenia with the use of heparin or
warfarin-induced skin necrosis. The prothrombotic environment of HIT makes
selection of adequate anticoagulation therapy a challenge. Unopposed warfarin
should be avoided in HIT in order to prevent systemic thrombotic events. Only a
few reports have described an association between WISN and HIT.
38
UNUSUAL INFECTION CAUSING HEART FAILURE
Panikkath D, Soontrapa S, Panikkath R, Perez Verdia A. Texas Tech University of health sciences, Lubbock, LUBBOCK, TX.
Case Report: INTRODUCTION - Infection is a serious complication of
pacemaker (PPM) and implantable cardioverter defibrillator (ICD) implantation.Cardiac device infections can present with pocket infection, blood
stream infection (BSI) and device related endocarditis. METHOD - This is
a case report of a patient who developed ICD related endocarditis. RESULTS We present a 68-year-old female with weakness, fatigue, dyspnea on exertion and pedal edema of 6-8 weeks duration, which was gradually worsening.
She denied fever, cough or chest pain. She had paroxysmal ventricular tachycardia (VT) for which ICD implantation was done in July 2011. She had systemic hypertension and rheumatoid arthritis for which she was on
immunosuppressants, methotrexate and prednisone. Examination revealed a
new systolic murmur and petechiae on the toes. The other systems were
within normal limits. Lab investigations were significant for anemia,
leucocytosis (34,400), elevated brain natriuretic peptide (34,600). The electrocardiogram was normal. New onset heart failure possibly due to Infective
Endocarditis was suspected. Transthoracic echocardiogram showed a 2.6 x
1.1 cm vegetation in the right atrium, attached to the ICD lead as well as
worsening of the ejection fraction to 30-34% compared to 54% previously.
Blood cultures showed gram negative rods identified later as Morganella
morgagni. The infected device and leads were subsequently removed. Culture of the lead tip grew the same organism with a colony count too numerous to count. Patient was given meropenam and vancomycin for 6 weeks
along with an external defibrillator until the course was completed. The
immunosuppressants were stopped for this time period. CONCLUSION Device related infection is an important complication of cardiac device use
with a reported incidence rates of 0-0.8%. Staph species accounts for more
than two-thirds of cases in most series. Gram negative rods are implicated
less often. Morganella morgagni is a very rare device related infection/
endocarditis. This case highlights the importance of considering device
related infection in patients who present with functional decline, even
without clinical signs of infection. Early diagnosis, appropriate antibiotics and
removal of infected hardware increase the chance of survival.
39
BRUGADA BLUES
Isaac J1, Bennett BA1,2. 1University of Mississippi Medical Center, Jackson,
MS and 2University of Mississippi Medical Center, Jackson, MS.
Case Report: Brugada Syndrome (BrS) is associated with ventricular
arrhythmias in structurally normal hearts and sudden cardiac death (SCD)
is often its initial presentation. The condition has a genetic component involving myocardial sodium channel mutations. The characteristic findings
of BrS on electrocardiogram (ECG) are a right bundle branch block pattern
with coved ST elevation in leads V1-V3. While there are several known
triggers that can induce ventricular arrhythmias in patients with BrS, this
case presents a potential trigger that has never been described.
A 17 year old black male with a family history of SCD presented to the
Emergency Department (ED) after a witnessed syncopal episode. He was
intubated in transport for airway protection and sedated at the time of arrival
in the ED. Pupils were sluggish and initial laboratory data was unremarkable.
However, he was noted to have Brugada pattern on ECG (Figure). Computed
Tomographic images of the head were unremarkable and neurologic evaluation including electroencephalogram suggested the patient suffered a hypoxic seizure. Echocardiography was normal. Initial history and medication
review did not provide insight into the mechanism of the patient’s cardiac arrest. However, on the night of admission an anonymous caller reported
that the patient smoked “Blue Cheese Weed” (a psychotropic variant of
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
marijuana) on the day of his arrest. The patient regained consciousness and
full neurologic function.
Several pathophysiologic (fever, infection, sympathomimetic activation)
and pharmacologic stimuli for malignant arrhythmias in patients with BrS
have been identified. Only a few case reports have identified marijuana as
a potential trigger for BrS; however this is the first report of “Blue Cheese
Weed” associated with SCD in BrS.
was compatible with an irregularly-irregular rhythm and “long” thumbs of
both hands. Family history revealed a father with associated upper limb abnormalities and an Atrial Septal Defect.
A 24-hour Holter revealed episodes of atrial flutter and atrial fibrillation,
with associated sinus pauses greater than 3 seconds. Transthoracic echocardiogram revealed a normal structural heart with preserved LV systolic function. Electrophysiologic study showed a counterclockwise typical atrial
flutter. A successful cavotricuspid isthmus ablation was performed. A permanent pacemaker was placed in view of symptomatic Sick Sinus Syndrome.
Holt Oram Syndrome also known as “Heart-Hand” Syndrome is an autosomal dominant inherited disorder characterized by skeletal abnormalities of
the upper limbs and heart problems. It is estimated to affect 1 in 100,000
individuals. It is secondary to a mutation in the TBX5 gene that plays a role
in dividing the heart into four chambers (cardiac septation) and plays a critical role in regulating the development of bones in the arms and hands. Cardiac abnormalities include ASD, VSD and conduction abnormalities like
bradycardia and arrhythmias. Upper limb abnormalities include affected carpal bones in the wrist area, a hypoplastic thumb or a thumb that looks like a
finger. These skeletal abnormalities may affect one or both upper limbs.
40
NOT JUST ACUTE CHEST
Adimora-Nweke D, Bowles E. Tulane University SOM, New Orleans, LA.
Case Report: A 16 year-old African American male with hemoglobin SS
disease and history of acute chest syndrome presented with progressively
worsening sharp substernal chest pain and dyspnea on exertion for one week.
The pain was worse with any movement. He denied cough, any recent
illnesses, but he complained of fatigue, nausea with one episode of vomiting.
Prior vaso-occlusive crises were in limb joints. Family history was significant
for sibling with sickle cell disease and lupus.
On exam, he was tachycardic to 140s with SpO2 of 90%. He spoke in full
sentences and his lungs were clear to auscultation. His cardiac exam revealed
muffled heart sounds without murmurs or rubs. There was no jugular venous
distension.
Chest x-ray revealed enlarged cardiac silhouette. EKG showed electrical
alternans. Echocardiogram showed large pericardial effusion with respiratory
variation of mitral inflow with right atrial and ventricular collapse. The patient was diagnosed with cardiac tamponade and subsequently underwent
emergent pericardial window with drainage of 850 mL of serous fluid. Pericardial fluid was sent for lab analysis.
When evaluating a pediatric patient with chest pain, it is important to
consider the most common etiology and also the most life threatening. Musculoskeletal conditions including costochondritis and slipping rib syndrome account for the largest category of known etiologies. In patients with underlying
comorbidities, diagnoses unique to these conditions must also be considered.
In patients with sickle cell disease, acute chest syndrome is a potentially
fatal cause of chest pain and must be considered. Along with chest pain,
these patient present with a new pulmonary infiltrate involving at least a lung
segment, fever, tachypnea, wheezing, or cough. Conversely, other causes of
chest pain including cardiac conditions must equally be considered. Cardiac
conditions like myocarditis, pericarditis, tachyarrhythmia, aortic root
anomalies, left ventricular outflow obstruction, and coronary anomalies are
serious causes of chest pain and can also occur in patients with sickle cell
disease as with other patients. Patients with angina chest pain, dyspnea on
exertion not attributed to a respiratory cause, and syncope should be evaluated for an underlying cardiac etiology. This evaluation involves history,
physical exam, EKG, CXR, and echocardiogram.
41
HOLT ORAM: THE FORGOTTEN HEART-HAND SYNDROME
Ricci Gorbea F, Cox Rosario R. University of Puerto Rico Medical Science
Campus, San Juan.
Case Report: A 29 years-old male presented with two months history of
palpitations, shortness of breath and near-syncope episodes. Physical exam
420
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Adolescent Medicine and Pediatrics
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
Southern Regional Meeting Abstracts
Conclusions: Overall, data indicate that both iloprost and SNP can restore
responses to H2S that have been blocked by COX inhibition. These data in
newborn pigs suggest that a signal common to PGI2 and NO is required
for H2S-induced cerebrovascular dilation to occur.
44
42
PREVALENCE OF SEXUALLY TRANSMITTED INFECTIONS
AMONG ADOLESCENTS ENTERING CHILD PROTECTIVE
SERVICES
Benjamins L1, Montgomery D2,3, Cheung K3. 1University of Texas School of
Medicine, Houston, Houston, TX; 2Texas Woman’s University College of
Nursing, Houston, TX and 3University of Texas School of Medicine,
Houston, Houston, TX.
Purpose of Study: Adolescents and young adults ages 15-24 years old have
the highest rates of sexually transmitted infections (STIs). Persons in this age
group have been estimated to acquire about half of all incident STIs although
they represent only 25% of the sexually active population. Many believe that
adolescents in protective services custody are at even greater risk of acquiring STIs, however current recommendations from the American Academy of
Pediatrics are to test for STIs based on history and/or symptoms. We set out
to assess the prevalence of STIs among adolescents between the ages of 12 to
18 seen in the Harris County Protective Services Clinic in order to better assess the need for routine screening.
Methods Used: We conducted a retrospective chart review of adolescents
presenting to the Harris County CPS clinic from January 2009 to December
2011. Results of STI testing were abstracted from the charts. Approval from
the Institutional Review Board was obtained.
Summary of Results: Among 437 patients, about 60% were female, mean
age of 15. Approximately 45% were African-American, not Hispanic; just
over 20% were Hispanic. There were 27 (6%) cases of chlamydia, 12 gonorrhea, 3 cases of syphilis, and no cases of HIV. Three of the teens who
reported never having sex were positive for an STI. Female, older than 14
and reporting having had sexual intercourse at least once were all significant
risk factors for testing positive for an STI.
Conclusions: The prevalence of sexually transmitted infections in
adolescents entering foster care was comparable to the national average.
Screening by history alone would have missed about 10% of chlamydia
infections. Therefore, a recommendation for routine screening of all youth
in this population for STIs, including HIV testing, is important to ensure better detection and treatment.
43
PERMISSIVE CONTRIBUTIONS OF PROSTACYCLIN AND NO
TO CEREBRAL VASODILATION TO H2S IN NEWBORN PIGS
Chang K, Berger C, Fedinec AL, Leffler CW. UTHSC, Memphis, TN.
Purpose of Study: Hypoxic-ischemic brain injury is a leading cause of mortality and chronic disability in newborns, causing brain dysfunction and neuronal death. Therefore, it is important to understand the pathophysiology of
hypoxic-ischemic insult and use modulators of cerebral vascular responses
as potential key targets for intervention. Hydrogen sulfide (H2S) is a gaseous, endogenously produced, signaling molecule involved in control of
blood pressure and tone of arteries and arterioles from certain vascular beds.
H2S is a functionally significant dilator in the newborn cerebrovascular circulation. Regulatory mechanisms of dilation due to H2S exposure are unclear. Prostaglandins and nitric oxide (NO) are also important in regulation
of neonatal cerebral blood flow that affect similar regulatory pathways as
H2S. Therefore, this study is designed to address the hypothesis that prostacyclin and NO provide a permissive enabling influence on the newborn cerebrovascular circulation that allows vasodilation to H2S.
Methods Used: In vivo experiments were conducted using newborn pigs
with surgically implanted, closed, cranial windows.
Summary of Results: Topical application of H2S (10-6, 10-5, and 2 x 10-5 M)
dilated pial arterioles in a concentration-dependent manner. This dilation was
blocked by indomethacin, a COX inhibitor (10-6 M) that blocks production
of prostacyclin. The prostacyclin analog iloprost restored dilation in response
to H2S in indomethacin-treated piglets. The NO donor sodium nitroprusside
(SNP) also restored H2S-induced dilation blocked by indomethacin.
EVALUATION OF FAMILY PERCEPTIONS OF SAFETY IN A
PEDIATRIC EMERGENCY DEPARTMENT
Dick R1,2, Sidhu N1,2, Gohman T1,2, Willis C1,2, Thompson T1,2. 1University
of Arkansas for Medical Sciences, Little Rock, AR and 2Arkansas Children’s
Hospital, Little Rock, AR.
Purpose of Study: Arkansas Children’s Hospital utilizes the services of a national firm that specializes in improving health care by surveying customers
after discharge from hospitals. The firm generates results of the surveys,
compares findings from various hospitals and provides feedback to the
hospitals. The emergency department staff at Arkansas Children’s Hospital
wished to improve the scores they receive regarding safety. The questions
that are asked on the standard survey administered by the national firm do
not delineate specific aspects of safety. In this study, the specific issues considered by families concerning safety in the emergency department are
investigated.
Methods Used: Parents and patients were surveyed during their visit to the
Arkansas Children’s Hospital Emergency Department. The purpose of the
survey was explained to parents or patients and they were asked if they
would be willing to participate. The survey contained the following
questions: “Using a scale from 1 to 5, 1 being the lowest and 5 being the
highest, how would you rate the level of safety experienced during your
visit? We are trying to understand what safety means to you. Specifically,
what factors did you consider when scoring the question?” This project
was deemed as a quality improvement project exempt from IRB review at
our institution.
Summary of Results: Fifty four families agreed to participate in the survey
and answer the questions. Families identified several factors that affect their
perception of safety at Arkansas Children’s Hospital Emergency Department.
Preliminary analysis of the data shows the most common issues as follows:
security/police 29.6% (n=16), cleanliness 20.3% (n=11), staff 11.1% (n=9),
metal detectors 12.9% (n=7), care 11.1% (n=6), security badges 11.1%
(n=6) and multiple checks to determine correct patient 11.1% (n=6). The majority of those surveyed (92.6%) rated overall safety as 5.
Conclusions: Patients and families are generally satisfied with the level of
safety at Arkansas Children’s Hospital Emergency Department. Multiple
factors were identified that affect perceptions of safety. These factors may
be utilized by leaders and staff to focus efforts in maintaining and improving
safety for patients and families.
45
ASSOCIATION OF SYSTEMIC INFLAMMATORY RESPONSE
SYNDROME WITH CLINICAL OUTCOMES OF PEDIATRIC
PNEUMONIA PATIENTS
Frazier SB, Sepanski R, Zaritsky AL, Bovat C, Mangum C, Oelberg DG,
Godambe SA. Children’s Hospital of The King’s Daughter’s, Norfolk, VA.
Purpose of Study: The systemic inflammatory response syndrome(SIRS)
may complicate pneumonia; when present, it suggests the patient’s pneumonia is more severe. If so, recognition of SIRS in a child with pneumonia
requires more careful evaluation. To evaluate this hypothesis, we examined
the relationship between the presence of SIRS and adverse clinical outcomes
amongst children with pneumonia.
Methods Used: Retrospective chart review was performed on children with
community-acquired pneumonia who presented to the Children’s Hospital of
The King’s Daughters Emergency Department(CHKD-ED) from Jan 1-Mar
31, 2013. Subjects were identified using ICD-9 codes for pneumonia. Vital
signs and white blood cell counts while in the CHKD-ED were utilized for
this analysis. SIRS was determined by a modified criteria developed by
Goldstein et al. that requires an abnormal temperature-corrected heart rate
or respiratory rate and either an abnormal temperature or white blood cell
count(WBC). Abnormal vitals and WBC used to determine SIRS were adjusted based on the patient’s age. Morbidity endpoints included one or more
of the following: inpatient(IP)/observation(Obs) status or subsequent return
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
421
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
to the ED for pneumonia, need for video-assisted thoracoscopic surgery
(VATS), and total hospital length of stay(LOS), measured from ED triage assessment to final discharge from the hospital(ED, Obs, or IP).
Summary of Results: 276 children met study criteria and were included in
the analysis. Pneumonia patients with SIRS (n=38) had a 79% rate of hospital admission or ED readmission compared with 34.5% of SIRS (-) patients
(n=238) (p<.0001). Pneumonia patients with SIRS were at greater risk of requiring VATS (18.4% vs. 0.8%, p<.0001). Additionally, pneumonia patients
with SIRS had a significantly longer median LOS than pneumonia patients
without SIRS (2.7 vs 0.19 days, p<.0001).
Conclusions: SIRS in children with community-acquired pneumonia is associated with a significantly higher likelihood of experiencing one or more
of the following outcomes: IP/Obs hospital admission or later return to the
ED; subsequent VATS procedure; or longer LOS. Based on these
observations, a sepsis screening tool that identifies SIRS in children with
pneumonia should increase the physician’s concern about the patient.
46
STEP WISE APPROACH TO TRANSITION OF CARE AND
EXISTING CHALLENGES IN ADOLESCENTS WITH SICKLE
CELL DISEASE (SCD)
Freeman B1,3, Benton M2, Gold H3, Wong S3, Chavan R2,3. 1Tulane University, New Orleans, LA; 2Tulane University, New Orleans, LA and 3Tulane
University, New Orleans, LA.
Purpose of Study: A patient education resource to help patients with SCD to
successfully transition from adolescent to adult care and become aware of
common complications of sickle cell disease in the adolescent and young
adult age range.
Methods Used: Major organ complications that are associated with SCD
and specific to adolescence were highlighted. Additionally, the most relevant
and identifiable symptoms for each organ system were provided. Next, the
challenges of transition to adult health care were addressed. Both the psychological effect on adolescent with SCD and the processes involved in adultcentered care are discussed. Finally, important tips for staying healthy and
managing SCD are provided in an informative “dos and don’ts” format.
Summary of Results: With appropriate treatment and management, the life
expectancies for children with SCD have significantly improved and many
are surviving well into adulthood. However, the period of transition to adult
care is a particularly vulnerable time, for the following reasons: 1) In addition to acute pain, SCD is associated with numerous organ complications,
many of which are specific to adolescence; 2) SCD complications may be exacerbated by the inevitable developmental and lifestyle changes associated
with adolescence; and 3) Compliance with self-care and medical treatments,
as well as adjusting to the constraints of living with a chronic disease, is a
challenging issue in this age group.
Conclusions: While the survival rates for children with SCD have dramatically increased, due to the many complications and challenges associated
with adolescence, transition programs are critical in helping SCD patients
successfully transition into adult care. As a part of a step-wise, patientcentered transition program, this information will serve as a patient education
tool that will empower SCD adolescent patients and help them adequately
prepare for transition.
47
A CROSS SECTIONAL STUDY OF INTESTINAL
MICROBIOME IN CHILDREN WITH CYSTIC FIBROSIS
Kasi N1, Harris WT3, Morrow C2, Dimmitt R1, Matthew S1. 1University of
Alabama at Birmingham, Birmingham, AL; 2University of Alabama at
Birmingham, Birmingham, AL and 3University of Alabama at Birmingham,
Birmingham, AL.
Purpose of Study: Children with cystic fibrosis (CF) are prone to intestinal
dysbiosis due to CFTR dysfunction in the intestinal epithelium, recurrent antibiotic exposure, and a CF-specific high fat diet. The clinical relevance of
the disturbed intestinal microbiome to CF clinical outcomes is not wellunderstood.
We hypothesize that intestinal dysbiosis in pediatric CF leads to malnutrition and pulmonary deterioration.
Our study aims to analyze the microbiome in pediatric CF, identifying
alterations in CF patients compared to healthy controls. We will study the
422
presence of dysbiosis in children with CF and its relation to nutrition
(BMI), vitamin deficiency, gastrointestinal manifestations (abdominal pain,
GERD, hepatobiliary disease), and pulmonary function.
Methods Used: To test this hypothesis we conducted a Cross sectional observational study to profile the fecal microbiome in children with CF. We compared
the fecal microbiome of children 3-18 yrs of age to age matched healthy
controls. Intestinal microbiome analysis was performed on stool specimens using culture independent 16S rRNA gene sequencing technology.
Summary of Results: Children with CF had significantly lower microbial
diversity compared to controls (Shannon index, p = 0.008 and Simpson index, p = 0.0114). There was also a significant variability of the microbiota
between the samples, within the CF group compared to controls (beta diversity). The major intestinal phyla were comparable between the two groups.
The CF group had significantly higher colonization by pathogenic enterobacteriales (p= 0.008).
Conclusions: The results of our study provides evidence of intestinal
dysbiosis in children with Cystic fibrosis. A longitudinal study of the temporal variability in the intestinal microbiome in association with antibiotic exposure, nutritional status, gastrointestinal symptoms, and pulmonary
function, will provide the foundation for understanding the impact of the intestinal microbiome in pediatric cystic fibrosis.
48
CLINICAL VARIABILITY OF OSTEOGENESIS IMPERFECTA
WITHIN FAMILIES
Kavanagh K, Andersson H, Morava-Kozicz E. Tulane, New Orleans, LA.
Purpose of Study: Osteogenesis Imperfecta (OI) is a rare connective tissue
disease affecting less than 200,000 patients in the U.S. The current classification system categorizes OI into types I, II, III and IV by clinical symptoms
and genetic components. The difference between the four main clinical
subtypes is their prognosis, dentinogenesis (DI), hearing loss, color of sclera,
stature, skeletal dysplasia and heritability. The current classification system
based on clinical phenotype makes it more difficult to diagnose OI subtype
in members of families with differing phenotypes. We evaluated the variability of OI phenotypes within two families to assess the accuracy of clinical
prognostication in affected offspring.
Methods Used: In one family, the patient had few postnatal fractures and
blue sclera. Her mother had blue sclera, pathologic fractures, short stature
and DI, while her maternal uncle had only hearing loss. All three family
members carry the same mutation in the COL1A1 gene but exhibit differing
levels of severity. In the second family, the patient was noted to have
rhizomelic shortening of her lower limbs on prenatal ultrasound and at birth
was found to have skeletal dysplasia and hearing loss. Her mother had postnatal fractures, short stature, DI and blue sclera. Family history revealed that
Mom has a paternal aunt with a more severe form of OI and no other affected
family members.
Summary of Results: Two families with OI showed phenotypes ranging
from mild to severe with the same gene mutation. The first patient had few
fractures and blue sclera, while her mother had blue sclera, pathologic
fractures, short stature and DI, and her paternal uncle had only hearing loss.
In the second family, the patient had prenatal skeletal dysplasia and hearing
loss, while her mom had postnatal fractures, short stature, blue sclera and DI.
Mom’s father was unaffected and mom’s paternal aunt had a more severe
form with multiple fractures and inability to have children.
Conclusions: Based on the clinical variability observed within families carrying the same mutation, we suggest that OI is a clinical continuum, since
even first degree family members could be diagnosed with the severe neonatal form (type III) or the mildest form (type IV). We emphasize that having a
mild phenotype with OI doesn’t mean that the next generation cannot develop a life threatening form of OI.
49
CHARACTERISTICS OF PEDIATRIC PATIENTS ENROLLED
IN DIABETES TRANSITION PROGRAM
Khawaja M, Lewis K, Paulo R, Bowlby D. Medical University of South
Carolina, Charleston, SC.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: Medical care for adolescents with diabetes mellitus (DM)
as they transition from pediatric to adult care is important. Studies have shown
poor glycemic control, higher rates of diabetic ketoacidosis (DKA) admissions
and lower clinic attendance in adolescents. Several factors contribute to poor
management of DM during this transition period. The multidisciplinary Diabetes Transition Program at the Medical University of South Carolina (MUSC)
will improve patient care. The purpose of this study is to characterize the initial
cohort enrolled in the Diabetes Transition Program at MUSC.
Methods Used: A retrospective chart review collected data (N=133) from
May 31st 2012 to June 1st 2013 on patients enrolled in the Diabetes Transition Program.
Summary of Results: The average age was 17.05 ± 1.08 yrs with 51% males
and 71% were non-Hispanic white. 88% of patients were type 1 DM, and 71% were
using multiple daily insulin injections. The average A1C was 9.17 ± 1.92%, with
only 19% of patients reaching their A1C goal. 6% of patients had a DKA admission. 9% had nephropathy, 8% had hyperlipidemia, 7% had thyroid disease, and
5% had celiac. 15% had ADHD and 9% had depression. 67% of patients lived
in a 2 parent household; however Department of Social Services involvement was
present in 7% of patients. Only 70% of patients received annual screening evaluation. Only 32% of patients had at least 4 annual clinic visits. 27% had Medicaid.
Conclusions: This study supports previous data that has shown adolescents
are poorly managing their DM. The goal of the Diabetes Transition Program
at MUSC is to improve DM care including attaining A1C goal, decreasing
DKA admissions, and increasing clinic visits. This initial cohort data will
be used to monitor outcomes as patients transition from pediatric to adult
care in the Diabetes Transition Program.
50
NEUROLOGIC ADVERSE EVENTS DURING PEDIATRIC
CHEMOTHERAPY PREDICT NEUROCOGNITIVE DEFICIT
McCulloch M1,2, Krull K2. 1University of Tennessee College of Medicine,
Memphis, TN and 2St. Jude Children’s Research Hospital, Memphis, TN.
Purpose of Study: The impact of chemotherapeutic agents on health outcomes
in cancer survivors is currently a popular area of study. However, there is a lack
of studies examining the relationship between actual adverse events experienced during chemotherapy and post-therapy outcomes. This study was
conducted to further investigate the potential of adverse events to predict specific deficits after chemotherapy. Specifically, this study examined the relationship between adverse events during pediatric acute lymphoblastic leukemia
(ALL) chemotherapy and neurocognitive deficits at the end of therapy.
Methods Used: Adverse event data were collected according to National
Cancer Institute criteria for patients on an ALL treatment protocol without
prophylactic cranial irradiation. Patients underwent a comprehensive neurocognitive battery at the end of therapy to screen for deficits in intelligence,
attention, academics, and behavior. Analyses were performed to examine
associations between specific AEs and neurocognitive deficits.
Summary of Results: Thirty-three percent of enrollees experienced a neurological AE. The overall cohort showed significantly impaired means as well
as impairment rates for attention (P<.0001) and psychosomatic measures
(P<.0001). Patients who had a seizure showed a below-average math score
compared to those without a seizure (P=.003). Patients who experienced neuropathy (sensory or motor) or more than two types of neurological AE demonstrated diminished intelligence quotients compared to those without
neuropathy (P=.006 and P=.005, respectively). Finally, children with severe
neuropathy showed highly impaired psychosomatic scores (P=.001).
Conclusions: These results support the role of neurological AEs in predicting
the risk for neurocognitive deficit after chemotherapy in pediatric ALL. Specific
AEs should be used as indicators to initiate interventions that limit associated
neurocognitive deficits and chemotoxicities. More broadly, this study is one
of the first to demonstrate that chemotherapy adverse events can predict impaired outcomes after treatment and thus hold great clinical utility.
Southern Regional Meeting Abstracts
Purpose of Study: The pathogenic roles of cytogenomic aberrations for isolated multicystic dysplastic kidney (MCDK) has not been well investigated
yet. We performed Array Comparative Genomic Hybridization (aCGH) in
10 children with isolated MCDK to reveal the pathogenic mechanisms underlying MCDK.
Methods Used: Patients (six female and four males, mean age 8.5+/- 1.1
years) were diagnosed with MCDK by ultrasonography. All patients had normal renal function at the time of blood specimen collection. Genomic DNA
was isolated from blood leukocytes and buccal cells. aCGH was performed
on Agilent 105K array according to the manufacturer’s protocol. Data was
analyzed using Cytogenomic software package from Agilent. Quantitative
PCR (qPCR) was performed on patients and their parents for each detected
aberration with reference control. The relative copy number was calculated
by DDCt method.
Summary of Results: Three pathogenic aberrations were detected in three
patients. The first aberration (deletion of 2.07 Mb at 7p14.3) includes a region containing 12 genes, including BBS9 and BMPER genes. The second
aberration (duplication of 3.28 Mb at 16p13.11p12.3) includes a region
encoding 20 genes. The third aberration is a monosomy X for a female patient. qPCR demonstrated that deletion at 7p14.3 was paternally inherited,
while the duplication at 16p13.11-p12.3 was maternally inherited. The third
aberration was monosomy X that resulted from meiotic nondisjunction in oogenesis or spermatogenesis.
Conclusions: Our results demonstrate that 30% of MCDK patients possessed
cytogenomic aberrations. Mutations in BBS9 and BMPER have been shown to
produce cystic kidney dysplasia with cystic kidneys, suggesting the pathogenic
function for the deletion at 7p14.3. However, these two aberrations were
inherited from parents, indicating that complex molecular mechanisms underlie
pediatric MCDK. We conclude that: 1) cytogenomic aberrations represent a
common genetic defect in MCDK patients, and 2) aCGH is a valuable tool to
reveal pathogenic mechanisms of MCDK in humans.
52
GASTROSCHISIS: OUTCOMES OF EXTREMELY
PREMATURE INFANTS
Nice T 1 , Russell R 1 , Harmon CM 1 , Fineberg NS 2 , Rogers D 1 , Martin C 1 ,
Chaignaud B1, Anderson S1, Chen M1, Beierle E1. 1Children’s of Alabama,
Birmingham, AL and 2University of Alabama at Birmingham, Birmingham, AL.
Purpose of Study: While prior research has examined the management of
gastroschisis in premature infants, there has been little information focused
on extremely premature infants. The purpose of this study was to identify potential differences in treatments and outcomes of gastroschisis in very premature versus later gestational age infants.
Methods Used: A retrospective review of all infants with gastroschisis was
performed from October 1999 to December 2012. Infants were stratified based
on gestational age: group 1 (very premature <32 weeks); group 2 (near-term
32-37 weeks); and group 3 (term > 37 weeks). Demographic and treatment
data were collected. Outcome measures included length of stay, TPN days, total
operations and non-CVL(central venous line)-related operations, sepsis, NEC,
and death. Analysis was done using Chi square and ANOVA.
Summary of Results: A total of 247 infants were included: 13 very premature, 121 near-term, 113 term. Demographically the groups differ only by
gestational age, birthweight, and lower APGAR scores in group 1. Treatment
was similar across all groups. Group 3 had lower risk of complicated
gastroschisis. Compared to both other groups, group 1 was more likely to require a non-CVL-related operation within the first year. Group 1 also experienced a longer length of stay, increased ventilator days, increased TPN days,
increased risk of sepsis, and increased mortality. [Table 1]
51
CYTOGENOMIC ABERRATIONS ARE COMMON DEFECTS
IN PATIENTS WITH ISOLATED MULTICYSTIC DYSPLASTIC
KIDNEY
Monlezun D1, Chen T1,2, Ramalingam A1,2, Song R1,2, Janssen A1,2,
Preston G1,2, Yospiv I1. 1Tulane University School of Medicine, New
Orleans, LA and 2Tulane University School of Medicine, New Orleans, LA.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
423
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Conclusions: Extremely premature infants with gastroschisis underwent
similar treatment but required more operations, total parenteral nutrition
and ventilator days, and had a higher risk for sepsis and death. While many
did well, survival is markedly lower (62%) and proper parental counseling is
important for these infants.
53
VERTEBRAL OSTEOMYELITIS DUE TO EIKENELLA
CORRODENS AND STREPTOCOCCUS CONSTELLATUS IN A
PEDIATRIC PATIENT
Nye S1, Balan A1, Ramji F2. 1University of Oklahoma Health Science Center, Oklahoma City, OK and 2University of Oklahoma Health Science Center,
Oklahoma City, OK.
Case Report: Introduction: Vertebral osteomyelitis is an uncommon infection in children representing 1-2% of osteomyelitis cases. Infections due to
Eikenella species have been described in adults; however pediatric infections
due to Eikenella species have only rarely been reported. We present the case
of an 11-year old boy with vertebral osteomyelitis due to Eikenella corrodens
and Streptococcus constellatus.
Case Description: A previously healthy 11-year old Hispanic boy
presented with a 6-month history of worsening right-sided occipital
headaches and pain radiating down his neck. He reported phonophobia,
but denied any visual changes, nausea, vomiting, or fever. He had been taking pain medications around the clock. On physical exam, he had trismus,
decreased range of motion and tenderness to palpation over the right side
of his neck, but no erythema or warmth was present.
An MRI of the head and cervical spine revealed a rim-enhancing fluid
collection extending from the atlanto-occipital articulation into the
surrounding soft tissues. In addition, hyper-intense changes in the marrow
of the right petrous, mastoid and temporal bones, occiput, and C1 vertebra
were demonstrated, consistent with osteomyelitis. The middle ear cavities
were clear.
Given his chronic indolent course, tuberculosis was highly suspected, and
he was briefly on anti-tuberculous drugs.
Though technically difficult, a CT-guided needle aspiration of the fluid
collection was performed and the culture was positive for E. corrodens ampicillin MIC 0.25 mcg/ml and S. constellatus penicillin MIC<0.03 mcg/ml.
The patient received antibiotics for six months. Follow up imaging revealed
resolution of his osteomyelitis.
Discussion: While E. corrodens and S. constellatus are commensal
organisms, they can cause serious infections, typically within the head and
neck. Our report describes an uncommon infection due to uncommon
organisms and illustrates the utmost importance of obtaining an adequate
specimen for culture to ensure adequate therapy and excellent outcome.
secrete numerous cytokines that will infiltrate and damage tissues causing
multi-organ destruction. The natural killer cell activity will be depressed or
absent in these patients.
Features of the disease may seem quite non-specific and can look similar
to a febrile illness. The clinical signs can include a prolonged fever, hepatomegaly, lymphadenopathy, splenomegaly, neurological findings and rash.
Laboratory findings are remarkable for cytopenias, a markedly elevated ferritin, elevated liver transaminases and hypertriglyceridemia. All patients with
suspected HLH should have a bone marrow examination to assess for
hemophagocytosis and/or the cause of cytopenias.
Conclusion: Hemophagocytic lymphohistiocytosis is a condition where the
diagnosis can easily be missed due to lack of awareness. A judicious diagnosis is imperative for achieving optimal treatment results and reducing mortality from rapid disease progression.
55
KAWASAKI DISEASE, WHY SO COMPLICATED?
Patel B. University of South Alabama, Mobile, AL.
Case Report: Atypical Kawasaki disease presents with five days of fever and
four of the five following criteria; erythema of palms, soles, edema of hands
and feet, or periungual peeling of fingers and toes after 2-3 weeks, polymorphous exanthem, bilateral non-exudative conjunctival injection, changes in
lips of oral cavity with erythema or cracking, strawberry tongue, or diffuse
erythema of oral or pharyngeal mucosa, unilateral cervical lymphadenopathy, all in the absence of other confounding diseases. Atypical Kawasaki disease presents with fever of 5 days and two of the clinical features of
Kawasaki disease. This disease has the potential for a variety of
complications including: congestive heart failure, myocarditis, pericarditis,
valvular regurgitation, coronary artery abnormalities, aneurysms of medium-sized non-coronary arteries, raynaud phenomenon, peripheral gangrene, arthritis, arthralgias, hepatic dysfunction, hydrops of gallbladder,
aseptic meningitis, extreme irritability, sensorineual hearing loss, urethritis,
meatitis, or anterior uveitis.
We encountered the case of a 5-year old African American male who
presented in hypovolemic shock secondary to complications from atypical
Kawasaki disease. This patient had an unusual and complicated hospital
course developing transaminitis, acute renal failure secondary to renal vasculitis, and carditis with dilated right and left coronary arteries eventually leading to the formation of a saccular aneurysm.
This unique case illustrated the importance of knowing the common and uncommon complications associated with Kawasaki disease and being able to
quickly recognize the disease process so that complications could be avoided.
54
56
FEVER AND A RECURRENT RASH: A CASE OF
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
MRSA STRIKES AGAIN
Paccione R, Leong K, Khalique S, Roy M. LSUHSC-New orleans, New
Orleans, LA.
Case Report: Introduction: Hemophagocytic lymphohistiocytosis (HLH) is
an immune system over activation that can be life-threatening without appropriate diagnosis and treatment.
Case: A 16 month old female presented after a complicated course which
initially began as three skin lesions on her left cheek associated with a high
fever. She was placed on various antibiotics, but the rash persisted and
progressed to extreme swelling of her face, eyes and lips. She was treated
with IVIG and IV solumedrol for two weeks with apparent improvement.
Upon discontinuation, the fever recurred. Upon arrival to CHNOLA, she
was re-started on IV steroids, IVIG and covered with broad spectrum
antibiotics. On laboratory workup, thrombocytopenia, anemia and an elevated ferritin of 3966 ng/ml were found. Further evaluation of NK function,
soluble IL-2R and PRFl gene sequence revealed absent NK cell function.
Bone marrow biopsy revealed hymophagocytosis. Based on these findings,
the diagnosis of HLH was made.
Discussion: Hemophagocytic lymphohistiocytosis is a rare and life threatening condition. It involves over activation of histiocytes and T cells, which
424
Patel SK, Monroe K, Diaz F. UAB, Birmingham, AL.
Purpose of Study: Patient is a 13yo AAM previously healthy presenting
with shortness of breath. He was seen in the ED two days prior with left
shoulder pain and subjective fever for several days. Shoulder Xray was read
as normal and he was discharged with scheduled ibuprofen. That same day,
he was getting off the couch and hit his left leg on the table. Over the next
two days, he had worsening shoulder and leg pain. On day of admission,
he began complaining of shortness of breath. On arrival, vitals were concerning for tachycardia and tachypnea and saturations of 96-99% on 4L
O2 (88% on RA). He was afebrile. Physical exam revealed significant point
tenderness on the left humerus and tibia with a 3cm circumferential difference of left calf compared to the right. His lung exam revealed diffuse rales
and crackles bilaterally. He was alert and oriented. In addition, he had mild
scleral icterus.
Methods Used: Lab work revealed a Lactate of 4.7 with a normal venous
blood gas. WBC was 7.7. CMP was significant for Cr to 1.2, BUN of 40, Bicarbonate 16, Chloride 95, ALT 93, AST 225, and Total Bilirubin of 4.8 with
direct predominance. LDH (1380), UA (26), ESR (16) and CRP (26) were
elevated. CTa revealed multiple bilateral lung nodules and small pulmonary
artery thrombus. D-dimer was >10,000.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Blood cultures were obtained and Vancomycin was started for coverage
of potential underlying sepsis. Leg ultrasound showed a nonocclussive
DVT from distal left superficial femoral vein through the popliteal vein.
Summary of Results: He was admitted to the PICU and remained on 4L of
O2 for some time but was switched over to CPAP for respiratory support. He
received multiple rounds of fluid resuscitation. Few hours later, he was
intubated and required inotropes. He developed and continued to have fevers.
Lovenox was started for DVT management. Vancomycin and Nafcillin were
started for concern of MRSA/MSSA sepsis. The initial set of blood cultures
grew staph, later speciated to MRSA. Repeat daily blood cultures continued
to grow MRSA.
Conclusions: A bone scan revealed osteomyelitis of left femur and left tibia
that was confirmed by MRI. The Orthopedics team performed serial taps to
these locations with cultures that grew MRSA.
The patient was extubated to BIPAP two weeks after admission, and
blood cultures 10 days after admission stopped growing MRSA. He made
a full recovery and was discharged home.
57
DOES OBESITY CONTRIBUTE TO MISSED DIAGNOSIS OF
APPENDICITIS IN CHILDREN AND ADOLESCENTS?
A RETROSPECTIVE REVIEW
Patel S1, Shipon C1, Warner R2, Burns JJ1, Walck J1. 1FSU College of Medicine, Pensacola, FL and 2Lake Erie College of Osteopathic Medicine,
Bradenton, FL.
Purpose of Study: Obesity rates in the United States among children and
adolescents are approximately 17% with 12.5 million affected. Appendicitis
is one of the most common pediatric conditions requiring emergent abdominal surgery. The diagnosis of appendicitis in the pediatric population can be
challenging. Although in one study, obese patients had higher rates of perforation at presentation, there is scant literature that evaluates if obesity is associated with an increased frequency of missed-diagnosis of appendicitis.
The purpose of this study is to determine if obesity is associated with
missed-diagnosis of appendicitis in children and adolescents.
Methods Used: A retrospective chart review from 2008 to 2013, of children
ages 0-17 years, with a confirmed diagnosis of appendicitis at a West Florida
Children’s Hospital, were selected. Obesity was defined as having a BMI
equal to or greater than the 95th percentile for age and sex based on CDC
charts. Cases where BMI percentiles could not be calculated because of
missing age, sex, height or weight data were excluded. The definition of
missed-diagnosis was having had more than one visit to the Emergency Department prior to diagnosis being made. Additional data collected included:
age, sex and MANTRELS score, which is a measure developed to improve
diagnostic accuracy of appendicitis. Statistical analysis was performed using
multiple 2×2 analysis for univariate categorical data analysis, and logistic regression analysis for multivariate analysis.
Summary of Results: There were a total of 209 patients who were entered
into the analysis with 16.7% found to be obese. 25.4% had a missed-diagnosis
on at least one health care visit. No statistically significant relationship
was found between obesity status and missed-diagnosis of appendicitis
(Chi-square 0.818, df 1, p=0.366). Using a multivariate logistic regression,
with age, sex, MANTRELS score and obesity entered into the analysis only
age and sex were related to missed-diagnosis with females (p=0.001) and
younger patients (p=0.004) being more likely to have a missed-diagnosis.
Conclusions: Obesity did not relate to having missed-diagnosis of appendicitis at the time of diagnosis. Younger age and female sex were related to
missed-diagnosis.
58
THE RELATIONSHIP OF MANTRELS SCORE TO
APPENDICEAL PERFORATION IN PEDIATRIC PATIENTS:
A RETROSPECTIVE REVIEW
Patel S1, Shipon C1, Warner R2, Burns JJ1, Walck J1. 1FSU College of Medicine, Pensacola, FL and 2Lake Erie College of Osteopathic Medicine,
Bradenton, FL.
Purpose of Study: Appendicitis is one of the most common conditions
needing emergent operation in the pediatric population and can be difficult
to diagnose. Studies have reported appendiceal perforation to be more
Southern Regional Meeting Abstracts
common in children than adults with duration of symptoms, minority race
and Medicaid insurance as risk factors. The purpose of this study is to determine if MANTRELS score is related to appendiceal perforation.
Methods Used: Children (ages 0-17 years) who had confirmed diagnosis of
appendicitis from 2008 to 2013, at a West Florida Children’s Hospital were included in a retrospective chart review. MANTRELS score, a measure that was
developed to improve diagnostic accuracy of appendicitis, was calculated for
each patient. The scoring system includes the following elements: 2 points
given for right lower quadrant abdominal pain and leukocytosis, 1 point given
for presence of migration of pain to the right lower quadrant (RLQ), anorexia,
nausea/vomiting, rebound pain, elevated temperature, leftward shift of white
blood cell differential. Scores ≥7 are considered “appendicitis likely”.The
MANTRELS score ≥ 7 was evaluated for relationship to appendiceal perforation using Chi-square 2×2 analysis; age, sex and MANTRELS score ≥ 7 was
used for multivariate analysis using logistic regression.
Summary of Results: There were 203 patients entered into the analysis. On
univariate analysis, a statistically significant relationship between
MANTRELS score ≥7 and appendiceal perforation was found. (Chi-squared
11.744; p = 0.001). Using a multivariate logistic regression, with age, sex,
and MANTRELS score entered into the analysis, a MANTRELS score of ≥7
was related to appendiceal perforation (p=0.004).
Conclusions: A MANTRELS score ≥7 was associated with appendiceal
perforation in a pediatric population.
Multivariate Logistic Regression
59
PSYCHOLOGICAL AND NEUROCOGNITIVE OUTCOMES IN
SURVIVORS DIAGNOSED WITH CANCER AS AYA: A REPORT
FROM THE CHILDHOOD CANCER SURVIVOR STUDY
Prasad P1, Hardy K2, Zhang N3, Armstrong G3, Krull K3. 1LSUHSC, New
Orleans, LA; 2Children’s National Medical Center, DC, DC and 3St Jude’s
Research Hospital, Memphis, TN.
Purpose of Study: To characterize psychological and neurocognitive function in cancer survivors diagnosed during AYA in the Childhood Cancer Survivor Study (CCSS), and to identify risk factors associated with impairment.
Methods Used: Participants were survivors (n=6192; mean 23.6 years from
diagnosis) and siblings (n=390) who completed the CCSS Follow-up 2003
(FU 2003) survey, including the Brief Symptom Inventory and the CCSS Neurocognitive Questionnaire. The associations between functional outcomes
(employment, education, and living independently) and psychological and
neurocognitive impairment were examined using logistic regression.
Summary of Results: Among the 6192 survivors, 2589 were diagnosed as
AYA (1602 between 11-15 and 987 between 16-21). Compared to siblings,
survivors reported significantly higher rates of somatization (15.6 vs.
6.7%; OR 2.36, 95%CI 1.55-3.60), depression (11.7% vs. 8.0%; OR 1.55,
95%CI 1.04-2.30), and anxiety (7.4% vs. 4.4%; OR 2.00, 95%CI 1.173.43). AYAs had higher impairment on the CCSS-NCQ, including problems
with Task Efficiency (17.2% vs. 10.8%; OR 1.72, 95%CI 1.21-2.43), Emotional Regulation (19.1% vs. 14.1%; OR 1.74, 95%CI 1.26-2.40), and Memory (25.9% vs. 19.0%; OR 1.44, 95%CI 1.09-1.89); compared to survivors
diagnosed at younger ages. Among survivors with a history of central nervous treatment (i.e., brain tumors or acute lymphoblastic leukemia) those
treated during AYA had greater memory impairment compared to those
treated at younger ages (34.9% vs. 30.8%; OR 1.21, 95%CI 1.02-1.43). Adjusted for current age and sex, survivors diagnosed as AYAs with impaired
task efficiency (OR 2.93, 95%CI 2.28-3.77, p<.0001) or depression (OR
1.94, 95%CI 1.43-2.63, p<.0001) were more likely to be unemployed, while
survivors with memory problems demonstrated reduced educational attainment (OR 1.45, 95%CI 1.17-1.79, p=0.0006).
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
425
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Conclusions: AYA is a critical period of socioemotional growth and the diagnosis of cancer during this period has the potential to disrupt developmental
processes related to educational goals and functions required for independent
living. Since psychological and neurocognitive impairment is related to functional outcomes, further follow-up with the AYA survivors is necessary.
60
OUTCOMES OF ARTHROSCOPICALLY TREATED
FEMOROACETABULAR IMPINGEMENT IN ADOLESCENTS
WITH SLIPPED CAPITAL FEMORAL EPIPHYSIS AND
LEGG-CALVE-PERTHES DISEASE
Rudd J1, Suri M1,2, Heinrich S2, Choate S1. 1Ochsner Clinical Foundation,
Jefferson, LA and 2Children’s Hospital, New Orleans, LA.
Purpose of Study: Legg-Calve-Perthes disease and slipped capital femoral
epiphysis are childhood hip diseases that have lifelong effects. They are severe deformities that lead to femoroacetabular impingement and ultimately
earlier symptomatic osteoarthritis of the hip. Traditional open procedures
to treat FAI cause significant morbidity and have significant recovery time.
Arthroscopic hip osteoplasty has been described to treat these patients in a
minimally-invasive manner. The purpose of this paper is to report outcomes
of arthroscopically-treated FAI in SCFE and Perthes patients 18 years of age
and younger.
Methods Used: Each patient that underwent hip arthroscopy by the senior
author completed the Modified Harris Hip Score, VAS numeric pain scale,
and the SF 12 questionnaire at standard intervals as part of an on-going quality improvement project. The data was collected prospectively and
maintained in a password-protected spreadsheet. With IRB approval, we
performed a retrospective review of the collected data and medical records
focusing on adolescents who were 18 years of age or younger with diagnosed
SCFE or Perthes disease.
Summary of Results: We had 8 patients that conformed to the criteria: 3
previous SCFE patients and 5 patients with Perthes disease. All patients underwent femoral neck osteoplasty with 1 SCFE patient and 3 Perthes patients
having their labrum repaired. Six of the 8 patients had at least 2 year follow
up. With the exception of one Perthes patient, all patients improved per the
administered questionnaires. The one Perthes patient that did not improve
had drastically worsening hip pain with worsening of each score on the
questionnaires.
Conclusions: To our knowledge, there have not been any published results
for post-SCFE arthroscopic osteoplasty in adolescents. There have been
reports of the management and successful outcomes of hip arthroscopy in
Perthes disease, however these studies include all ages treated and do not
focus specifically on the adolescent population. Our study demonstrates
that FAI in SCFE and Perthes adolescents can be treated successfully
arthroscopically with reliable results to relieve symptoms and providing a
less invasive option than the traditional open procedures to these patients.
61
12Q24.31 MICRO DUPLICATION: A NEW CASE AND REVIEW
OF THE LITERATURE DR.LOCHAN SUBEDI M.D; DR.
MARTINEZ M.D
Subedi L Martinez J. University of South Alabama Children’s & Women’s
Hospital, MOBILE, AL.
Case Report: Duplication of chromosome 12q is uncommon. Previously
there has been few case reported for deletion at chromosome 12,but till date
only one case has been reported for 12q24.31 duplication. Going to report a
case of a male child born full term,normal vaginal delivery,no complication.
Mom had an uneventful pregnancy was not on any medication.He was
healthy after birth At age of 4 month he started to have respiratory problem
like stridor,wheezing. He also had a weak ankle for which he use to wear
braces. As he was consistently having respiratory problem he was seen by
Pulmonologist at the age of 17 month when he was diagnosed with
laryngomalacia. He was noticed to have dysmorphic facial feature along with
speech and motor delay.For this region he was referred to Genetics. During
his visit it was noticed that his HC was at 25%ile, height was 75th %ile,
weight was 50th %ile. He had craniofacial dimorphism for low posterior
hairline, short neck, thin upper lip,downward turned mouth, high forehead
426
and dysmorphic looking ear. He also had inverted nipples and hypoplastic
genitalia with a small scrotum. He was hypotonic and had global developmental delay. Genetic work up was done with chromosome analysis, DNA
analysis for Fragile X syndrome and CGH array.CGH chromosomal array
reveled a duplication of 12q24.31 region. 12q24.31 duplication is a rare genetic condition,which is associated with developmental delay.The first case
reported on 12q24.31 duplication was in 2004. It was reported that duplication of the 12q region is a recognizable syndrome characterized by abnormally shaped skull,hypertelorism,flat nasal bridge with a downturned
mouth, short neck with redundant skin, widely spaced nipples,occasional
brain malformation and heart defect,cryptorchidism and intellectual disability. Our patient does have some of the feature that has been already described
like short neck,downturned mouth along with which he also has other
features like low posterior hairline, thin upper lip, high forehead and
dysmorphic looking ear. He also had inverted nipples and hypoplastic genitalia with a small scrotum. He is also hypotonic and has global developmental delay. On last visit when he was 5 year and 6 month old it was noticed that
he has underdeveloped teeth, which is widely spaced.
Adult Clinical Case
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
62
RENAL INVOLVEMENT AS SOLE PRESENTATION OF
GOODPASTURE’S SYNDROME
Ali E, Fenire M, Bashir M, Mazek H, Aly M, Mohamed A. Texas Tech University Health Science Center/Lubbock, Lubbock, TX.
Purpose of Study: 1. We report a case of Goodpasture’s with only renal
involvement.
2. Highlight that early aggressive treatment and supportive care are crucial. 3- Absence of pulmonary manifestation does not rule out Goodpasture’s.
Methods Used: Case analysis and literature review.
Summary of Results: 34 years old female with no significant past medical
history presented with dysuria, polyuria and foamy urine associated with
nausea, vomiting, generalized weakness and facial puffiness of one week duration. She was also found to have blood pressure of 178/92. Labs revealed
creatinine of 5.3mg/dl, blood urea nitrogen of 60mg/dl and glormerular filtration rate of 9.26. Urine analysis revealed red blood cell casts, 300mg/dl
proteinuria, and microscopic hematuria. Additional labs revealed positive
antinuclear antibody and negative double standard DNA antibody, antistreptolysin antibody, and hepatitis panel. Antigolmerular basement membrance antibodies (Anti-GBM antibody) were strongly positive at 7.5 AI.
Renal biopsy confirmed Anti-GBM glomerulonephritis (GN) with crescentic
GN and rapidly progressive GN (RPGN). Treatment with plasmapheresis,
prednisolone and cyclophosphamide was started. Anti-GBM antibody level became negatives, however, the patient progressed to end stage renal failure along
with nephrotic range prteinuria and needed hemodialysis. Patient is undergoing
work up for renal transplant at this time.
Conclusions: Goodpasture’s syndrome is a rare but severe and could be a
life-threatening organ-specific autoimmune disorder that involves kidney
and lungs with incidence of 0.5 to 1 per million. GBM antigen triggers immune attack against non-collagenous domain of chain α3 of type IV collagen
and lead to deposition of anti-GMB immune complexes that manifests as
pulmonary hemorrhage and RPGN with subsequent renal failure. Studies
have shown HLA types strongly influences susceptibility specially types
DR15 and DR4. Timely diagnosis and triple therapy with plasmapheresis,
corticosteroids, and immunosuppression has significant impact on prognosis
and renal function outcome. Most studies have shown a clear association between the extent of glomerular crescent formation and renal outcome. In general 5-year survival rate exceeds 80%. However, no more than 40% of
patients will have independent renal function at 1 year.
63
FULMINANT LIVER FAILURE IN PREGNANCY
Almeida Lalama MV, Rodriguez C, MA. Taylor S. LSU-HSC, New Orleans, LA.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Case Report: The etiology of liver disease in pregnancy ranges from preexisting conditions like chronic hepatitis B or C or autoimmune hepatitis;
liver disease related to pregnancy like intrahepatic cholestatis, acute fatty
liver, pre-eclampsia, Eclampsia or HELLP; to liver disease co-incidental to
pregnancy like drug induced or acute viral hepatitis.
A 32-year-old woman G2, P1 with 26 weeks gestation intrauterine pregnancy presented to an outside hospital with fever, chills, nausea and vomiting
for 4 days. She was admitted with a diagnosis of urinary tract infection and
was started on ampicillin and gentamicin. She continued to be febrile, developed respiratory distress and her liver enzymes were noted to be markedly
elevated. Her temperature was 104.4, pulse 125x’, respirations 26x’ and
blood pressure 110/58, she had an O2 sat of 95% (FiO2 40%). She was alert,
oriented x3, she had conjunctival injection and oral thrush. She had bibasilar
crackles. Abdomen was consistent with gestational age, and was tender in
epigastrium. Extremities were edematous. Laboratory results demonstrated
a WBC of 2.8 (N12% Bands 30%) and anemia but platelet count was normal. Electrolytes and renal function were normal except for a CO2 of 29.
All cultures were negative. Upon transfer her AST was elevated at 2435
and ALT 1157. Total bilirubin was 1.2 and LDH 9003. PTT and PT/INR
were prolonged. Chest x-ray showed mild bibasilar opacities. Due to multiorgan failure, she was intubated, and started on vasopressors, meropenem,
linezolid, ampicillin, acyclovir, and micafungin. Further workup revealed a
positive herpes simplex virus (HSV) type-2 IgM and IgG as well as a detectable serum PCR. At this point all of the antibiotics and antifungal were
stopped. On day 5 she was weaned off pressors and was successfully
extubated. She was discharge on day 12 on valacyclovir to be taken throughout pregnancy.
Approximately 63% pregnant women are HSV-1 positive, 22% are positive for HSV-2 and 13% are positive for both. Since the first reported case of
gestational herpetic hepatitis in 1969, a total of 33 cases have been reported.
The American College of Obstetrics and Gynecology recommends to treat
genital herpes in pregnancy with Acyclovir for 7 to 10 days, with suppressive
therapy through delivery but there is no consensus for treatment of disseminated herpes infection.
64
MARCHIAFAVA-BIGNAMI DISEASE IN A PATIENT WITH
HISTORY OF
ALCOHOL AND COCAINE ABUSE
Al-Obaidi M, Echendu CE, Ahmed M. Texas Tech Univ Health Sciences
Center, Amarillo, TX.
Case Report: Abstract: We report a case of a 59 year old African American
male with history of alcohol and cocaine abuse, and a recent history of a fall
with C2 and wrist fractures, presented with gait disturbance and change in
mentation for the last few months, patient was admitted to the hospital and
was found to have gait abnormalities, scoring low on Mini-Mental Examination. We started the patient on thiamine and folate treatment with mild alleviation of his symptoms. CT scan of the head was done and it did not
show any significant findings, we ordered MRI of the brain, which showed
lesions involving the corpus callosum, these latter findings with patient’s
presenting history correlated with the diagnosis of Marchiafava-Bignami
Disease (MBD). We continued thiamine and folate replacement therapy,
and discharged the patient to follow up with his primary care physician
and physical therapy as an outpatient. We also explore briefly the history
of MBD, methods that have been implemented the diagnosis of this condition and the etiology behind it.
Key words: Marchiafava-Bignami Disease, MBD, Alcohol neurotoxicity,
Cocaine related neurological impairment
65
HYPERCALCEMIC CRISIS AS THE FIRST MANIFESTATION
OF RECURRENT NON-HODGKIN’S LYMPHOMA
Aly M, Bashir M, Nusrat M, Panikkath R, Prabhakar S. Texas Tech Lubbock,
Lubbock, TX.
Purpose of Study: This case report highlights a rare presentation of NonHodgkin Lymphoma (NHL) recurrence with hypercalcemic crisis. Hypercalcemia is a rather uncommon feature of NHL and it is even rarer to see it as
Southern Regional Meeting Abstracts
the presenting feature. Interestingly, this particular patient presented with extra-nodal manifestations (lung nodules, bone, and breast mass) which added to
the complexity of differential diagnosis and lymphoma could have been
overlooked easily. This emphasizes the importance of careful history, examination as well as keeping high level of suspicion about rarer presentations.
Methods Used: Case analysis and literature review.
Summary of Results: An eighty-two year old female with complex history
of past B-cell lymphoma in 1995 and 2005 was in remission since 2007
and history of renal cell carcinoma in 2012 status post nephrectomy was admitted with rapidly deteriorating well-being and extreme fatigue over few
days for no apparent reason. Physical exam showed signs of hypovolemia, altered consciousness, and left axillary lymphadenopathy. Lab investigations
showed serum Ca 17mg/dl, BUN 57 mg/dl, serum Cr 2 mg/dl and low iPTH
level. PET scan showed hypermetabolic pulmonary nodules, bone lesions in
the spine, left breast mass with ipsilateral lymphadenopathy as well as supra
and infra diaphragmatic lymphadenopathy. Steroids were used in addition to
hydration, calcitonin and bisphosphonates. Left axillary node biopsy confirmed B-cell lymphoma and treatment with R-CHOP was initiated. The
patient’s hypercalcemia responded adequately to this management.
Conclusions: Lymphoma as a cause of hypercalcemic crisis is a rare but
should always be considered and investigated. A recent retrospective study
documented 7% incidence of lymphoma in a large series (Majumdar et al,
J Clin Pathol 2002). The cause of hypercalcemia is unclear although many
are associated with increased PTHrp levels. Steroids play a role in the treatment of lymphoma related hypercalcemia. Hypercalcemia is associated with
decreased mean survival and poorer prognosis in NHL. Extranodal lymphoma in the breast, lungs and bones can mislead the diagnosis but a careful
history and considering lymphoma with such manifestations is important for
precise diagnosis, optimal therapy and better prognosis.
66
SEVERE RHABDOMYOLYSIS AS PRESENTATION OF
DISTAL RENAL TUBULAR ACIDOSIS
Aly M, Panikkath R, Ali E, Fenire M, Mohamed A, Buscemi D. Texas Tech
Lubbock, Lubbock, TX.
Purpose of Study: This case highlights a rare case of rhabdomyolysis associated with severe hypokalemia as a presenting complaint of Distal Renal Tubular Acidosis (RTA).
We report a case of a patient who presented emergently with severe hypokalemia, rhabdomyolysis and paralysis; the final diagnosis was distal
RTA. Clinicians need to have increased awareness of RTA as a cause of such
an emergency condition.
Methods Used: Case analysis and literature review.
Summary of Results: A 55 year-old female was hospitalized due to new onset acute, severe weakness and paralysis. The patient reported no recent nausea, vomiting or diarrhea. Her past medical history was positive for
hypertension, depression and hypothyroidism, but no diabetes mellitus. No
history of rheumatologic or renal diseases but recalled a history of recurrent
UTIs when she was young. Her medications were atenolol 50 mg daily and
chlorthalidone 25 mg daily. She had no family history of hypokalemia.
Physical examination showed global hypotonia, with no focal neurological deficits. The patient was hypotensive on presentation and responded to
fluid resuscitation and brief use of vasopressors.
Her serum potassium was 1.1 meq/L, phosphate 1.4mg/dl, Magnesium
1.4mg/dl, with normal gap metabolic acidosis. CK level was above 14K IU/L.
Urine PH was 7 with high 24 hours urine potassium excretion; this remained
high even after stopping chlorthalidone. Renal sonogram was consistent with
chronic kidney disease.
The patient responded well clinically to holding chlorthalidone, replacing
potassium, and adding sodium citrate; she was referred to a urologist to evaluate her recalled history of recurrent UTIs.
Conclusions: Distal RTA is a metabolic disorder characterized by nonazotemic
defect of distal nephron with failure to excrete hydrogen ions causing inability
to acidify the urine to below 5.5 despite the severity of the metabolic acidosis.
Rarely, loss of potassium can severe enough to cause paralysis and/or
rhabdomyolysis as in our case. A new recurrence of UTI can act as the trigger.
In literature, few similar cases have been reported and had different underlying
causes of Distal RTA. Physicians should have a high suspicious index to check
for this disorder, especially when the patient has no apparent clinical explanation of hypokalemia or has been on a stable dose of diuretic for a long time.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
427
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
67
AN UNUSUAL PRESENTATION OF A POSTERIOR
MEDIASTINAL TUMOR
Babbar A, Ramsaran V, Jones L. UF Health, Jacksonville, FL.
Case Report: A 37 year old lady was admitted to the hospital for acute alcohol withdrawal. She had a history of cervical radiculopathy. Surgical history included an anterior cervical disk fusion 2 years prior with no relief of
her symptoms of severe, incapacitating right shoulder and subscapular pain.
She had a 20 pack year history of smoking. Examination revealed an anxious
female in no distress with normal vital signs and a mild tremor. Chest examination revealed tenderness to palpation over her right scapula and normal
breath sounds bilaterally. The rest of the physical examination was normal.
Laboratory investigations were all within normal limits including a toxicology screen and alcohol levels. A chest X-ray done on admission revealed
a right suprahilar mass. Subsequent CT scan of the chest with intravenous
contrast showed a right posteromedial pleural based mass. MRI showed the
mass extending through the foramen at T5 into the spinal canal. A CT guided
biopsy of the mass was performed that revealed a neural tumor consistent
with neurofibroma. The patient underwent a tumor excision by Neurosurgery
in conjunction with Cardiothoracic surgery with subsequent relief of her neuropathic symptoms.
The patient in this case was diagnosed with a Dumb-bell neurofibroma.
Approximately 10% of all neurogenic tumors located in the posterior mediastinum extend into the spinal canal via intervertebral foramina and are
called Dumb-bell tumors. Neurogenic tumors are usually asymptomatic
and discovered during routine chest x-ray. However, they can cause neurological symptoms in 60% of patients with intraspinal extension.
Our patient presented with severe refractory pain. The region of the invasion on MRI was T5-T6, however the patient had pain suggestive of a lesion
in the cervical spine. Her symptoms however were from the actual neurofibroma itself causing pain as it invaded the rib and intercostals muscles. After
having the tumor excised she had complete resolution of her symptoms with
full functionality and returned to work two months post operatively.
68
BRUGADA SYNDROME
Banchs H1, Rivera N2, Banchs-Pieretti H1, Altieri P1. 1University of Puerto
Rico School of Medicine, San Juan and 2VA Caribbean Healthcare System,
San Juan.
Case Report: We present the case of a 29 year old male who presented to the
emergency room with chest pain and loss of consciousness. Physical examination was unremarkable as well as laboratory workup, including cardiac
markers. An electrocardiogram (ECG) was concerning for cardiac ischemia
due to anteroseptal ST segment elevation. Closer inspection revealed ST segment elevations of more than 2mm present in leads V1-V3 with inverted T
waves, compatible with Brugada type I pattern. Echocardiogram showed preserved systolic function without structural abnormalities. Follow up ECG
demonstrated resolution of Brugada pattern. Brugada Syndrome is a hereditary cardiac channelopathy associated with sudden cardiac death in patients
with structurally normal hearts and has a distinctive ECG pattern which
supports its diagnosis. This case reminds us that Brugada Syndrome should
be kept in the differential diagnosis of a patient with ST segment elevation on
the right precordial leads. It is important to note that the Brugada ECG pattern may be transient, as in this case. Associated symptoms are due to ventricular arrhythmias which may be lethal. Patients with Brugada Syndrome
should be evaluated thoroughly for ICD placement and genetic counseling.
Follow up ECG with resolution of type 1 Brugada pattern.
69
A RARE TYPE OF ANEMIA ASSOCIATED WITH MIXED
CONNECTIVE TISSUE DISEASE
Brookins S, Landa C. University of Florida College of Medicine,
Jacksonville, FL.
Case Report: A 70 year old African American female with a medical history
of mixed connective tissue disease presented with malaise and fatigue. The
patient reported onset of symptoms about one week prior with difficulty
performing her usual housework. The patient denied any weight loss, anorexia, fever, chills, or ill contacts. Physical exam was significant for ulnar
deviation bilaterally. Initial hemoglobin was 6.2 g/dL and a hemolytic work
up revealed a haptoglobin less than 10 mg/dL, LDH of 300 U/L, and total
bilirubin 2.4 mg/dL. Reticulocyte count was 11.3% with an index of 5.
The patient was transfused without improvement. Direct antibody test was
positive for both warm and cold agglutinins with no underlying allo-antibody. Prednisone 40 mg daily was started with a mild improvement of the
anemia. Hematology was consulted and the patient was started on 4 cycles
of Rituximab. The hemoglobin improved to 10 g/dL and Prednisone was tapered to 5 mg daily.
The phenomenon of mixed autoimmune hemolytic anemia was first proposed in 1975 by Crookston . The etiology of this syndrome can be idiopathic, or secondary to lymphoproliferative disorders or connective tissue
diseases. Mixed autoimmune hemolytic anemia runs a chronic course with
intermittent exacerbations that do not correlate to cold exposure and may
not manifest with Raynaud’s phenomenom or acrocyanosis. In fact, most
cold agglutinins have no clinical significance and occasionally have high
enough titers to indicate cold agglutinin syndrome. This is the case with
our patient who had not been exposed to the cold but developed significant
hemolytic anemia. Since exposure to the cold does not necessarily trigger hemolysis in mixed hemolytic anemia, avoidance is not an adequate treatment
modality. Mixed autoimmune hemolytic anemia patients seem to respond
similar to the treatment for pure warm hemolytic anemia. Only two cases
have reported the association of mixed hemolytic anemia with mixed connective tissue disease and have demonstrated the efficacy of using Rituximab,
especially in refractory cases to corticosteroids. We postulate that Rituximab
may affect or simultaneously treat the underlying mixed connective tissue
disease, leading to significant resolution of ongoing hemolysis.
70
TREATMENT OF AXIAL PAIN WITH STIMULATION
UTILIZING “CROSS-TALK” BETWEEN EPIDURAL
AND SUBCUTANEOUS PERIPHERAL FIELD LEADS:
A CASE-STUDY
ST segment elevations of more than 2mm V1-V3 with inverted T waves,
consistent with Type 1 Brugada ECG pattern.
428
Clasby CD1, Eissa H2. 1Louisiana State University, New Orleans, LA and
2
Ochsner Baptist, New Orleans, LA.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: 51-year-old female with a 15 year history of chronic neck
and upper back pain following two cervical fusions. Failed pain control included an unsuccessful trial with an epidural spinal cord stimulator, which failed
to cover her pain when the lead was blocked by scar tissue at the C2 level.
Methods Used: Two disparately positioned Octrode® leads were used: one
epidural lead at the C2 level and a second lead subcutaneously across the occiput. Both were connected to an MTS programmed to “cross-talk” and fully
cover her upper back and neck pain. Individual programming of each lead
provided only near-localized coverage of her upper back or neck respectively.
The combined, joint-programming of leads provided near-total coverage of
her pain.
Summary of Results: Previously, the patient reported pain levels averaging
10/10, with best and worst levels of 6/10 and 10/10. Following implantation,
the patient reported greater than 50% relief of her pain, and continued to realize this initial level of pain control.
Conclusions: The patient responded favorably to combined epidural and
subcutaneous field stimulation, with approximately 50% resolution of her
pain. Review of the literature reveals this as the first reported case of combining epidural stimulation and subcutaneous field stimulation using cross-talk
as a method for extending the effective range of SCS to areas otherwise inaccessible to conventional SCS lead placement.
71
PERICARDIAL TAMPONADE AS THE FIRST PRESENTATION
OF SYSTEMIC LUPUS ERYTHEMATOSUS
Copeland S, Rivas M, Hosiriluck N, Nugent K. TTUHSC, Lubbock, TX.
Case Report: Pericardial tamponade is a life threatening condition. It is
rarely seen as the presenting symptom of SLE. We present a case of asymptomatic large pericardial effusion with echocardiographic evidence of early
tamponade physiology. The patient is a 52 year old female with PMH of hypertension who presented to the emergency center with chief complaint of
acute right hemiparesis. Neurologic symptoms resolved within 4 hours and
a transient ischemic attack was diagnosed. A portable chest radiograph was
performed in the EC which showed a “water bottle” shaped heart. The patient
denied fever, chills, weight loss, arthralgias, or arthritis. Physical exam was
positive for distant heart sounds. TTE was performed which showed a large
pericardial effusion with early tamponade physiology. A pericardiocentesis
was performed in which 2.3L or serous fluid was removed. Fluid analysis
was sent which revealed, yellow hazy fluid with 3,675 white blood cells/
mm3. The differential was reported as 53% lymphocytes, 35% macrophages.
Southern Regional Meeting Abstracts
Viral and bacterial cultures were negative. Mycobacteria was also negative.
There was no evidence of malignancy by cytological examination. Serum
analysis was positive for ANA and anti DS DNA with an index of 9 IU/mL.
Anti-Smith antibodies were negative. She was subsequently diagnosed with
SLE. The patient was started on hydroxychloroquine and a prednisone taper.
She currently is doing well with no evidence of recurrence of pericardial effusion. Cardiac involvement in the setting of SLE is common with symptomatic
pericarditis occurring in about 25% of the patients and asymptomatic pericardial involvement in more than half of the cases. Less common is the diagnosis
of pericardial tamponade as the first presentation of SLE. It is important to make
the distinction of whether tamponade physiology is present because this affects
treatment. In instances of effusion without tamponade, corticosteroids are usually effective in resolving symptoms and elimination of pericardial fluid. However, the treatment of cardiac tamponade is to remove the pericardial fluid. This
is usually done by pericardiocentesis or pericardial window. Factors that suggest
the development of tamponade are female sex, african american race, and low
C4 complement levels.
72
EYE OF THE BEHOLDER: DIAGNOSIS OF ACROMEGALY
Cosentino G, Uwaifo G, Richards R, de Silva TN. LSUHSC-NO, New
Orleans, LA.
Case Report: While the clinical signs of acromegaly are distinct, their onset
is insidious. Often the patient, their family members, and primary care
providers who see the patient will not notice the clinical signs of the disease
and diagnosis only occurs when the patient presents to a new provider. Because of this, the average time from onset of symptoms to time of diagnosis
is about 12 years, by which time most patients already have macroadenomas
and associated mass effect. The consequences of excess growth hormone include risk of cardiac complications as well as damage to many other body
systems. Many of the effects are reverseable with removal of the source of
hormone production, usually a pituitary adenoma. In this case we describe
a patient with the classic signs of acromegaly who was diagnosed by the urology team during evaluation of a kidney stone.
This is a 60 year old woman with arthritis who for the past couple years
had struggled with difficult to control diabetes. She was on almost 200 units
of insulin daily and still had glucoses in the 500–600 mg/dL range. She had
arthritis, particularly the hands. She reported that 20 years ago her ring and
shoe size had increased greatly —however both had stabilized many years
prior. She denied vision changes, but she started to have headaches of increasing frequency and severity. Finally when she was admitted for removal
of a nephrostomy tube, a urology resident noticed that she had coarse facial
features and called for an evaluation by the endocrinology service for possible acromegaly. Her family history was notable for a lack of diabetes mellitus
or any endocrine neoplasms. On physical exam, she was noted to have frontal
bossing, prognathia, and coarse facial features. She had enlargement of the
hands and feet and particularly enlarged joints. Laboratory evaluation
revealed a hemoglobin A1c of 15% and a greatly elevated insulin-like growth
factor-1 of 684 ng/mL. After hospital discharge, she was found to have a
3.1 cm pituitary macroadenoma displacing the anterior cerebral arteries
and the optic chiasm as well as extending through the sphenoid sinus. Three
months later she had transsphenoidal surgery to have the tumor removed and
has done well post-operatively without evidence of diabetes insipidus. Two
weeks after surgery she started to notice lower fasting and post-prandial glucose readings.
73
DIFFUSE ALVEOLAR HEMORRHAGE: A RARE
COMPLICATION POST EPTIFIBATIDE INFUSION IN A
PERIPHERAL PERCUTANOUS INTERVENTION
El-Bakush A, Edriss H, Nugent K. Texas Tech University Health Sciences
Center, Lubbock, TX.
Case Report: Introduction: GP IIb/IIIa inhibitors are used in acute and subacute limb ischemia. Although there are no clear guidelines for their use in
peripheral percutaneous interventions, studies have shown their use is associated with lower rates of embolization, less secondary reintervention and
improved limb salvage. Bleeding is a common side effect of their use and
can occur at the access site and in the gastrointestinal or genitourinary tracts.
Alveolar hemorrhage is a rare complication. We present a patient who had a
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
percutaneous lower extremity intervention for critical limb ischemia and developed diffuse alveolar hemorrhage and hematuria.
Case: A 76-year-old man with CAD, hypertension, peripheral arterial disease,
and atrial fibrillation was admitted with a non-healing right foot wound and
right leg pain. He had 80% stenosis of the right internal and external iliac arteries, right common femoral artery stenosis, right superficial femoral artery total
occlusion, and 80% stenosis in the popliteal artery. He underwent percutaneous
transluminal angioplasty and placement of stents. Following the procedure, he
received aspirin and Plavix and was started on Eptifibatide infusion. Over the
next two days he developed hematuria, shortness of breath, and hemoptysis.
His exam revealed decreased breath sounds and crackles. His oxygen requirements increased from room air to 6L/min oxygen by nasal cannula. Chest
x-ray showed bilateral diffuse alveolar opacification consistent with alveolar
hemorrhage or pulmonary edema. His hemoglobin dropped 4g/dL, and he required 4 units of RBC transfusion. Platelets, APTT and INR were all normal.
Echo showed severe pulmonary hypertension. Eptifibatide was discontinued after an 18 hour infusion; the patient was hemodynamically stable. Within 6 days
the hematuria and hemoptysis resolved, and the chest x-ray improved. He was
discharged home on room air.
Discussion: Alveolar hemorrhage is a rare complication of GP IIb/IIIa use
but can be life threatening. Cases have been reported in the literature after
use for acute coronary syndromes and related interventions but infrequently
with percutaneous peripheral interventions. The diagnosis can mimic pulmonary edema and requires a high index of suspicion.
74
DIVERSE COMPLICATIONS IN A PATIENT WITH
LITHIUM TOXICITY
El-Bakush A, Fuller A, Tello W, Nugent K. Texas Tech University Health
Sciences Center, Lubbock, TX.
Case Report: Lithium has been used in management of many psychiatric
disorders. It has frequent side-effects, a narrow therapeutic window and
requires regular monitoring.
Case: 25 year-old woman presented with 2 weeks abdominal pain, nausea, and
vomiting. History was significant for mental retardation, schizophrenia, bipolar
disorder, and hypothyroidism. Medications included lithium, Seroquel, citalopram, Ambien, clonazepam, and levothyroxine. Lithium dose was recently increased. Exam: Respiratory rate of 50, temperature 106.7 F, and Glasgow coma
scale 7. Her level of consciousness deteriorated, and she needed intubation.
Labs showed K+ 2.1mmol/L, Na+ 153 mmol/L, and creatinine 2.7mg/dl.
TSH was suppressed (0.002 mu/ml), and free T4 was high (1.96ng/dl); 5 months
preadmission TSH was 2.540 mu/ml on levothyroxine 25mcg. Antithyroid peroxidase antibodies were 193 Iunits/ml. Lithium level was 4.6mmol/L. She was
given propylthyouracil; cooling measures and hemodialysis were initiated. After hemodialysis the lithium level was 1.0. She developed polyuria and was diagnosed with diabetes insipidus. Seizures were present with jerky movements
of her limbs, suppressed by propofol or lorazepam. She remained unresponsive
7 days. Encephalopathy evaluation included normal CSF analysis, negative bacterial and viral meningitides panels, including West Nile virus. Paraneoplastic
autoantibodies, including AMPA-R, GABA-B-R, NMDA-R antibodies,
were negative. EEG showed triphasic waves and continuous generalized
slowing. MRI was unremarkable. Differential diagnosis: Hashimoto’s encephalopathy, immune mediated encephalopathy, or lithium-induced encephalopathy. She received intravenous steroids for five days. She slowly
improved, became more responsive, and was extubated.
Discussion: Our patient had multiple complications during this episode of
lithium toxicity even though dialysis was started on arrival. She had hyperthermia, diabetes insipidus, and several electrolyte disorders. Prolonged encephalopathy secondary to lithium toxicity is an uncommon complication,
in our patient it developed over a very short period of time. Hypothyroidism
and goiter are common side effects of lithium; our patient developed hyperthyroidism which occurs very infrequently and was probably secondary to silent thyroiditis.
75
TOXIC BELCH: A THREAT TO THE HEALTH CARE TEAM
Farooqi S1, Mughal A2. 1TTUHSC-Lubbock, Lubbock, TX and
Elizabeth’s Medical Center, Boston, MA.
430
2
St.
Case Report: In leading a team of health care providers, physicians must
protect coworkers. This highlights a case of toxic ingestion, which may pose
a risk to the safety of the health care team.
An 18 year old male with no significant history was transferred to
our facility after being found unresponsive. The patient argued with his girlfriend and joked of ingesting a home pesticide. The patient arrived intubated,
unresponsive, and on a dopamine drip. His skin had a grey, ashen appearance. Blood pressure was 50/30mmHg, pulses were faint, thready, and irregularly 30-40bpm. Bilateral crackles were present. Significant labs included
an anion gap metabolic acidosis, remarkable hyperglycemia, and thrombocytopenia. The patient worsened and despite proper ACLS and three vasopressor support, the patient expired after 13 cycles of CPR. EMS provided a
canister of aluminum phosphide (AIP). Post-mortem exam was consistent
with AIP ingestion.
AIP is a boll weevil toxin, used in under developed countries, to protect
grain and tobacco stores during storage and shipment. There are no other
reported cases of intentional ingestion in the U.S. Singer et al hypothesized
the toxicity involved decreased mitochondrial function and oxidative phosphorylation. Ingested orally, it reacts with water and stomach acid to produce
phosphine gas. This is readily absorbed into the bloodstream and epithelium,
and denatures oxyhemoglobin to disrupt enzymes for respiration and metabolism. Lemoine et al. reported the only U.S. cases of accidental exposure, involving 6 family members and two child fatalities. A Simplified Acute
Physiology Score II (SAPSII)5 can be used to predict outcomes although survival is unlikely if >1.5g is ingested. Complications include cardiogenic
shock, metabolic acidosis, respiratory failure and hemolytic anemia. This
toxin poses a significant risk to all providers as in Iran two patients were
noted to have flames erupt from the NGT lavage after mixing to potassium
permanganate, which oxidizes phosphide to phosphate.
76
TRANSIENT LEFT VENTRICULAR APICAL BALLOONING
SYNDROME
Fenire M, Mulkey Z, Mohamed A, Ali E, Mazek H. Texas Tech University
Health Science Center, Lubbock, Lubbock, TX.
Purpose of Study: 1-Report a case of transient left ventricular (LV) apical
ballooning syndrome. 2-Demonstrate the typical clinical and diagnostic
features of the disease
Methods Used: Case analysis and review of literature.
Summary of Results: A 64-year-old woman presented to the emergency department two hours after the acute onset of chest pain (CP) while at her
Mother’s Day party. She had not experienced CP prior to this and at the time,
she was very depressed and less communicative after she remembered her
son who had died two years prior. The CP was severe, central, and pressure-like which radiated to the left arm. Electrocardiogram (EKG) on arrival
showed ST elevation in leads V2 and V3. Troponin T was 0.43 ng/ml and
CK-MB was 14.7 ng/ml. Left heart catheterization showed no significant
coronary artery disease, 40% ejection fraction (EF) and apical ballooning
(panel 1). Transthoracic echocardiography (TTE) on the day of admission
revealed inferoapical and anteroapical hypokinesis. A repeat TTE two days
later showed normal EF and normal wall motion. Repeated Troponin T was
0.09 ng/ml and the patient was discharged home.
Conclusions: Transient LV apical ballooning syndrome is characterized by
transient LV dysfunction, EKG changes that can mimic acute myocardial infarction (MI), and minimal release of myocardial enzymes in the absence
of obstructive coronary artery disease (CAD). It was initially recognized in
Japan in 1990 with the first report emerging from the United States in
1998. A recent study reported a prevalence of 0.9% in patients suspected
of having ST segment elevation MI and admitted for primary percutaneous
coronary intervention. Most reports have noted a clear gender discrepancy,
with much higher prevalence in women. It is typically preceded by exposure
to emotional or physical stressors such as an unexpected death in the family,
abuse, a quarrel, or exhausting work, although in some cases, precipitant
stressors have not been identified. The most frequent clinical symptoms on
admission are chest pain and dyspnea, resembling acute MI. In another study,
only 1.1% of reported patients died during the hospitalization period and almost all surviving patients recovered fully. In the absence of specific
guidelines, the treatment remains standard supportive care and individualized according to the patient characteristics at presentation.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
77
FAT EMBOLISM SYNDROME
Fenire M, Nugent K, Ali E, Edriss H, Zedan A, Mazek H. Texas Tech University Health Science Center, Lubbock, Lubbock, TX.
Purpose of Study: 1).To report a case of fatal fat embolism syndrome FES
in a Down’s syndrome patient with bilateral femoral neck fractures, 2). Provide images of the typical skin, lung, and brain findings.
Methods Used: Case report and review of literature.
Summary of Results: A 59-year-old man with Down’s syndrome in foster
care complained of hip pain. His care taker for the last 8 years woke him
early that morning. He stood from bed and sat in his bedroom chair as usual.
He did not come down for breakfast prompting her to check on him. She
found him complaining that his hips hurt, and he was lying on the ground.
His activity status is that he normally ambulates within the home without
any assistive devices, bathes himself, and converse in a meaningful conversation. Extremity exam showed pain with log roll or any movement attempted
on the hips. Skin exam failed to show any signs of trauma. X-rays revealed
bilateral femoral neck fracture. He had bilateral hemiarthroplasty within six
hours from his injury. He developed respiratory distress and decreased level
of consciousness while in the post anesthesia care unit and had to be
reintubated within an hour from his extubation. Over the subsequent
24 hours, he developed skin rash shown in the panel (1). Despite supportive
care, he remained unresponsive, developed multiorgan failure, and eventually died.
Conclusions: Fat embolism (FE) occurs in nearly all patients (> 90%) with
bone fractures during orthopedic prosthesis procedures and rarely in other
pathologic conditions. Only 3 to 4% will acquire the classical triad of the
FES, which consists of respiratory distress, cerebral abnormalities, and petechial hemorrhages; the rest of these patients remain asymptomatic. The clinical pattern of this syndrome involves a bimodal distribution; some patients
clearly develop a fulminant course with the onset of symptoms within
12 hours of injury. Most patients tend to have a more progressive course with
symptoms appearing 24 to 72 hours after injury. Eileen et al reported only
33% of patients in this study had documented petechiae in a 10-year retrospective review of FES. The most prominent manifestation of the syndrome
is acute hypoxia. Even when lung injury is obvious, its cause may be attributed to infection, aspiration, or traumatic ARDS, rather than to FE. The main
treatment for FES is supportive care.
78
EARLY DIAGNOSIS AND SUPPORTIVE CARE ARE KEY
Fenire M, Nugent K, Mazek H, Mohamed A, Edriss H. Texas Tech University Health Science Center, Lubbock, Lubbock, TX.
Purpose of Study: 1- To report a case of transfusion-related acute lung injury (TRALI), and discuss the differential diagnosis (DDx).2- Early recognition and supportive care are the key.
Methods Used: Case analysis and literature review.
Summary of Results: A 57-year-old man presented with the acute onset of
hematemesis, epigastric pain, melena, and dizziness. He had a low blood
pressure, tachycardia, and O2 sats of 98% on room air. His hemoglobin
was 5.1gm/dl. He received immediate blood transfusion. Two hours later,
the patient’s O2 sats dropped to 89%, and he started on O2. Upper endoscopy
showed a duodenal ulcer spurting blood. He had progressive hypoxia, and
28h later he was intubated. Chest x-ray and computed tomography scan
(CT) revealed diffuse bilateral infiltrates and air bronchograms (Panel 1).
Transthoracic echocardiogram (TTE) revealed normal systolic and diastolic
function. He continued to bleed and he underwent exploratory laparotomy
and pyloroplasty. He stayed on ventilator support for 9 days before he was
successfully extubated. Blood, urine, and lungs washings cultures were negative. Repeat CXR showed resolution of his lung infiltrates. He was seen in
the clinic 18 months after his discharge with no residual lung disease.
Conclusions: TRALI is defined as a “new acute lung injury that occurs during or within 6hrs after transfusion of blood products with clear temporal relationship to transfusion, in patients without or with risk factors for acute
lung injury (ALI) other than transfusion” According to the Food and Drug
Administration, 45% of the fatalities related to blood transfusion in 2012
were caused by TRALI, making it as one of the leading causes of
Southern Regional Meeting Abstracts
transfusion-related fatalities. DDx includes transfusion-associated cardiac
overload (TACO). A history of heart disease, positive fluid balance, increased jugular venous pressure, and the presence of S3 will favor TACO.
Radiographic features of TRALI are nonspecific. CXR initially shows interstitial opacities and lung haziness. This rapidly progresses to widespread bilateral alveolar and interstitial opacities. Parenchymal consolidation and air
bronchograms are seen in heterogeneous distribution on CT scan. These lung
opacities usually clear within 96h without long term sequellae. Diuretics may
worsen TRALI. Supportive care is the main treatment.
79
THE DIFFICULTIES OF ALLOGENEIC STEM CELL
TRANSPLANT: DIFFERENTIATING HEPATIC
VENO-OCCLUSIVE DISEASE FROM ACUTE GRAFT
VERSUS HOST DISEASE
Hansen DK, Milner CP, Elkins S, Buck T. University of Mississippi Medical
Center, Jackson, MS.
Introduction: Hepatic Veno-Occlusive Disease (VOD) is a well-recognized
complication of hematopoietic stem cell transplantation.
In this case report, we describe a non-fatal case of hepatic VOD in a patient who received allogeneic stem cell transplantation after cyclophosphamide and busulfan conditioning.
Case Report: A 25 year old African American male with acute myeloid leukemia with poor risk cytogenetics underwent mismatched unrelated donor allogeneic peripheral blood stem cell transplant with cyclophosphamide and
busulfan conditioning.
The patient developed febrile neutropenia unresponsive to antibiotics
and acetaminophen. Additionally, he developed a generalized skin rash
concerning for graft versus host disease (GVHD). He received steroids and
defervesced. However, by day + 9, he had a thirty pound weight gain and developed direct hyperbilirubinemia and transaminase elevation. Hepatic ultrasound demonstrated hepatomegaly but normal hepatic vasculature.
Differential diagnosis included hepatic VOD and hyperacute GVHD.
Due to further weight gain, rise in bilirubin, and known risks of his conditioning regimen, the patient warranted treatment for VOD. He was transferred to the MICU for emergent tPA in the setting of pancytopenia. The
following day, he was enrolled in a clinical trial with defibrotide therapy.
His bilirubin and transaminases improved with defibrotide, but he required
fresh frozen plasma frequently for supratherapeutic INR. Patient’s course improved with a decline in his bilirubin from a maximum of 32 to 16 mg/dL.
However, on day + 36, his bilirubin began to rise again thus, he had a liver
biopsy, which was consistent with only GVHD; VOD was likely to have been
treated with defibrotide.
Discussion: Significant weight gain, direct hyperbilirubinemia and signs of
hepatic congestion form the diagnostic cornerstone of hepatic VOD.
This case illustrates the formidable challenge of diagnosis and treatment
of hepatic VOD in patients undergoing allogeneic stem cell transplantation.
A high clinical index of suspicion is required to identify patients with
VOD and to initiate timely treatment. In this case, it was difficult clinically
to identify hepatic VOD due to concomitant presence of hyperacute GVHD.
80
WHEN A WHITE COUNT IS NEEDED THE MOST: THE FIRST
CASE REPORT OF A NEUTROPENIC PATIENT SURVIVING
SPOROPACHYDERMIA CEREANA FUNGEMIA
Hansen DK, Milner CP, Zamora A, Albrecht S, Webb R, Buck T, Herrin V.
University of Mississippi Medical Center, Jackson, MS.
Introduction: Sporopachydermia cereana is a cactophilic yeast first identified in 1978.
Only three other cases of S. cereana have been reported. It is exceptionally
rare as a human pathogen and has proved fatal in the other neutropenic patients.
Here, we report the first surviving patient with S. cereana fungemia per
literature review.
Case Report: A 58 year old African American woman with acute myeloid
leukemia underwent successful re-induction chemotherapy with cytarabine,
etoposide, vincristine, and daunorubicin.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Following re-induction, she became profoundly pancytopenic and remained febrile despite broad spectrum antimicrobials, including thirty days
of micafungin. Four consecutive blood cultures demonstrated an unidentifiable Candida species. Micafungin was discontinued and liposomal amphotericin B initiated.
Patient developed renal failure likely secondary to amphotericin B and
respiratory failure from volume overload requiring intubation. Posaconazole
and caspofungin replaced amphotericin for broad coverage of yeast. The candida isolate was identified by a reference laboratory as Sporopachydermia
cereana. The patient denied having any previous or recent exposure to cactus.
While intubated with respiratory failure, patient’s white blood cell count
returned in a timely manner, likely contributing to the resolution of her infection. Repeat cultures demonstrated no growth and the patient’s fevers resolved.
Clinically, patient continued to improve after 20 days of total treatment
with caspofungin and posaconazole and remained without evidence of
fungemia or residual leukemia.
Discussion: Sporopachydermia cereana is an opportunistic cactophilic yeast
which has occured in the setting of refractory hematologic malignancy but
previously resulted in death.
S. cereana is notoriously difficult to detect using conventional mycological
identification techniques. Future work is needed to identify alternative hosts for
S. cereana and to establish optimal treatment and antibiotic duration.
As far as we are aware, this patient represents the first literature report of
patient survival after S. cereana infection acquired during recovery from induction chemotherapy.
81
LATE RECURRENCE OF LIGHT CHAIN DEPOSITION
DISEASE AFTER KIDNEY TRANSPLANTATION TREATED
WITH BORTEZOMIB: A CASE REPORT
Javed T, Moiz A, Garces J, Staffeld C. Ochsner Clinic Foundation,
New Orleans, LA.
Case Report: Background: Herein, we present a case of a patient who developed recurrence of LCDD, several years after kidney transplantation, leading
to allograft dysfunction requiring renal replacement therapy. The patient had
a significant improvement in her renal function after receiving bortezomib
and was able to come off dialysis.
Methods: A 55 YO Caucasian female with history of Type 2 DM and HTN,
ESRD secondary to LCCD underwent living-unrelated kidney transplantation. Her induction consisted of IV solumedrol and rabbit thymoglobulin
(4.5mg/kg in 3 divided dosages). She had immediate allograft function without the need of renal replacement therapy. Her creatinine stabilized between
0.8-1.0mg/dl. After 6 years of transplantation routine labs showed a Cr of
1.8mg/dl and nephrotic range proteinuria (9.5g/g) on spot protein to creatinine ratio. She subsequently underwent a kidney biopsy. A diagnosis of recurrent light-chain deposition disease was made based on the biopsy
findings. Oncology was consulted and a bone marrow biopsy was performed
which was consistent with a plasma cell dyscrasia and she met criteria for
multiple myeloma. Patient initially received five cycles of plasmapharesis
without any improvement. She eventually required renal replacement therapy
three times a week. She then received one cycle of Bortezomib (Velcade
1.3mg/m2 intravenously) along with IV dexamethasone. She received 4
doses of Bortezomib on day 1, 4, 8 and 11. There was no immediate clinical
response to the treatment and she remained dialysis dependent. She was
discharged to a rehabilitation center due to significant debility and deconditioning. Her renal function was closely monitored while she remained on dialysis. She gradually exhibited signs of renal recovery after staying on dialysis
for 2 months. Dialysis was held when her urine output improved (> 1L/day),
and her creatinine eventually settled between 1.5-1.7 mg/dl.
Results:
Conclusions: Bortezomib may provide an alternative to currently available
treatment options in patient with LCDD recurrence after transplantation.
82
A CASE OF HYPOKALEMIA IN A MAN WITH GENERALIZED
WEAKNESS - THYROTOXIC PERIODIC PARALYSIS
Joseph Z, Gosmanov A. University of Tennessee, Memphis, TN.
Case Report: A 31-year old African American male presented to the hospital with history of sudden onset of generalized weakness in all extremities.
432
Patient denied prior medical problems and drug abuse. His review of systems
was positive for occasional palpitations and anxiety spells prior to this presentation. On examination, he had elevated blood pressure of 170/90 mmHg,
heart rate of 109 bpm and was alert and oriented. He had significant proximal
and distal weakness in extremities. He also had bilateral enlargement of thyroid gland with bruits. Initial laboratory evaluation was significant for potassium level of 1.3 (3.5-5.0 mmol/L). Computed tomography of head did not
show any abnormalities. A 12-lead EKG showed sinus tachycardia, no QT
prolongation, and normal QRS complexes. He was admitted for aggressive
electrolyte repletion and telemetry monitoring. Two hours after initiation of
intravenous potassium replacement, his neurologic symptoms resolved. Further inpatient studies demonstrated TSH of <0.05 (0.3-3.0 mIU/L), free T3
of 26 (1.4-4.2 mIU/L) and free T4 of 7.5 (1.4-3.6 mIU/L). Diagnosis of
Thyrotoxic Periodic Paralysis (TPP) was made and the patient was started
on methimazole 20 mg twice daily and metoprolol 25 mg twice daily. Two
months after hyperthyroidism treatment, he became euthyroid and his potassium concentration was 4.4 mmol/L.
Discussion: Profound generalized weakness is a non-specific complaint with
a broad differential diagnosis of potential causes. Intracranial pathology must
be ruled out first. The diagnosis of global weakness requires obtaining a
complete history with special consideration of timing, duration, and distribution of symptoms. Hypokalemia can cause generalized muscle weakness
when potassium level is below 3 mmol/L. TPP is a disorder characterized
by an abrupt onset of hypokalemia and paralysis in patients with hyperthyroidism. The clinical features of TPP are similar to those seen with other
forms of hypokalemic periodic paralysis, but also include the symptoms of
thyrotoxicosis such as weight loss, tachycardia, and anxiety. In patients
who develop TPP, however, the symptoms of hyperthyroidism are often moderate and may be overlooked. Therefore, we recommend evaluation of thyroid function in patients presenting with severe hypokalemia.
83
FILLING DEFECT IS NOT ALWAYS A PULMONARY
THROMBOEMBOLISM
Khawar MU1, Bhardwaj H2, Awab A2. 1OUHSC, Oklahoma city, OK and
2
OUHSC, Oklahoma CIity, OK.
Case Report: Pulmonary artery sarcoma (PAS) is a rare malignancy arising
from the mesenchymal cells of the intima of pulmonary artery. PAS is often
misdiagnosed as pulmonary thromboembolism (PTE), as both diseases initially appear as intraluminal “filling defects” in the pulmonary artery system
on contrast-enhanced CT-scans. The differentiation of these two entities is
important to avoid misdiagnosis of a potentially fatal malignancy and unnecessary anticoagulation therapy. We describe a patient initially misdiagnosed
as PTE not responsive to prolonged anticoagulation therapy, who later was
found to have PAS.
A 76-years-old male with medical history of hypertension presented with
dry non-productive cough, pleuritic chest pain and dyspnea. Computed tomographic (CT) angiography scan revealed a filling defect in the left main
pulmonary artery. Unfractionated heparin was started and bridged to
Coumadin; patient was discharged home with the diagnosis of PTE. After
discharge, patient’s symptoms kept worsening with additional anorexia &
weight loss. Due to worsening dyspnea and weight loss of 20 pounds over
the next 3 months, a follow-up CT-Chest angiogram was performed. This
revealed a 16 x 10 x 8 cm mass in the left main pulmonary artery which
seemed to have originated from the filling defect seen on the initial CT-scan.
An ultrasound-guided biopsy of the mass was consistent with the diagnosis
of PAS on histo-pathological analysis. Due to extensive disease, only the palliative chemotherapy was offered, which he declined.
PAS is a rare and often lethal tumor usually diagnosed during surgery or
autopsy. The absence of predisposing factors for PTE and the progressive
worsening dyspnea in patients with an intraluminal filling defect despite
anticoagulation therapy are factors suggestive of PAS. Recently, it has been
reported that, positron emission tomographic scan (PET-CT) may help to
make a differential diagnosis between PAS and PTE. If detected earlier, surgical resection remains the primary treatment. For these reasons, a correct
early diagnosis is of fundamental importance. Clinicians should maintain
high suspicion of PAS in patients with a filling defect in the pulmonary artery
without any underlying risk factors for PTE and worsening of symptoms despite anticoagulation therapy.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
84
ANOMALOUS ORIGIN OF THE RIGHT CORONARY ARTERY
FROM THE LEFT CORONARY SINUS
Mazek H, Omar S, Strefling J, Edriss H, Mohamed A, Wischmeyer J. Texas
Tech University Health Science Center, Lubbock, TX.
Purpose of Study: Right coronary artery anomalies are very rare and when
present are found at birth. Usually these anomalies are asymptomatic and
discovered incidentally during coronary angiogram or computed tomography
scan. The most common coronary anomalies are related to left circumflex or
left anterior descending arteries. We are reporting a rare case of a right coronary artery originating from the left coronary sinus.
Methods Used: Case analysis and literature review.
Summary of Results: We are reporting a case of an anomalous origin of the
right coronary artery from the left coronary sinus in a 33 year old man with
no significant past medical history . The patient was admitted to our hospital
after he collapsed secondary to ventricular tachycardia requiring cardiopulmonary resuscitation and multiple defibrillating shocks. Electrocardiogram
showed inferior ST segment elevation with elevated troponin. The patient
was diagnosed with ST elevation myocardial infarction. The patient underwent urgent cardiac catheterization. Coronary angiogram showed an 80%
stenosis of the proximal left anterior descending coronary artery as well as
a likely anomalous takeoff of the right coronary artery from the left coronary
cusp. Cardiac CT angiography was preformed: revealing a right coronary artery arising from left coronary cusp, the arterial course run anteriorly between the aorta and the pulmonary artery that appeared to be stenosis of
70% of right coronary artery secondary to impingement from gear vessels.
The patient underwent coronary artery bypass grafting and recovered well
postoperatively.
Conclusions: Our case report focuses on a right coronary artery anomaly
which originated from the left coronary sinus. Although many C Anomalies
are asymptomatic, an anomaly that courses between great vessels can lead to angina pectoris, myocardial infarction, or sudden cardiac death, our case can be
explained by mechanical compression of the right coronary artery by the great
vessels. Cardiac CT angiography is important to determine the relationship of
the anomalies artery to the greats in order to guide for the best way to manage.
85
A RARE CASE OF ADULT ALVEOLAR SINONASAL
RHABDOMYOSARCOMA
Mazek H, Konala V, Nusrat M, Omar S, Warraich I, D’Cunha N. Texas Tech
University Health Science Center, Lubbock, TX.
Purpose of Study: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin thought to arise from cells committed to a skeletal muscle
lineage. It is a common childhood tumor, but very rare in adults. There are
four histological subtypes of RMS: embryonal, alveolar, pleomorphic and
botryoid. Little et al. reported 43% pleomorphic, 34% embryonal, and
23% alveolar in adult population.
Methods Used: Case analysis and literature review.
Summary of Results: A 60-year-old gentleman presents with right nasal obstruction associated with minimal nasal bleeding. Physical examination was
unremarkable. CT scan of the paranasal sinuses revealed a 1.5 x 2.7 cm
polyp in the right nasal cavity. Patient underwent endoscopic transnasal resection of the right nasal mass. Immunohistochemical staining showed that
the neoplastic cells are strongly reactive with antibodies to desmin, myogenin
and CD56 but negative for CD99, S100 protein, CAM5, chromogranin,
pankeratin or synaptophysin. These findings along with morphology strongly
support the diagnosis of alveolar RMS . No evidence of distant metastases
was found on further imaging. Patient treated with radiation to prevent local recurrence. He was started on chemotherapy with vincristine, dactinomycin, and
cyclophosphamide and planned for a total of one year.
Conclusions: RMS can develop in almost any part of the body, the most
common locations being head and neck (nearly 40% of all cases), genitourinary system and extremity. Histologically, the most common forms found in
the head and neck are the embryonal. Sinonasal RMS (SNRMS) is a rare malignancy which often presents with nasal obstruction, rhinorrhea and epistaxis. Immunohistochemical analysis plays an important role in the definitive diagnosis.
Treatment is multimodal, with surgical debulking followed by radiotherapy and
Southern Regional Meeting Abstracts
chemotherapy, which appears to permit effective control and possible cure
of this disease. A study from Wurm et al. reported 5-year survival with
embryonal SNRMS and alveolar SNRMS to be 55% and 33% respectively. Adult alveolar RMS is more aggressive with a worse overall survival than pediatric alveolar RMS.
86
PATIENT WITH DIFFICULT BREATHING AND
EPISTAXIS - A SUBTLE PRESENTATION OF A RARE AND
AGGRESSIVE NEOPLASM
Mazek H, Omar S, Suvorava N, Konala V, Warraich I, Figueroa J. Texas Tech
University Health Science Center, Lubbock, TX.
Purpose of Study: Melanoma is mostly of cutaneous origin, it can also occur in various extra cutaneous regions including ocular, mucosal and
leptomeningeal sites. Mucosal melanoma of head and neck (MMHN) is a
rare disease that makes up to less than 1% of all melanoma cases in United
States. We present a 63 year old patient with MMHN.
Methods Used: Case analysis and literature review.
Summary of Results: A 63 -year-old male presented with difficult breathing
and progressively increasing left nasal obstruction symptoms for about a
month with some episodes of a small amount of epistaxis. He was referred
to ENT surgeon for possible polyp or mass in the left nostril. Maxillofacial
CT scan revealed a 6.1 x 1.2 x 8.4 cm left nasal cavity mass involving the left
inferior turbinate. A staging PET/CT scan did not show any distant metastases. He underwent left endoscopic ethmoidectomy. The histopathology was
consistent with mucosal malignant melanoma (MM). BRAF mutation was
negative. Patient was treated with stereotactic radiation therapy to his nasal
cavity. Repeat PET/CT scan shows resolution of metabolic activity in the left
nasal maxillary region.
Conclusions: Mucosal MM within the nose and paranasal sinuses represents
approximately 4% of all nasosinusal neoplasm’s, affecting predominantly age
group over 60’s, equally in both sexes. They are asymptomatic in early stages,
which delays diagnosis and reduce the possibilities of cure. The majority of
the patients present with epistaxis, and progressively increasing nasal obstruction. The diagnostic tools include a thorough physical examination, imaging, biopsy, and even then diagnosis remains exceedingly difficult.
The treatment of choice in mucosal MM is surgery followed by radiation
therapy in cases of small or doubtful margins of resection. There is no effective systemic therapy for these aggressive tumors. Compared with cutaneous
and ocular melanoma, mucosal melanomas have poor prognosis with 8% to
30% five-year survival.
This case highlights that melanoma can occur at extracutaneous sites including mucosal origin. Given the delay in diagnosis because of minimal
symptoms, high index of suspicion is required.
87
OPPORTUNISTIC INFECTION IN IMMUNOSUPPRESSED
RENAL TRANSPLANT PATIENTS WITH RECURRENT
DISEASE
Mazek H, Jamal F, Fenire M, Ali E, Aly M, Laski M. Texas Tech University
Health Science Center, Lubbock, TX.
Purpose of Study: Treatment of allograft kidney involves risks of opportunistic and nosocomial infections, this risk increases if the treatment is altered
for any recurrence of disease or rejection of transplant kidney.
Methods Used: Case analysis and literature review.
Summary of Results: 61 year old man with a history of deceased donor kidney transplant 20 years prior, for ESRD, secondary to glomerulonephritis. He
had a good allograft function with a serum creatinine level of 1.6 mg/dl. He
was treated with Cyclosporine 100mg bid and Azathioprine 50 mg daily. Cell
counts of CD3 911, CD 4 475, CD8 403 and CD4/CD8 1.2.
Patient was noted to have a rise in serum creatinine to 2.5mg/dl. Urinalysis showed mild hematuria and mild proteinuria. ANCA levels were now
positive for anti-PR3 antibody. Transplant kidney biopsy showed 40% interstitial fibrosis and tubular atrophy. Glomeruli showed cellular and fibrous
crescents and moderate thickening and hyaline changes were seen with in
the arterioles. Impression was recurrent pauci immune necrotizing glomerulonephritis. Patient was treated with 3 doses of IV Cyclophosphamide and
pulse dose Solumedrol. After the 3rd dose, patient developed fever, chills
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
and left sided neck swelling. Examination revealed firm, tender, left sided parotid gland. No dental caries or abcess were seen. Biopsy of parotid gland
showed findings consistent with Actinomyces. Culture showed actinomyces.
He was treated with one month course of high dose IV Penicillin. Cyclophosphamide was discontinued and immunosuppression changed to Cyclosporine, Azathioprine and Prednisone. Patient’s renal function remained stable
after treatment.
Conclusions: Many Actinomyces species are opportunistic pathogens of
humans. Greatest risk factor for actinomyces is poor oral hygiene and one
predisposing factor is immunosuppression. Recurrence of Pauci-immune
vasculitis with positive ANCA is common, with renal involvement in more
than half of these recurrences but graft losses reported in only a minority.
One of the side effects of Cyclophosphamide therapy is a risk for opportunistic infections like actinomyces infection and in this case is the probable cause
of actinomyces infection.
88
FOREIGN BODY ASPIRATION MASQUERADING
AS ASTHMA
McCurdy K1, Ramsaran V2, Cury J2. 1UF COM Jacksonville, Jacksonville,
FL and 2UF COM Jacksonville, Jacksonville, FL.
Case Report: An 18 year old man with a three year history of asthma
presented with a left lung collapse. We present his clinical course and discuss
how a foreign body aspiration can mimic asthma.
The patient presented with fevers, dyspnea, and a productive cough after
being hospitalized 1 year ago for similar complaints. He had been diagnosed
with asthma 3 years prior.
On admission, he was in moderate respiratory distress. Lung examination
revealed markedly decreased breath sounds on the left and left sided dullness
to percussion. Right lung field examination was normal. CXR demonstrated
near complete opacification of the left hemi thorax. CT chest showed diffuse
consolidation of the left upper and lower lobes and occlusion of the distal left
mainstem bronchus.
Bronchoscopy was performed showing pus from the left mainstem bronchus and a left lower lobe endobronchial lesion. A foreign body in the left
lower lobe was visualized and removed intact. Gross pathology confirmed
the object to be a pen cap cover and the patient remembered choking on this
3 years prior. He was discharged on a course of oral antibiotics. Since discharge, he has stopped using his asthma medications as he no longer
wheezes. He is now able to perform physical activity at his normal level prior
to this illness.
Foreign body aspiration is the 5th most common cause of unintentional
injury-related mortality in the US claiming the lives of over 3,700 patients
in 2007.
Our case demonstrates the importance of a thorough history including
probing for remote events given the high incidence of remote inciting events
among adults aspirating foreign bodies. Clues which suggest possible remote
aspiration include; history of recurrent pneumonias or hospitalizations, unilateral wheezing, and patient propensity for aspiration.
Additionally, this case exemplifies the importance for clinicians to remain
critical of prior diagnoses and always employ or obtain confirmatory studies
prior to accepting past medical problems as true. Our patient had been incorrectly diagnosed and treated as asthma for 3 years following his aspiration
episode despite never undergoing confirmatory testing (i.e. PFTs with bronchodilator challenge). Clinicians can learn from this and not hold prior diagnoses as true until adequately confirmed.
89
COULD TUBE FEEDING CAUSE WEIGHT LOSS?
Mohamed A, Fenire M, Soape M, Mazek H, Aly M, Rakvit A. Texas Tech
university health science center, Lubbock, TX.
Purpose of Study: Clinicians should assess diarrhea closely and consider
tube misplacement when the diarrhea is persistent, all other stool studies
are inconclusive or a diarrhea not responding to abortive treatment in patients
with percutaneous endoscopic gastrostomy (PEG) tube feeds.
Methods Used: Case Analysis and Review of Literature.
Summary of Results: Patient is a 66 years old male with history of cervical
myelopathy lead to cervical fusion one year ago. He presented with intractable diarrhea, weakness, and 30 pounds weight loss. He denies any nausea,
434
vomiting, abdominal pain or blood in the stool. The patient has a history
of a PEG tube insertion due to pharyngeal muscle weakness and risk of aspiration 6 weeks prior to presentation. The patient looked cachectic. Abdominal exam revealed scant purulent discharge at the site of PEG tube. Labs
showed normocytic, normochromic anemia and decreased levels of pre-albumin, albumin, magnesium and phosphorus. Stool studies were negative for
any infection. A subsequent computed tomography scan of the abdomen
showed the PEG tube balloon tip in the transverse colon which is subsequently confirmed by a tubogram. The patient then underwent a surgical repair. A well matured colocutaneous fistula (CCF) was found during surgery.
A new G-tube was placed and tube feedings restarted. He was able to tolerate
tube feeds at a goal of 60 mL/hr.
Conclusions: The PEG tube was first described in 1980 but has since been
widely used as an option for nutritional replacement therapy. Known
complications of the procedure range from minor incidents including wound
infection and bleeding to rare incidents such as CCF formation. A CCF usually occurs weeks following insertion. It is thought that since the insertion of
the PEG tube is to some extent blind, it is placed occasionally through the
bowel before ending in the stomach. Over days to weeks, the balloon or bumper becomes dislodged and settles into the bowel, which is the transverse colon in the majority of cases. In a recent case series and review of literature
that included 28 cases, a majority of the cases involved some sort of tube replacement or revision. Interestingly, in our case, the PEG tube was never
modified, and the patient’s symptoms soon developed following placement
suggesting early migration rather than late.
90
KERNICTERUS AS A COMPLICATION OF
HYPERBILIRUBINEMIA IN ADULT PATIENT
Mohamed A, Aly M, Mazek H, Fenire M, Ali E. Texas Tech University
Health Science Center, Lubbock, TX.
Purpose of Study: This case highlights an extremely rare condition of reversible bilirubin neurotoxicity in an adult with sickle disease and Q fever induced biliary stasis. Our case is clinically interesting as it shows one of the
highest recorded bilirubin levels in a patient with sickle cell disease that
can lead to a condition considered extremely rare in adults, kernicterus.
The clinical picture can include myoclonus that could be easily misinterpreted as asterixis due to hepatic encephalopathy.
Methods Used: Case analysis and review of literature.
Summary of Results: The patient is 48 years old male with past medical history of sickle cell disease presented with abdominal pain, severe jaundice,
spiking fever, fatigue and myoclonus. He has a history of recent travel to
china and prolonged Doxycycline use. He denies any nausea, vomiting or
change in the color of the stool. On examination, the patient is icteric with
epigastric tenderness. Labs reveal a bilirubin level 77.5 (mainly indirect),
normocytic normochromic anemia with elevated reticulocyte count. Liver
enzymes including alkaline phosphatase were mildly elevated. Q fever Immunoglobulin M was positive. Abdominal imaging was suggestive of biliary
stasis with no evidence of intrahepatic or extra hepatic ducts dilation. The patient developed myoclonus that was reversed with the reduction of serum
bilirubin.
Conclusions: Bilirubin neurotoxicity is related to the free unconjugated bilirubin that can cross blood brain barrier. Early bilirubin neurotoxicity can be
reversible before permanent damage occurs from precipitation of bilirubin
(kernicterus). Kernicterus is known to happen in children and extremely rare
in adults; however in extremely high bilirubin levels, adult blood brain barrier can fail, our case being an example. Reversibility of neurotoxicity can
happen with prompt decrease in bilirubin. Myoclonic movement disorder is
one of the manifestation of bilirubin neurotoxicity which should be differentiated from negative myoclonus (Asterixis). Clinicians should be aware of
myoclonus as a feature of bilirubin neurotoxicity and not misinterpret it as
asterixis, as management of both conditions are different.
91
TAKOTSUBO CARDIOMYOPATHY ASSOCIATED WITH
HYPERTHYROIDISM TREATED WITH THYROIDECTOMY
Omar S, Soontrapa S, Ali E, Mazek H, Zedan A, Suarez J. Texas Tech University Health Science center, Lubbock, TX.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: Takotsubo cardiomayopathy is an uncommon clinical entity, also called apical ballooning syndrome, characterized by transient systolic dysfunction of the apical and/or mid segments of the left ventricle.
This syndrome mimics myocardial infarction in the absence of obstructive
coronary artery disease. The exact mechanism of the wall motion abnormalities is unknown. but it was thought to be related with hyperstimulation of
endogenous catecholamines inducing myocardial dysfunction and stunning.
It is usually associated with emotional and physical stress. Thyrotoxicosis
is a rare cause of takotsubo cardiomyopathy related to its direct and indirect
effect on the heart.
Methods Used: Case analysis and literature review.
Summary of Results: We are reporting a 61 year old female with unknown
past medical history , who was admitted with shortness of breath and palpitation and subsequently found to have atrial fibrillation with rapid ventricular
response secondary to hyperthyroidism. She was complaining of shortness of
breath but the patient denied any chest pain. The electrocardiogarm showed
atrial fibrillation with non specific ST-T wave changes and elevated troponin
T. Due to moderately decreased left ventricular systolic function seen on
echocardiography, coronary angiogram was done; however, it did not show
any coronary artery stenosis, but large apical ballooning with an ejection
fraction of 30 %. Propylthiouracil and corticosteroids were added to treat
thyrotoxicosis but the hyperthyroid state was difficult to control, finally decision was made to proceed with thyroidectomy to control the symptoms. Five
days later the patient’s clinical improved significantly.
Conclusions: we are reporting a rare case of takotsubo syndrome in the setting of thyrotoxicosis, which was resistant to supportive and medical management, and required thyroidectomy. The association of takotsubo
syndrome and hyperthyroidism has been reported before. Awareness of this
possible association is of importance in establishing the diagnosis and instituting a proper medical or surgical treatment plan.
92
DOCETAXEL ASSOCIATED EPIPHORA
Orellana-Barrios MA, Payne J, Sotello D, Riaz J, Cobos E. Texas Tech University Health Science Center, Lubbock, TX.
Case Report: A 59 year old female presented to the ER with bilateral eye
edema, onset approximately 24 hours before presentation. She was diagnosed with Stage 4 intraductal carcinoma with multiple bone metastases.
Her breast cancer was ER positive (99%), PR negative, HER2/neu negative,
Ki67 high at 60%. She received radiotherapy for painful bilateral hip bone
metastasis, and was placed on monthly denosumab, daily anastrozole, and
docetaxel every three weeks. Her last docetaxel dose was 24 hours before
her eye symptoms began.
On admission, her vital signs were normal and eye exam revealed
epiphora, moderate to severe bipalpebral bilateral eyelid edema, normal
color conjunctiva, normal eye movements, direct and indirect pupillary light
reflexes were normal and she complained of slight blurred vision. The rest of
her physical exam was normal. Also, her basic metabolic panel and hematology were within normal limits. She was diagnosed with Docetaxel induced
epiphora and eyelid edema, and was placed on intravenous antihistamines,
corticosteroids and opthalmic moxifloxacin and ocular lubricant. Ophthalmologic evaluation for lacrimal outflow passages obstruction was negative,
Southern Regional Meeting Abstracts
although the patient clinically continued with epiphora and eyelid edema.
Her edema improved and she was discharged on day 3.
Doxetacel is a commonly used taxane with indication for prostate, breast
and non-small cell carcinoma. Standard treatment dose of 60-100 mg/m2 every
3 weeks. Infrequently the Doxetacel is administered in weekly doses and a
smaller dose of 20-40mg/m2. Common side effects include myelosuppression
and leukopenia as well as asthenia and peripheral neuropathy. In this case we
present a photo of epiphora (excessive tearing due to narrowing of the lacrimal
duct). This adverse effect severely hampers quality of life for patients and if not
treated quickly can be permanently detrimental.
93
AN UNUSUAL CASE OF CARDIAC
ARREST - CARDIOLOGISTS BEWARE
Panikkath D, Panikkath R, Aly M, Jumper C. Texas Tech University of
Health Sciences, Lubbock, TX.
Introduction: Coronary artery disease is undoubtedly the most common
cause of out of hospital sudden cardiac arrest (SCA) accounting for more
than 90% of cases. However, uncommonly SCA can be due to other reasons,
and might have disastrous consequences with empirical treatment directed at
coronary artery disease.
Case Report: A 28 year old man without any significant past medical illness
except chronic pain was found unresponsive without pulse, one day morning.
Family members started cardiopulmonary resuscitation (CPR) and called
911. The emergency medical response team found asystole. He regained
pulse after 10 minutes of CPR. Advanced airway was secured and therapeutic hypothermia initiated. Cardiology was consulted by the emergency room
physician for T wave inversions in lateral leads and gross elevation of cardiac
troponins. Cardiology team was planning to start anti platelets and low molecular weight heparin for possible ACS as a cause for SCA. However, on detailed history, his wife reported that he had mild headache prior to going to
bed, the previous night. This prompted a computerized tomography scan of
the head, which showed diffuse subarachnoid hemorrhage (SAH). Patient
remained unresponsive without brain stem reflexes. A brain scan later confirmed brain death. The relatives chose to withdraw care and patient expired.
Conclusion: Spontaneous subarachnoid hemorrhage is an unusual cause of
cardiac arrest and has an extremely poor prognosis due to the combined damage to the brain due to SAH and ischemic insult due to cardiac arrest. Sympathetic stimulation and release of catecholamines could be the reason for
ventricular arrhythmias and cardiac arrest. ECG changes in patients with
SAH may be pronounced and might mimic myocardial infarction or ischemia. This might lead to delayed recognition/ non recognition of the primary
problem. SAH as a cause of cardiac arrest needs to be recognized, as treatment with anti-platelets and low molecular weight heparin (for suspected
ACS leading to cardiac arrest) can lead to disastrous consequences in such
patients. Although severe headache prior to the arrest is the clue to the diagnosis, it is well known that almost half of these patients may develop sudden
collapse without any prodromal symptoms.
94
AN UNUSUAL CASE OF PERICARDITIS
Sotello D, Rivas M, Nugent K, Mckenzie P, Temple B. Texas Tech University
Health Sciences Center, Lubbock, TX.
Case Report: A 27-year-old African American man without a past medical
history was admitted with 1 day of continuous substernal chest pain which
radiated to the back, was relieved by leaning forward, and was associated
with fever, dyspnea on exertion, and orthopnea. Vital signs: BP-109/78
mmHg, HR-131 beats per minute, RR-24 per minute, temperature-96.5 °F.
Physical examination: mild respiratory distress, lungs clear to auscultation, tachycardic, no murmurs, muffled cardiac sounds, no JVD, diffuse
abdominal tenderness, and no peripheral edema. Initial laboratory:
WBC-10.6 x103/mm3, Hb-13.9 g/dl, Platelets-131 x103/mm3, electrolytes
within normal limits, creatinine 2.9 mg/dl, BUN 30 mg/dl, and UDS positive
for marijuana. ECG: Sinus tachycardia and diffuse ST elevation. Transthoracic echocardiogram (TTE): EF 20-24%, large pericardial effusion with
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
tamponade physiology. Blood cultures were positive for Streptococcus
pneumoniae. Eventually the patient developed cardiac tamponade and underwent pericardiocentesis with pericardial window removing 400 ml of purulent fluid which also grew Streptococcus pneumoniae. The patient
completed 6 weeks of ampicillin/sulbactam, and his kidney function returned
to normal. The patient returned 3 weeks after discharge with restrictive pericarditis requiring anterior pericardiectomy.
Discussion: Streptococcal pericarditis (SP) is an uncommon diagnosis. It
generally presents with acute cardiovascular decompensation and signs of
sepsis. Its incidence has declined with the introduction of antibiotics and
with the implementation of pneumococcal vaccine. SP usually occurs secondary to hematogenous spread due to concomitant infection, such as pneumonia (93%), osteomyelitis, otitis media, mediastinitis, impetigo, meningitis,
etc. Primary streptococcal pericarditis is a very rare diagnosis, usually
occurring the setting of immunosuppression (hypogammaglobulinemia, alcoholism, and diabetes). The occurrence of primary SP in previously healthy
patients is very unusual. Diagnosis is suspected with clinical findings, including TTE and ECG, and ultimately confirmed with pericardiocentesis
and fluid analysis. Treatment is with intravenous antibiotics (penicillins)
for at least 4 weeks and surgery when indicated.
95
A CASE OF SUBSEROSAL EOSINOPHILIC
GASTROENTERITIS PRESENTING WITH ABDOMINAL PAIN
AND DISTENSION
Spera M, Gupta M, Engel LS, Nicaud M, Raines D. LSU Health Sciences
Center, New Orleans, LA.
Case Report: Eosinophilic gastroenteritis is a rare condition that presents
with varying gastrointestinal complaints depending on layer of involvement;
mucosal, muscular, or subserosal.
Case: A 27 year old Mexican woman presented with three weeks of intermittent supra umbilical abdominal pain and progressively worsening abdominal
distension. On physical examination the patient was noted to have abdominal
distension with shifting dullness but was non tender to palpation. The CBC
with manual differential showed a WBC of 19.4 x103/ul with 39% eosinophils. Computed tomography of her abdomen and pelvis with contrast
showed diffuse mural thickening and edema of the small bowel with a large
volume of free mesenteric and intra peritoneal fluid. Upper double balloon
enteroscopy revealed diffuse congested mucosa in the third and fourth part
of the duodenum. Patchy congestion was found throughout the jejunum.
Pathologic exam of biopsies taken from the stomach, duodenum and jejunum
showed increased lamina propria and mildly increased epithelial eosinophilia
with an epithelial eosinophil count of 14 per high power field (range of 0-14)
in the duodenum and jejunum. Cytology was negative for malignant cells.
Ultrasound guided paracentesis was performed for diagnostic purposes.
The serum to ascites albumin gradient was 0.5 g/dL. The ascitic fluid was
amber and clear with a WBC count of 9960/ul with the differential of 76%
eosinophils, 19% mesothelial cells, 3% monocytes and 2 % lymphocytes.
The ascites fluid adenosine deaminase level was unremarkable. The patient
was treated with an empiric course of albendazole for possible underlying intestinal parasite infection prior to the initiation of oral steroids as a treatment
for serosal eosinophilic gastroenteritis.
Discussion: Patient with serosal eosinophilic gastroenteritis typically present
with eosinophilic ascites with abdominal bloating and a high peripheral eosinophilia. Diagnosis is made with endoscopic biopsy of the affected area
which usually shows greater than 20 eosinophils per high power field. When
endoscopic biopsy is non diagnostic, full thickness surgical biopsy should be
undertaken.
96
SMALL CELL CARCINOMA IS NOT EXCLUSIVELY
A LUNG CANCER
Suvorava N1, Konala V1,2, Aulakh A1,2, Tijani L1,2. 1TTUHSC, Lubbock,
Lubbock, TX and 2TTUHSC, Lubbock, TX.
Case Report: Extra pulmonary small cell carcinoma (EPSCC) represents
about 2-5% of all small cell carcinomas (SCC). It’s a rare malignancy with
aggressive course, frequent recurrences and early metastases. EPSCC can
436
affect any site within the body except the central nervous system. We present
a patient with SCC of lymph nodes.
A 62-year-old male presents with rapidly growing painless left sided axillary mass noticed about a year ago. It was rapidly increasing in size over 8 weeks
with no associated symptoms. His past medical history was significant for coronary artery disease and 15-pack-year smoking history. He quit smoking
21 years ago. On physical exam, a firm and non-tender mass was noted over
left axilla, measuring approximately 10cmx5cm and a palpable left
supraclavicular node with left arm edema. CT chest revealed lobulated mass
at the left axilla, measuring 11.5cm in maximal dimension and multiple clustered left supraclavicular lymph nodes. Fine needle aspiration of the mass
revealed a SCC. The PET/CT scan revealed extremely hypermetabolic bulky
supraclavicular, retroclavicular lymphadenopathy as well as left axillary mass.
The patient underwent concurrent chemo-radiation with four cycles of
cisplatin and etoposide along with external beam radiation for a total of 35
treatments. Repeat PET/CT scan after completion of treatment showed resolution of the left supraclavicular lymphadenopathy with marked reduction in
the left axillary adenopathy.
Early recognition is important since limited stage disease has better outcome. Survival with EPSCC is variable, ranging from 5 to 50 months for limited
stage disease and 5-12 months for extensive stage disease. Multi-modality therapy using surgery, chemotherapy, and radiation is essential for cure in patients
with limited stage of disease.
This case highlights that SCC is not an exclusively lung malignancy and
could be found in sites such as genitourinary tract, gastrointestinal organs,
nasal cavity, head and neck, parotid gland, pancreas, lymph nodes.
Depending on primary site the prognosis may be variable, with SCC localized to lymph nodes carrying a better prognosis. The limited number of
reported cases makes this type of tumors challenging to deal with and more
clinical trials are needed to determine the effective treatment options.
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SPINAL METASTASIS AS INITIAL PRESENTATION OF
HEPATOCELLULAR CARCINOMA IN A PATIENT WITH
METASTATIC CARCINOID TUMOR
Suvorava N1, Konala V1,2, Aulakh A1,2, Tijani L1,2, Warraich I3. 1TTUHSC,
Lubbock, Lubbock, TX; 2TTUHSC, Lubbock, TX and 3TTUHSC, Lubbock, TX.
Case Report:
Introduction: Spinal metastasis can occur both in Carcinoid tumor and Hepatocellular carcinoma (HCC) with low incidence compared to other solid
tumors. We present a patient with worsening abdominal, neck and back pain
with metastatic neuroendocrine carcinoma of the intestine.
Case Presentation: 60 year old male with past medical history of Hepatitis
C with liver cirrhosis and neuroendocrine carcinoma of the intestine with
metastasis to liver diagnosed in 2011, treated with octreotide presented with
worsening right sided abdominal pain, neck and back pain in spite of being
on methadone and hydrocodone as needed for pain. He had 30 pack year
smoking history, uses marijuana daily. Physical exam was significant for tenderness on palpation of the right upper quadrant.
CT scan of abdomen and pelvis showed osseous lytic lesions thoracic and
lumbar spine with pathological fracture of ninth thoracic vertebrae. It also
showed both carcinoid tumor at the primary site and liver metastases were
stable compared to previous imaging. Subsequent MRI of the spine confirms
multiple lytic lesions in cervical, thoracic and lumbar spine. Ultrasound
guided biopsy of the cervical bone lesions revealed metastatic HCC. Alpha-Feto protein was mildly elevated at 17.7 International units/ml. Patient
received symptomatic treatment with analgesics, pamidronate and was
started on palliative radiation treatment to the spine.
Discussion: Carcinoid tumors metastasize to lymph nodes, liver, lungs. Skeletal metastases are becoming more prevalent due to longer survival and
advances in earlier detection. Incidence of skeletal metastases is approximately 10%.
HCC commonly metastasizes to lungs, followed by the lymph nodes and
bone. The incidence of bone lesions in HCC ranges from 1% to 20% in
autopsied cases.
Given our patient has liver cirrhosis secondary to hepatitis C and stable
metastatic carcinoid tumor on octeotride, new onset symptoms alerted to further investigate the cause of spinal metastasis. High index of suspicion is
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
required when a patient presents with spinal metastasis in the presence of primary tumor to suspect a second tumor in the presence of risk factors.
98
PASTEURELLA MULTOCIDA CELLULITIS OF THE KNEE IN
A PATIENT WITH RECENT TOTAL KNEE ARTHROPLASTY
Suvorava N1, Dobronski L1, Mazek H1, Nichols J1, Winn R1,2. 1TTUHSC,
Lubbock, TX and 2TTUHSC, Lubbock, TX.
Case Report: Pasteurella multocida is part of the oral flora of wild and domestic animals, known to cause infection after animal bites. Infection may
occur without physical contact or from contact with animal secretions. We
present a case of P. multocida cellulitis after a dog lick.
A 59-year-old female developed fever, hypotension, vomiting, leg pain,
redness and swelling over 9 hours. Heart disease, chronic kidney disease,
obesity, and osteoarthritis with bilateral knee replacements were reported.
She had 2 dogs and allowed them to lick her legs. The right knee was erythematous, edematous, hot and tender to palpation with limited ROM due
to severe pain. Purulent drainage was noted from one of the previous surgical
incision sites on the leg. Leukocyte count and inflammatory markers were elevated. X-rays showed no abnormalities of the prosthetic joint; moderate
suprapatellar joint effusion and edema of subcutaneous tissues of the knee
were present.
ICU admission with administration of vasopressors, fluids, levofloxacin,
piperacillin-tazobactam and vancomycin was necessitated. Joint aspiration
was declined due to overlying cellulitis. A culture from the wound revealed
P. multocida. Blood cultures were negative. She improved and Infection
was eradicated with continued outpatient antibiotics.
P. multocida cellulitis is characterized by rapid inflammatory signs within
hours. Septic arthritis occurs in up to 6%. For prosthetic joint infection antibiotic therapy without removal of an infected TKA may be appropriate for
acute infection, when the prosthesis is not loose and the infection is treated
early. For wound infections due to P. multocida antimicrobics such as penicillin, amoxicillin/clavulanate or doxycycline can be used or, as alternative,
second and third generation cephalosporins or aztreonam can be considered.
Susceptibility testing is recommended.
P. multocida infection/disease may result in severe morbidity or death. History of animal exposures/bites particularly with coexisting immunocompromise, artificial valves or arthroplasties may be of critical importance.
Direct injection of Pasteurella through bites or scratches clearly can lead to infection as can exposure to saliva from licking of open wounds by pets.
99
AN UNUSUAL CASE OF PCP PNEUMONIA IN
A NON-HIV PATIENT
Tariq K, Landa C. University of Florida College of Medicine, Jacksonville, FL.
Case Report: A previously healthy 38 year old Caucasian male was admitted to the Intensive Care Unit for evaluation and treatment of acute respiratory failure and septic shock. He was found to have bilateral, multilobar
infiltrates on CXR but initial respiratory gram stain and culture failed to reveal a causative agent. Blood cultures and urinalysis with culture were negative. Eventually a PCP smear showed Pneumocystis jiroveci. This
discovery led to further investigation for an immunocompromised state.
The patient did not have any risk factors per history. The work up included
two negative HIV tests, including DNA PCR. A hepatitis panel was positive
for Hepatitis C antibodies but RNA PCR was not detectable. Finally, the CD
4 T lymphocyte count returned at 73. The patient had a difficult hospital
course requiring prolonged ventilator support and the use of pressors, complicated by bilateral pleural effusions requiring chest tube placement. After
an extensive course, the patient fully recovered and was discharged home
on appropriate medications.
HIV infection has become synonymous with immunosuppression and a
low CD4+ T lymphocyte count. However, it is not the only cause of reduced
levels. Idiopathic CD4+ T cell Lymphocytopenia (ICL) is a very rare syndrome that mirrors HIV/AIDs in many ways. The criteria for ICL was first
established by the CDC in 1993, which includes a CD4 count less than
300 cells per microliter, or less than 20% of T lymphocytes are CD4+, lack
of laboratory evidence of HIV infection and absence of any alternative
Southern Regional Meeting Abstracts
explanation for the CD4 T cell lymphocytopenia. The most common opportunistic infections associated with ICL are Cryptococcus Neoformans, nonTuberculous Mycobacteria, Progressive Multifocal Leukoencephalopathy,
Kaposi Sarcoma and the genital Human Papilloma Virus. Pneumocystis
pneumonia is a very uncommon infection in ICL. Our case emphasizes the
importance to consider ICL in HIV negative patients affected by AIDS defining opportunistic infections.
100
WHAT’S IN THE DIFFERENTIAL? A CASE OF
HYPEREOSINOPHILIC SYNDROME
MASQUERADING AS CEREBRAL VASCULITIS
Weeks A, Williams JC, Grover I, Elkins S. University of Mississippi Medical
Center, Jackson, MS.
Case Report: Peripheral blood eosinophilia is precipitated by reactive or
clonal processes including atopic and allergic diseases, helminthic parasite
infections, adverse drug reactions, and neoplasms. Classically a diagnosis
of exclusion, hypereosinophilic syndrome (HES) is a rare disorder defined
by persistent blood eosinophilia (≥ 1.5 x 10x9/L), eosinophil mediated
end-organ damage, and absence of a secondary cause. Here we report a case
of idiopathic HES manifesting as neurological dysfunction responsive to
prednisone.
FISH for CHIC2, 4q Deletion (FIP1L1 and PDGFRA Fusion); Translocation (5;12) (PDGFRB); 8p11.2 (FGFR1) performed at Mayo Medical Laboratory. Routine imaging, CSF, blood, and cytogenetic testing were
performed at our institution.
A 78 year old white female with history of diabetes mellitus, hypertension, COPD, and squamous cell skin cancer initially presented to an outside
hospital with a 3 day history of right upper and lower extremity weakness,
gait disturbance, confusion, and disorientation. Eleven days later, she was
transferred to our facility with the diagnosis of worsening cerebral vasculitis.
Physical exam was notable for right sided weakness, confusion, lethargy,
diplopia, and decreased alertness. Review of outside records revealed an absolute eosinophil count of 17.5 10x9/L. Medication profile uncovered no
drug related causes. CSF studies for multiple sclerosis, viral, bacterial, and
fungal etiologies were negative. Parasitic infection was negated by serum
and stool testing. MRI brain revealed multiple scattered infarcts suspicious
for vasculitis; however, subsequent cerebral angiogram was normal. Cytogenetic and FISH analysis from peripheral blood and bone marrow were normal. The patient was immediately started on weight based prednisone. One
week later, the patient’s mental status returned to baseline and she was
discharged with an eosinophil count of 5.8 x 10x9/L.
Both early recognition of HES and introduction of eosinophil-reducing
agents are vital to patient survival. This case highlights the importance of
careful history taking and thorough evaluation when reporting a diagnosis
of exclusion. Furthermore, it reinforces that clinical judgment combined with
available data trumps isolated imaging reports and previous diagnoses.
101
“THE MIKE TYSON CHALLENGE: AN EXTREME CASE OF
RHABDOMYOLYSIS”
Wright A, Spiegel J, Engel LS. LSU Health Sciences Center, New Orleans, LA.
Case Report:
Introduction: There are many causes of rhabdoymyolysis, including excessive
exercise. One of the most serious complications of rhabdoymyolysis remains
acute kidney injury (AKI), which is caused by non-protein heme pigment that
is released from myoglobin. Furthermore, inflammation of the muscle can compress structures in the same fascial compartment resulting in compartment
syndrome.
Case: A 24 year old inmate was brought to the emergency department with
severe thigh pain and dark colored urine after participating in “The Mike
Tyson Challenge” During this challenge he performed 372 squats over a
45 minute time interval. The thigh pain began the next morning followed
by “coca cola” colored urine. On physical exam his thighs were extremely tense
and he underwent emergent bilateral fasciotomy for compartment syndrome.
He was found to have a creatinine kinase of 401,880 U/L, BUN of 45 mg/dl,
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
437
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
serum creatinine 7.21 mg/dl, and potassium of 6.3 mmol/l. Urinalysis revealed
250 blood/ul but only 3-5 red blood cells/HPF. He received insulin, sodium
polystyrene sulfonate, IV fluid hydration, and hemodialysis. He slowly improved while receiving 3 weeks of hemodialysis and physical therapy in the
hospital.
Discussion: In his prime Iron Mike Tyson was the self proclaimed ‘baddest
man on the planet’. A workout based on his physical training was developed
and named the Mike Tyson Beast Workout or Challenge. Some rules of the
‘Beast Workout’ are as follows:1) The workout must be performed in no
more than one hour; 2) The aim is to perform dead lifts, bench press, squats,
chest press and dumbbell curls through-out the hour. Our patient barely survived the squats. Fortunately, aggressive surgical intervention, fluid hydration and hemodialysis were able to reverse the damage caused by the
release of myoglobin from injured muscle.
travel where she is stabilized. Imaging reveals a massive splenic infarct. Serial exams and labs along with repeat imaging exclude splenic sequestration
syndrome.
Discussion: As opposed to Hemoglobin SS disease in which patients almost
universally develop functional asplenia, in Hemoglobin SC disease the incidence of functional asplenia in persons older than twelve is approximately
45%. People with Hemoglobin SC disease and a functional spleen remain
at risk for splenic infarction throughout their life. Air travel and changes in
altitude are recognized factors associated with acute splenic sequestration
and splenic infarction for patients with Hemoglobin SC disease as well as
for those with sickle cell trait alone. Our patient, who described herself as
“never” having a pain crisis before, was at risk for experiencing an acute
splenic infarct or acute splenic sequestration with her travel to Bolivia. This
case highlights the importance of integrating knowledge about methods of
travel and the destination’s altitude into travel medicine.
102
ICE COLD IN MISSISSIPPI: A CASE OF NOSOCOMIAL
FROSTBITE
Yates R, Isaac J, Grover I. University of Mississippi Medical Center, Jackson, MS.
Case Report: Frostbite is severe, localized tissue injury induced by cold
temperatures. It varies in severity from first to fourth degree, fourth degree
being most severe with muscle and bone involvement. We present a case
of a young woman originally hospitalized for sepsis who developed severe
frostbite requiring amputation of her bilateral lower extremities. A 39-yearold African American female with uncontrolled diabetes and neuropathy
presented with sepsis secondary to pyelonephritis. She was admitted to the
intensive care unit due to hypotension and started on norepinephrine and
broad spectrum antibiotics. She improved and was transferred to the general
medicine service. Two days later, we discovered new onset, sharply demarcated, hyperpigmented skin lesions with blistering in a stocking pattern on
her bilateral lower extremities. No other areas were involved. Initial differential included contact dermatitis or a drug reaction, but further questioning
revealed that the patient had retrieved a bucket of ice in which she soaked
her feet for about 30 minutes to relieve burning, neuropathic pain. Pedal
pulses were not palpable. Frostbite treatment protocol was initiated, including passive and active rewarming of the lower extremities. Vascular surgery
was consulted and recommended computed tomography angiography with
runoff for further evaluation. It confirmed lack of appropriate blood flow below the knees bilaterally. Heparin infusion was initiated. She was taken to the
operating room to attempt intra-arterial tissue plasminogen activator, but this
was unsuccessful. Due to imminent tissue necrosis and risk of infection, the
patient underwent bilateral below the knee amputations. Pathology confirmed severe frostbite as the etiology of her skin findings. While frostbite
is the most common cold injury, it is much more prevalent in northern
climates and in groups frequently exposed to cold temperatures such as
mountaineers. Only a handful of nosocomial cases are reported in the literature. In addition, diabetes and neuropathy are known risk factors for frostbite
injury, and this case demonstrates this well. Finally, this case illustrates that
frostbite should be included in the differential of dermatoses presenting in
the stocking-glove distribution.
102A
TRAVEL MEDICINE AND HEMOGLOBINOPATHIES
Henegan JC, Herrin V. University of Mississippi Medical Center, Jackson,
Mississippi.
Introduction: As opposed to Hemoglobin SS disease, patients with Hemoglobin SC disease may have a functional spleen. However, they remain at
risk for an acute splenic infarct. Knowledge of a destination’s altitude is an
important component of travel medicine.
Case Report: A 32 year-old female dentist with Hemoglobin SC disease
travels from Jackson, Mississippi, (elevation: 279 feet) to the capital of
Bolivia (elevation: 9,220 feet) for a weeklong medical missions trip. Within
an hour after landing in Bolivia she develops a dull abdominal ache that
progresses to a continuous, intense pain. She is stabilized with available
supplies that include intravenous hydration, analgesics, and supplemental oxygen. Her hematologist is contacted and recommends that the patient return
to the United States as soon as possible. Three days after initially arriving the
patient flies back to New Orleans, LA, where it has been arranged for her to
be evaluated soon after arrival. She presents to a hospital aware of her recent
438
Allergy, Immunology and Rheumatology
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
103
CORNEAL MELT SYNDROME: A COMMON
MANIFESTATION OF AUTO-IMMUNE DISORDERS
Bhawal J, Majithia V, McMurray R. UMMC, Jackson MS.
Case Report: Corneal melting is inflammatory condition of cornea involve
disruption of epithelial and corneal stroma which are protective layer of cornea, and cause byconnective tissue disorder like RA, lupus, AS and wegners
granulomatosis. it may be a rare complication of rheumatoid arthritis,
Sjögren’s syndrome and may progress rapidly within days lead to complete
blindness.
Cases: 1. An 84 y/o female with multiple PMH was evaluated due to positive
ANA, SS-A, negative R,F previous uveitis and corneal melt. She had cataract
surgery about 4 years ago and developed uveitis in right eye which lead to
enucleation of the eye due to a complicated course. she developed infection
in the left eye and perforation of the cornea leading to emergent corneal
transplant.She did report dry mouth and dry eyes for a couple of years and
raynaud’s. She was started on prednisone daily, and azathioprine 150 mg/day.
2. 79 y/o F with multiple medical problem including RA was, pt was
evaluated due to necrotizing scleritis of left eye,pt described onset like had
foreign body sensation in her Left eye started from About 4 weeks ago, subsequently effecting her vision and was prescribed prednisone eye drops, but
lher vision started getting worse and Saw ophthalmologist, subsequently biopsy and scleral patching of her Eye was done and was started on prednisone, patient started complaining of severe pain and complete loss of
vision in her left eye. She was started on solumedrol 125 mg IV daily and
quantiferon and hepatitis profile negative and started on rituximab infusion
every 2 weeks.
Discussion: Healthy cornea contains inactivators of proteinase and a nice
tear film, which protects it from damage. Any disorder, trigger or decreased
tearing leads to increased cytokines, tumor necrosis factor and interleukin-1
leading to activation of corneal proteinase, which cause keratolysis. A mild
case of keratitis can be treated with NSAIDS alone, but a moderate to severe
case requires treatment with methotrexate and prednisone. In the most severe
cases oral cyclophosphamide may be needed followed by azathioprine or cyclosporine for maintenance therapy. Rituximab, TNF blockers and other
biologics have been used successfully in some cases too.
104
A CASE OF DISSEMINATED MUCORMYCOSIS
Mains TB, Majithia V, McMurray R. University of Mississippi Medical Center, Jackson, MS.
Case Report: Case Report: Introduction: Mucormycosis is aggressive fungal infection, classically occurring in poorly controlled diabetics, or immunodeficient individuals. It may be possible that some medications used in
the treatment of rheumatic diseases may increase the risk of this infection.
Case Presentation: 48 y.o white female with Systemic Lupus Erythematous
(SLE) with severe arthritis presented to UMC after hospitalization at an OSH
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
for CVA and thrombocytopenia. She was taking Plaquenil 200mg bid, Methotrexate 20mg/wk but stopped anti-TNF therapy 2 months prior to her hospitalization due to financial constraints.
Initially she presented with dysarthria, left facial droop, and Right sided
weakness. Imaging was revealed a left cerebral infarct. CVA evaluation discovered a valvular vegetation on echo, left carotid stenosis, normal complements,
leukocytosis, thrombocytopenia, negative cultures and APLS panel. The thrombocytopenia diagnosed as Immune thrombocytopenia purpura (ITP) secondary
to her lupus after bone marrow and labs were not diagnostic. She was treated
with IVIg, followed by 4 weekly doses of Rituxan. Prior to her family transferring her to UMC, the patient developed a thigh abscess that required surgical
drainage and initiation of antibiotics.
At UMC she was found have SIRS with an acidosis and acute kidney injury, started on broad spectrum antibiotics and admitted to medicine. She
quickly decompensated had an episode of cardiac arrest. EKG after transfer
to the ICU revealed ST segment elevation. Cardiac catheterization revealed
likely coronary vasospasm in the LAD. Clinical status further deteriorated
and anoxic brain injury was suspected. Repeat imaging showed diffuse areas
of ischemia. After another episode of cardiac arrest she expired. Autopsy
revealed disseminated mucormycosis with fungal emboli.
Discussion: Mucormycosis is a fulminating, highly aggressive fungal infection, typically occurring in uncontrolled diabetics, or immunodeficient
individuals. Many patients with rheumatic diseases frequently require immunosuppressive medications which may increase the risk the risk atypical
infections such as mucormycosis.
105
“COMPARATORS OF PROTECTIVE FACTORS OF
BONE MINERAL DENSITY IN A NEW ORLEANS
SARCOIDOSIS POPULATION”
Walker M, Janot A, Yu M, Lammi MR, Saketkoo LA. LSU Health Sciences
Center, New Orleans, LA.
Purpose of Study: The pathophysiology of sarcoidosis involves dysregulation of dependent factors of bone metabolism, such as vitamin D and
calcium, and high dose persistent treatment with steroids may lead to low
bone mineral density (LBMD). LBMD in sarcoidosis is presumed but has
yet to be described.
Methods Used: A retrospective chart review of biopsy-proven patients with
a diagnosis of sarcoidosis for >1 year was used to compare parameters of
prevalence, age (at chart review), gender, smoking status and designation
of LBMD based results of Dual Energy Xray Absorptiometry (DEXA) studies. All calculations are based on non-parametric analyses using Fisher’s exact for categorical data and Mann Whitney tests for continuous variables.
Summary of Results: 61 patients (86.9% African-American) were identified
that met criteria, 38 (62.3%) with LBMD (30 with osteopenia and 8 with osteoporosis). A significant difference was found in occurrence of LBMD in
patients with BMI > vs <30 with patients BMI > 30 having lower levels of
LBMD (as is expected in the general population), but no significant differences
(table) were found in occurrence of LBMD in patients age > vs < 65, in ever vs
never smokers (age between ever smokers and never smokers was not significantly different), or in males vs females (with females being significantly older
than males and female age range 34-79).
Conclusions: Factors protective against LBMD in the general population
were not demonstrated in this population of sarcoidosis patients. A lower risk
of LBMD was not conferred by age < 65, male gender, or non-smoking status. These trends including significantly older age of females vs males
suggests an abnormal distribution of LBMD in our sarcoidosis population
that is not expected in the general population. Further examination of LBMD
in sarcoidosis may yield evidence to support increased vigilance in steroid
use and perhaps consideration/consensus to initiate steroid sparing agents
earlier in the disease course.
106
GENDER INFLUENCES ON ORGAN MANIFESTATIONS IN
A NEW ORLEANS SARCOIDOSIS POPULATION
Walker M1, Janot A1,2, Grewal H1, Yu M1, Lammi MR1, Saketkoo LA1.
1
LSU Health Sciences center, New Orleans, LA and 2Medical of Virgina,
Richmond, VA.
Southern Regional Meeting Abstracts
Purpose of Study: Sarcoidosis is a multi-organ disease of unknown etiology
characterized by granuloma formation and fibrosis in affected organs. Prior studies
have shown a gender association with organ specific manifestations. Male gender
is associated with a more severe radiographic picture and female gender with
more frequent extra-pulmonary manifestations (EPM). We conducted a survey
of gender-based differences of organ-specific manifestations of sarcoidosis in a
New Orleans cohort from a tertiary care institution cohort.
Methods Used: A retrospective chart review on patients seen in our clinics
from 2006-2012 with biopsy proven sarcoidosis and a diagnosis >1 year
were included. Data was collected on gender, smoking status, presence of
EPMs (cutaneous, ocular, cardiac, neurologic, GI, and renal), chest
radiographs and pulmonary function testing (PFT). Differences in gender
and presence of EPMs and pulmonary sarcoidosis were calculated. Gender
differences in FVC, TLC, and DLCO were calculated using a t-test for the
mean. PFTs were stratified by time of diagnosis in five year intervals.
Summary of Results: Of 511 charts reviewed, 156 pts met inclusion criteria.
Males had ocular sarcoidosis (OS) more frequently (M=0.28, F=0.104,
p=0.005) and relative risk was calculated to be 2.24 (p<0.05, 95% CI 1.09
to 4.63). Gender was not a significant risk factor for other EPMs, nor for
all EPMs combined. However, N limited statistical analysis of an apparent
trend in the presence of GI/splenic sarcoid disease favoring females
(F=11%, M=6%). The presence of pulmonary sarcoidosis was not associated
with gender (p=0.3). See table 1. PFTs showed significant gender difference
in that males had worse FVC (M=69.0, F=82.2, p=0.030) and TLC (M=63.0,
F=78.86, p=0.003) 5-10 years after diagnosis. Diffusing capacity also
showed gender specific differences with females having a worse DLCO than
males 10-15 years after diagnosis. (M=72.0, F=55.33, p= 0.024).
Conclusions: Male sex was a risk factor for ocular sarcoidosis. Though gender was not associated with radiographic sarcoidosis, when controlled for
smoking, increased severity of restrictive lung disease was demonstrated in
males. Differences in DLCO warrant investigation for risk of pulmonary vascular disease in females.
Cardiovascular
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
107
FUNCTIONAL AND DYSFUNCTIONAL HDL IN HEART
TRANSPLANT: THE IMPORTANCE OF CHANGES POST
TRANSPLANT
González W1, Altieri PI1,2, Banchs HL1,2, Iravedra D1, González-Cancel IF2,
Calderón RE1,2, Gurrea C2, Escobales N1, Crespo M1. 1University of Puerto
Rico, San Juan and 2Cardiovascular Center of Puerto Rico and the Caribbean, San Juan.
Purpose of Study: To study the metabolic changes post heart transplant.
Methods Used: 101 Patients (P.) with heart transplant were studied. 65 P.
were males and 36 were female, with a mean age of 51 years.
Summary of Results: Pre and post metabolic changes were:
1. BMI (25 ± 4-28 ± Kg/m2 P < 0.05).
2. Systolic blood pressure (107 ± 13-131 ± 20 P <0.05).
3. Diastolic blood pressure (70 ± 13-81 ± 10 mmHg).
4. Fasting blood sugar (107 ± 37-117 ± 55 mg%) N.S.
5. Total cholesterol (170 ± 55-189 ± 32mg/dl) P < 0.05.
6. Total HDL (38 ± 16-52 ± 17mg/dl) P < 0.05.
7. Total LDL (99 ± 20-83 ± 15mg/dl) N.S.
8. Triglycerides (163 ± 10-188 ± 12mg/dl) N.S.
Conclusions: Our data shows that all the P. developed Mets. The HDL of
the whole groups increased from 38 mg/dl to 52 mg/dl. These changes are
not explained only by the immunosuppressive treatment, HDL has a role
as an anti-inflammatory agent which is inherent in the lipoprotein. This will
also increase the HDL. 16 P. died of rejection. The HDL of the rejected P. increased from 47 ± 22 to 71 ± 40 mg/dl P. < 0.007. This increase persisted
through the course of the rejection period. The mean age of the transplanted
heart was 22 years. The rejection occurred in less than 5 years. (3.5 years).
Only 7 autopsies were done. 5 of them showed severe atherosclerotic
changes in the coronaries and aorta. This is probably explained on basis of
dysfunctional HDL. Probably the high levels and persistent elevation of
HDL in the rejected group can be used as a marker to predict rejection.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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108
implemented to avoid further deleterious effects caused by risk factors and
subsequent disease complications.
AUGMENTATION INDEX IS ASSOCIATED WITH RENAL
FUNCTION IN BLACK BUT NOT IN WHITE YOUNG ADULTS:
THE BOGALUSA HEART STUDY
110
Fernandez C, Li S, Chen W, Srinivasan SR, Berenson GS. Tulane University,
New Orleans, LA.
Purpose of Study: Increased Augmentation Index (AI) has been associated
with kidney damage in patients with hypertension and type 2 diabetes
mellitus. However, information is scant on the race-specific (black-white) association between AI and renal function/performance in asymptomatic
young adults.
Methods Used: As a part of the Bogalusa Heart Study, a long term community-based study, 884 non-institutionalized participants with a mean age of
43.5 years (29.4-51.3years; 68.3% white and 41.9% male) were considered.
AI was estimated non-invasively by applanation tonometry of the radial artery. eGFR was calculated using the MDRD study equation. Race-specific
independent association of AI with eGFR was tested through multivariable
adjusted linear regression analyses.
Summary of Results: Black vs white participants had higher AI and eGFR
levels (p<0.01). As regards gender differences, black females had significantly higher levels of AI and eGFR compared to black males (p<0.01). In
multivariable adjusted linear regression analyses, controlling for age, sex
and traditional cardiovascular risk factors, AI was significantly and directly
associated with eGFR in blacks (β=1.12, p<0.001), but not in whites
(β=0.039, p=0.34)
Conclusions: These findings support the hypothesis that underlying
mechanisms for cardiovascular disease related-end organ damage differ
among blacks vs whites. Further, this may aid to focus on race-specific
approaches to prevention and screening of cardio-metabolic risk factors,
diseases and associated complications.
109
LIFESPAN PREDICTORS OF CARDIOVASCULAR
MORBIDITY IN INDIVIDUALS REACHING MIDDLE AGE:
THE BOGALUSA HEART STUDY
Fernandez C, Li S, Chen W, Srinivasan SR, Berenson GS. Tulane University,
New Orleans, LA.
Purpose of Study: Considerable information is available on the clustering of
cardiovascular (CV) risk factors across the lifespan and their impact on cardiovascular disease (CVD) morbidity and mortality. In spite of this, information is scarce on the individual contribution of CV risk factors measured
longitudinally from childhood to adulthood, as well as their cumulative burden, on CVD morbidity.
Methods Used: The longitudinal study cohort comprised 118 individuals
aged 25-46 years (mean age 34.2 years); 44.1% black and 63.6% female,
who were residing in the semi-rural community of Bogalusa, Louisiana
and participated in the study since childhood. These participants had at least
3 traditional CV risk factors measurements since childhood and self-reported
information on adult CVD morbidity. Mean follow up period was 14.3 years.
CVD was defined as positive personal history of one or more of the following: angina, percutaneous coronary intervention, coronary artery bypass
grafting, myocardial infarction and/or stroke. The area under the curve
(AUC) was used to assess the cumulative burden of risk factors.
Summary of Results: After accounting for the effect of age, race, sex, cigarette smoking and family history of CVD, significant predictors of CVD
were: childhood body mass index (BMI) (Odds Ratio [OR], 1.36; 95% Confidence Interval [95% CI] 1.12-1.62) and non-high density lipoprotein cholesterol (nonHDL-C) (OR, 1.22; 95% CI, 1.15-1.73); adulthood BMI (OR,
1.13; 95%CI, 1.02-1.24), Systolic Blood Pressure (SBP) (OR, 1.32; 95%
CI, 1.07-1.65) and nonHDL-C (OR, 1.39; 95%CI, 1.11-1.56); and long-term
cumulative burden of BMI (OR, 1.29; 95%CI, 1.21-1.37) and nonHDL-C
(OR, 1.47; 95%CI, 1.38-1.71).
Conclusions: The observed adverse associations of childhood/adulthood
cardiovascular risk factors and their long-term cumulative burden on adult
CVD morbidity, are indicative of the individual impact of these risk factors
in the development of heart disease. Hence, risk factor-oriented interventions (through primordial prevention early in life) must continue to be
440
TOBACCO SMOKING STRENGTHENS THE ASSOCIATION
BETWEEN HIGH BLOOD PRESSURE AND ARTERIAL WALL
STIFFNESS: THE BOGALUSA HEART STUDY
Yun M1,2, Ge S1, Li S1, Fernandez C1, Chen W1, Srinivasan SR1,
Berenson GS1. 1Tulane Center for Cardiovascular Health and Department
of Epidemiology, Tulane University, New Orleans, LA and 2College of Life
and Environment Sciences, Minzu University, Beijing, China.
Purpose of Study: The synergistic effect of tobacco smoking and elevated
blood pressure (BP) is not well known, especially for the long-term burden
of elevated BP. This study assessed the hypothesis that smoking strengthens
the association between long-term cumulative burden of BP since childhood
and adult arterial stiffness.
Methods Used: The study cohort consisted of 945 adults (661 whites and
284 blacks, aged 24-43 years) who have BP measured 4-15 times since childhood (aged 4-16 years) in Bogalusa, LA since 1973. The adult arterial stiffness was measured as aorta-femoral pulse wave velocity (PWV); the total
area under the curve (AUC) and incremental AUC were used as a measure
of long-term burden and trends of BP, respectively.
Summary of Results: Higher systolic BP values of childhood (p=0.009),
adulthood (p<0.001), total AUC (p<0.001) and incremental AUC (p<0.001)
were all significantly associated with increased adult PWV, after adjusting for
age, race, gender, body mass index and heart rate; diastolic BP showed similar
patterns of association with PWV. Furthermore, years of smoking was a significant predictor of PWV (p=0.027). In the interaction analyses, the increasing
trend of PWV levels with increasing adult BP (p=0.003, see the figure below),
total AUC (p=0.010) and incremental AUC (p=0.049) among smokers was significantly greater than that among nonsmokers.
Conclusions: These results suggest that the adverse influence of BP levels
on arterial stiffness begins in childhood, and tobacco smoking accelerates
this process, indicating the importance of preventive strategies in early life
involving healthy lifestyles.
The Relationship of Adult Systolic Blood Pressure (SBP) to Adult Pulse
Wave Velocity (PWV) by Smoking Status
111
THE UTILITY OF SERUM TROPONIN IN PATIENTS
ADMITTED WITH SYNCOPE
Bhusri S, Pashkovetsky E, Bierzynski A, Armstrong DF, Daneshvar D,
Panagopoulos G, Coplan NL. Lenox Hill Hospital, New York, NY.
Purpose of Study: The objective of this study is to evaluate the immediate
clinical utility of measuring serum troponin in patients presenting with syncope.
Methods Used: Data from 200 consecutive admissions with an admitting diagnosis of syncope were collected from the Emergency Department. Each
admission was categorized based on initial serum troponin (negative
<0.015 ng/mL), age, sex, gender, cardiac risk factors, and ejection fraction
to assess the prevalence of adverse cardiac outcomes. Exclusion criteria
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
included significant ST changes and chest pain as part of the initial presentation.
Statistical analysis was performed using the Mann–Whitney Test, Chi-Square,
and T-test.
Summary of Results: After excluding patients that otherwise required a serum troponin check (i.e. chest pain and/or significant ST changes), levels
were obtained in the remaining 178 patients. Of these patients, 28 patients
(15.73%) had a positive troponin (>0.015). Troponin positive patients were
all male (p<0.01) and had a significant past history of arrhythmia (p=0.007).
In contrast, troponin negative patients were mostly female (p<0.01).
Outcomes data suggest a longer length of stay (<0.01) in the troponin positive group without any significant differences in ejection fraction, arrhythmia
detected and whether the patient was on a monitored unit.
Conclusions: Measurement of serum troponin levels has no significant impact on evaluation in patients with syncope.
112
CATION DYSHOMEOSTASIS AND DELAYED MYOCARDIAL
REPOLARIZATION WITH CARDIAC ARRHYTHMIAS IN THE
HOSPITAL SETTING
Spiegel SB, Khan MU, Weber KT. University of Tennessee Health Science
Center, Memphis, TN.
Purpose of Study: QTc interval prolongation, an ECG index of delayed
myocardial repolarization, is known to increase the propensity for supraand ventricular arrhythmias. A dyshomeostasis of K+, Mg2+ and Ca2+
resulting from acute stressor states and/or medications, such as diuretics,
may lead to QTc prolongation (>440 ms) and appearance of arrhythmias.
Herein, we studied the presence and association of a dyshomeostasis of these
cations in patients found to have prolonged QTc on standard 12-lead ECG.
Methods Used: A retrospective chart review of 200 consecutive patients
(52±1 yr) who were found to have QTc prolongation when presenting or admitted to the Regional Medical Center in Memphis between May and July
2013. We excluded patients with previously documented chronic renal failure (serum creatinine >2mg/dL), those receiving any other medications
known to prolong QTc, and outpatients where sufficient laboratory data
was not available. Lowest levels of serum Ca2+, K+, and Mg2+ were noted
within 24 hrs of obtaining the ECG, together with cardiac rhythm and
QTc interval duration.
Summary of Results: Potassium values <4.0 were present in 61% and in
19% it was <3.5; Mg2+ <2.0 in 51.4% and <1.8 in 19% of patients with
prolonged QTc. Hypocalcemia was present in 30% of patients. Eighty percent were found to have any form of cation dyshomeostasis of which
arrhythmias were seen in 52%. These arrhythmias were supraventricular in
39, ventricular in 12, and both supraventricular and ventricular in 8.
Conclusions: A prolonged QTc interval on standard 12-lead ECG and attendant cardiac arrhythmias are frequently associated with hypokalemia, hypomagnesemia and/or hypocalcemia. Serial surveillance and correction of
serum cation levels within strictly defined limits (K+ ≥4.0 and Mg2+ ≥2.0),
Southern Regional Meeting Abstracts
Mean±SEM (and normal range)
vis-à-vis laboratory-based range of nonbiologic normal values, is recommended to avoid arrhythmias.
113
RITUXIMAB: A DYSHOMEOSTASIS OF SERUM POTASSIUM,
MAGNESIUM AND CALCIUM WITH CARDIAC
ARRHYTHMIAS
Khalid H, Khan MU, Weber KT. University of Tennessee Health Science
Center, Memphis, TN.
Purpose of Study: Rituximab is a chimeric monoclonal antibody that targets
protein CD20 to destroy B cells. Its association with supra- and ventricular
arrhythmias and cardiac arrest has recently been reported; however, their
pathophysiologic origins are uncertain. We hypothesized rituximab is associated with dyshomeostasis of extra- and intracellular cations and accompanied by delayed myocardial repolarization, QTc interval prolongation on
the ECG, and therefore an increased propensity for atrial and ventricular
arrhythmias. Accordingly, we studied serum concentrations of K+, Mg2+
and Ca2+, QTc interval duration and the appearance of arrhythmias in
patients receiving rituximab therapy.
Methods Used: A retrospective chart review of 15 consecutive patients
(47±3 yrs; 5 females) who were started on rituximab, between January
and December 2012, for various lymphomas, including B cell, Burkitt’s,
Hodgkin’s, non-Hodgkin’s and follicular lymphomas. Patients with previously documented arrhythmia or chronic renal failure (serum creatinine
>2 mg/dL), or receiving any other medications known to prolong QTc
were excluded. Lowest total serum [Ca2+], ionized [Ca2+]o, K + and Mg2
+
, obtained within the first 8 wks of initiation of therapy, together with
QTc interval (ms) and cardiac rhythm from ECG or charted rhythm strips
obtained within 3 wks of these data, were noted.
Summary of Results: Hypocalcemia was present in 73% and ionized hypocalcemia in 67%; hypokalemia (K+ <4.0) in 100% and in 87% it was <3.5;
hypomagnesemia (Mg2+ <2.0) in 100% and <1.8 in 93%. Prolonged QTc
(>440 ms) in 87% when K+ was either <4.0, Mg2+ <2.0, and/or hypocalcemia was present. New-onset arrhythmias were seen in 73% when QTc was
prolonged: supraventricular, 9; and ventricular, 2.
Conclusions: Rituximab therapy was frequently accompanied by a
dyshomeostasis of serum K+, Mg2+ and Ca2+ with prolonged QTc and supraand ventricular arrhythmias. Serial surveillance and maintenance of these
cations within strictly defined limits (i. e., K+ ≥4.0 and Mg2+ ≥2.0) is
recommended to avoid this indirect cardiotoxicity during rituximab
administration.
Mean±SEM (and normal range):
114
CISPLATIN: A DYSHOMEOSTASIS OF EXTRACELLULAR
CATIONS AND CARDIAC ARRHYTHMIAS
King BJ, Khan MU, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose of Study: Cisplatin is an antineoplastic agent that acts by binding
to and causing crosslinking of DNA thereby leading to apoptosis of malignant cells. Cisplatin is known to cause renal toxicity which could interfere
with the absorption and excretion of K+, Mg2+ and Ca2+ leading to hypokalemia, hypomagnesemia and hypocalcemia thereby predisposing to cardiac
arrhythmias. We studied serum concentrations of these cations, QTc interval duration and cardiac rhythm on ECG in patients who had received cisplatin therapy.
Methods Used: A retrospective chart review of 19 consecutive patients
(55±9 yrs; 18 males) who were started on cisplatin between January to
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Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
441
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
December 2010, for various cancers, such as multiple myeloma and squamous cell carcinoma, who did not have previously documented arrhythmia or chronic renal failure (serum creatinine >2 mg/dL), or were
receiving any other medications known to prolong QTc. Lowest total serum Ca2+, K +, Mg2+, obtained within first 8 wks of initiation of chemotherapy, together with QTc (ms) and cardiac rhythm, from ECG or
rhythm records obtained within 3 wks of such labs, were noted.
Summary of Results: Hypocalcemia was present in 42%; K+ <4.0 in 74%
and in 47% it was <3.5; Mg2+ <2.0 in 95% and <1.8 in 58%. Prolonged
QTc was found in 15% when K+ was either <4.0, Mg2+ <2.0, and/or hypocalcemia was present. New-onset arrhythmias were seen in 100% when QTc
was prolonged (>440 ms): 1 supraventricular and 1 ventricular. One instance
of atrial fibrillation was noted with a normal QTc.
Conclusions: Cisplatin therapy is accompanied by a dyshomeostasis of extracellular concentrations of K+, Mg2+ and Ca2+ likely due to its renal toxicity. In this small study, we did not find a strong association between QTc
prolongation, an important risk factor for the development of cardiac
arrhythmias, and the presence of supra- or ventricular arrhythmias. This notwithstanding, serial surveillance and maintenance of cations within strictly
defined limits (K+ ≥4.0 and Mg2+ ≥2.0) is recommended to avoid the potential for arrhythmias in patients receiving cisplatin.
Mean±SEM (and normal range)
115
CYCLOPHOSPHAMIDE: CATION DYSHOMEOSTASIS AND
PROLONGED QTC INTERVAL LEADING TO CARDIAC
ARRHYTHMIAS
Flatt DM, Khan MU, Weber KT. University of Tennessee Health Science
Center, Memphis, TN.
Purpose of Study: Cyclophosphamide is a chemotherapeutic agent commonly used in the treatment of lymphoma and leukemia. Cyclophosphamide
may contribute to proarrhythmic pathophysiology that is not associated with
its known cardiomyopathic effects. Herein we hypothesized cyclophosphamide containing chemotherapy regimens lead to extra- and intracellular cation dyshomeostasis which increases the propensity for atrial and ventricular
arrhythmias by delaying myocardial repolarization and causing QTc interval
prolongation on the electrocardiogram.
Methods Used: A retrospective chart review of 200 consecutive patients was
performed finding 10 patients (48 yrs; 6 men) who were treated with cyclophosphamide chemotherapy for various cancers. These patients did not have
previously documented arrhythmia or chronic renal failure (serum creatinine
>2 mg/dL) and were not receiving other medications known to prolong QTc.
Our findings included lowest total serum Ca2+, K+, and Mg2+ (obtained
within first 8 weeks of initiation of chemotherapy) and QTc (ms).
Summary of Results: Hypocalcemia was present in 90% of patients; hypokalemia in 100% and hypomagnesemia in 60%. Prolonged QTc (>440 ms)
was found in 89% of patients and new-onset arrhythmias were seen in 67%
with prolonged QTc. Of those arrhythmias 50% were supraventricular and
50% were ventricular. Mean electrolyte levels and QTc interval are indicated
below.
Conclusions: Cyclophosphamide is accompanied by a dyshomeostasis of
extracellular K+, Mg2+ and Ca2+ with QTc prolongation and a predisposition
to supra- and ventricular arrhythmias. Serial surveillance and maintenance of
QTc and cations within strictly defined limits (K+ ≥4.0 and Mg2+ ≥2.0) is
recommended to avoid arrhythmias during cyclophosphamide treatment.
Results (and normal range)
442
116
CATION DYSHOMEOSTASIS, QTC INTERVAL
PROLONGATION AND ARRHYTHMIAS FOLLOWING
MAJOR SURGERY
Flatt DM, Shahbaz AU, Atta ur Rehman B, Weber KT. University of
Tennessee Health Science Center, Memphis, TN.
Purpose of Study: Neurohormonal activation with elevated circulating
catecholamines accompanies major surgery. Epinephrine and norepinephrine promote the translocation of circulating K+, Mg2+ and Ca2+ to systemic
tissues, including skeletal muscle. The resultant hypokalemia, hypomagnesemia and ionized hypocalcemia leads to the delay in myocardial repolarization, prolongation of the QTc interval of the electrocardiogram and
increased propensity for supra- and ventricular arrhythmias. Herein, we hypothesized such a dyshomeostasis of these cations with QTc interval prolongation (>440 ms) would be associated cardiac arrhythmias following
major surgery.
Methods Used: A prospective assessment of serum concentrations of
K+, Mg2+ and Ca2+ with QTc interval in 40 patients (64.2±1.5 yrs;
11 women) having supra- and/or ventricular arrhythmias within 24 hrs of
major surgery.
Summary of Results: Hypokalemia (3.48±0.08 mmol/L) was found in
88% of these patients while 70% had hypomagnesemia (1.76±0.06 mg/dL) and
73% ionized hypocalcemia (1.03±0.05 mmol/L). QTc prolongation was seen in
88% (496.83±9.4 ms) together with 10 having new-onset atrial fibrillation,
3 with ventricular tachycardia, and the remainder having PACs and PVCs,
or both.
Conclusions: In patients having major surgery, hypokalemia, hypomagnesemia and ionized hypocalcemia, together with prolongation of the
QTc interval, are frequently seen during the immediate postoperative period when they are accompanied by supra- and ventricular arrhythmias,
including atrial fibrillation. Serial surveillance and maintenance of these
cations and QTc interval within narrowly defined limits (e.g., K+ ≥4.0
and Mg 2+ ≥2.0) is recommended to prevent arrhythmias following major
surgery.
117
DOES CARDIAC SURGERY VASOPLEGIA TRIGGER THE
POST PERICARDIOTOMY SYNDROME?
Edriss H, Lorenzana J, Nugent K. Texas Tech University Health Science Center, Lubbock, TX.
Case Report: A 45-year-old healthy man underwent a mitral valve replacement for a flail posterior leaflet. The mitral valve was replaced through a right
atrial incision and the foramen ovale using cardiopulmonary bypass for
73 minutes. He had multiple mean blood pressure readings below 50 mmHg
during the operation; the lowest recorded value was 30 mmHg. A chest tube
was placed in the anterior mediastinum and a drain in the posterior pericardial space after surgery. He had hypotension following the surgical procedure
and required vasopressin for 1 day, norepinephrine for 2 days, and dopamine
for 3 days. His chest x-ray revealed the development of a left and then right
pleural effusions. The patient developed pleuritic chest pain and dyspnea. A
CT scan revealed bilateral pleural effusions, bilateral atelectasis/infiltrates,
and a pericardial effusion. He did not have fever but did have significant leukocytosis (peak WBC 39.8 K/μL) and transient acute kidney injury (peak Cr
2.8 mg/dl). He was started on Indocin (75 mg daily) on the 10th postoperative day and had a steady improvement in his symptoms and partial clearing
of his chest x-ray. Workup for infection, cardiac tamponade, and other postoperative complications was negative.
This patient had a biphasic postoperative course with initial hypotension
and then the development of the post pericardiotomy syndrome. This case
suggests that inflammatory response and vasoplegia associated with cardiopulmonary bypass triggered an immune response which in conjunction with
the surgical procedure produced the post pericardiotomy syndrome. Prospective studies are needed to determine whether or not this association allows
the prediction of this post-operative cardiac surgery complication.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
118
WOMEN HAVE HIGHER CENTRAL SYSTOLIC BP (CSBP)
THAN MEN AS DETECTED BY APPLANATION
TONOMETRY WITH SPHYGMOCOR AND OMRON
DEVICES–COMPARATIVE DIFFERENCES
Hsu RC, Sander GE, Fernandez C, Giles T, Berenson GS. Tulane University,
New Orleans, LA.
Purpose of Study: We have previously reported that women have a significantly higher cSBP as compared to peripheral systolic blood pressure (pSBP)
than do men as measured by Omron applanation tonometry. We have now
compared cSBP and augmentation index (AI) recorded with both
SphygmoCor and Omron instruments to determine if our previous results
were due to technological considerations.
Methods Used: Arterial pressure was measured with a standard cuff and
applanation tonometers from Omron HEM-9000 and SphygmoCor on the
same day on a total of 586 participants in the Bogalusa Heart Study;
participants were 30.6% black, 69.4% white and 45.4% were male (mean
age of 43.7 years ± 4.5). Linear correlation plot was used to describe the difference between cSBP and pSBP (cSBP-pSBP) and AI between Omron and
SphygmoCor devices. Importantly, the SphygmoCor instrument generates
central measurements by employing a transfer function from the radial artery
pulse wave, while the Omron utilizes a regression analysis.
Summary of Results: cSBP detected by Omron confirmed our earlier
findings that cSBP - pSBP was higher in women than men, suggesting reduced vascular compliance between sexes (Table 1). This observation was
also noted with SphgymoCor tonometry, but here cSBP was lower in both
men and women, but less reduced in women relative to men. A stronger linear correlation was detected for AI (r =0 .777) than for cSBP (r =0.679) between Omron and SphygmoCor devices.
Conclusions: Our study confirms that women have a higher cSBP relative to
men regardless of the instrument being used. However, SphgymoCor
provides consistently lower cSBP than Omron, and thus estimation of cardiovascular risk using cSBP measurement is dependent upon the instrument
used–device-specific criteria are necessary. Furthermore, since both devices utilize AI to calculate cSBP, AI alone may be a better indicator for evaluating cardiovascular status.
Computed tomography of Left ventricular pseudoaneurysm. LV = Left
ventricle. P = pseudoaneurysm. T = thrombus contained within
pseudoaneurysm.
In the era of early percutaneous intervention for acute coronary syndrome
(ACS), mechanical cardiac complications, including ventricular pseudoaneurysm, are very uncommon. The natural course of the disease is variable
and unpredictable, and patients are at risk for sudden death from rupture of
the pseudoaneurysm due to increased wall stress. Surgical repair is the
widely accepted treatment.
120
LEFT MAIN CORONARY ARTERY BIFURCATION STENTING
Saxon JT, Campbell WF. University of Mississippi Medical Center, Jackson, MS.
Case Report: This is a 90 year old man with a bioprosthetic aortic valve for
history of aortic stenosis, who reported new-onset angina. He underwent coronary angiography, revealing 60% distal Left Main stenosis and 90% ostial
Circumflex stenosis. Coronary artery bypass surgery was deemed too highrisk. Thus, he underwent high-risk percutaneous coronary intervention with
stent placement to the left main and circumflex arteries, using a “Cone Crush” bifurcation stenting technique. The result was excellent.
Discussion: Percutaneous coronary intervention is high-risk when performed
at the bifurcation of the left main coronary artery and its branches, due to the
large amount of myocardium at risk. This case demonstrates bifurcation stent
technique for a patient who was not a candidate for bypass surgery.
Hemodynamic characteristics by gender groups
119
LEFT VENTRICULAR PSEUDOANEURYSM:
AN UNCOMMON COMPLICATION OF
MYOCARDIAL INFARCTION
Saxon JT1, Sanders SW1, Aru G2, Moore CK1. 1University of Mississippi
Medical Center, Jackson, MS and 2University of Mississippi Medical Center,
Jackson, MS.
Case Report: The patient is a 60 year old woman who presented with chest
pain, and was determined to have an NSTEMI. Three days later, she underwent percutaneous coronary intervention to the proximal left anterior
descending artery, with drug-eluting stent placement. Left ventriculography
revealed anteroapical dyskinesis, with an ejection fraction of 35%. One year
later, the patient presented with decompensated heart failure. Transthoracic
echocardiography revealed a massive left ventricular pseudoaneurysm arising from the cardiac apex. Repeat left ventriculography to further characterized the pseudoaneurym was performed. The patient underwent surgical
repair including resection of the pseudoaneurysm and patch repair of the
ventricular wall. The patient was discharged to an inpatient rehabilitation facility for deconditioning following a prolonged hospital stay.
Coronary angiography before PCI. AV = prosthetic aortic valve. Arrow
= Left Main coronary artery. Arrowhead = Left Circuflex coronary
artery.
© 2014 The American Federation for Medical Research
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443
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
causing entrapment of the leaflet and severe hemodynamic dysfunction of
the valve.
Studies show that 1.2-5.7% of mitral mechanical valves under adequate
anticoagulation are complicated by thrombosis. It is estimated that 77% of
mechanical valve obstructions are caused by thrombus formation, 11% by
pannus, and 12% by a combination of both. It is extremely rare for a very
small thrombus to cause valve obstruction.
122
THE CLINICAL SIGNIFICANCE OF ALCOHOLISM IN
CONGESTIVE HEART FAILURE READMISSIONS - TO
DRINK OR NOT TO DRINK
Coronary angiography following PCI.
121
LEAFLET ENTRAPMENT OF A PROSTHETIC MITRAL
VALVE IN AN ANTICOAGULATED PATIENT
Perez RE, Calderon R, Banchs H. University of Puerto Rico, San Juan.
Case Report: We present the case of a 65 y/o female with history of Rheumatic Fever, severe mitral regurgitation requiring mitral valve replacement
on 2005 with a bi-leaflet mechanical valve, and on therapeutic anticoagulation that was found with leaflet entrapment. Patient developed shortness
of breath on exertion and increased abdominal girth two weeks prior to admission. Physical evaluation was remarkable for bilateral lung base crackles
and mild ascites. INR consistent with proper anticoagulation.
Patient had undergone routine echocardiography by primary cardiologist
every six months unremarkable. New transthoracic echocardiogram showed
elevated transmitral gradients compatible with possible stenosis. TEE confirmed this findings but no evidence of thrombus or pannus. Left and right
heart catheterization found an elevated transmitral mean pressure gradient
of 26mmHg, a valve area of 0.75cm2, and mean pulmonary artery pressure
of 44mmHg. Fluoroscopy revealed fixed movement of one of the prosthetic
valve leaflets. The previous findings were consistent with leaflet entrapment
and significant hemodynamic stenosis of the prosthetic valve.
Patient underwent replacement of the mitral prosthetic valve at which
time a very small thrombus was found on gross description of the stenotic
valve. This small thrombus, not visualized on other non-invasive tests, was
located on the leaflet hinges and prevented movement on the leaflet, thus
Panikkath R, Suvorava N, Ngo N, Umyarova E, Panikkath D, Meyyerose G.
Texas Tech University Health Sciences Center, Lubbock, Lubbock, TX.
Purpose of Study: Literature paradoxically quotes both beneficial and deleterious effects of alcohol on the heart. The prevalence of alcohol consumption among those with CHF readmissions and the characteristics of such
patients are not known.
Methods Used: This study comprises of all adult patients (>20 years of age)
who were readmitted for CHF within 30 days, after a hospitalization for heart
failure at a University hospital from January 1st 2007 to December 31st
2012. The subgroup of patients who had history of alcoholism were analyzed
and their characteristics were compared to those who were readmitted with
CHF but without history of alcoholism.
Summary of Results: Of the 204 patients with CHF who were readmitted,
130 were for CHF exacerbations. Seventy two (55.38%) were males (mean
age 66.99 ± 14.63 years). The mean time of readmission was 14.24 days.
Sixty nine (53.08%) had diabetes and 83 (63.85%) patients were hypertensive. Only 32 patients had history of alcoholism (24.46%). Mean brain natriuretic peptide (BNP) among them was 10290 ± 13953. The mean ejection
fraction was 37.98 ± 17.04%. The mean length of hospital stay was 5.79 ±
3.97 days. In hospital mortality was observed in 6 (4.6%) patients.
The mean age was significantly lower in patients with history of alcoholism in comparison to those without (62.04 ± 11.40 vs. 67.35 ± 15.14 years;
p=0.03) with a male to female ratio 78% vs. 52.31%; p=0.006). The mean
BNP was lower in patients with history of alcoholism compared to those
without history of alcoholism (82143 ± 14014 vs. 104486 ± 14264,
p=0.51). The mean EF was significantly lower in patients with history of alcoholism compared to those without (35.43 ± 18.51% vs. 39.37 ± 16.47%,
p=0.04). The length of stay was slightly longer in patients with history of alcoholism although it was not statistically significant 6.08 ± 4.52 vs. 5.41 ±
3.70 days, p=0.52).
Conclusions: Alcoholism contributed to only less than quarter of CHF hospital readmissions. However, the mean age of patients with alcoholism was
significantly younger and they were predominantly males compared to the
sex matched distribution of subjects without history of alcoholism. The mean
ejection fraction was lower and the hospital stay was slightly longer in
patients with history of alcoholism.
123
AN INCIDENTAL DIAGNOSIS OF PENETRATING AORTIC
ULCER OF THE ASCENDING AORTA LEADING TYPE A
AORTIC DISSECTION
Hwang I2,1, Giri S2,1, Mallisho M2,1, Alsafwah S1,2. 1University of Tennessee,
Memphis, TN and 2University of Tennessee, Memphis, TN.
Case Report: A 51-year-old female with CHF with 20% EF on TTE, admitted for cardiac catheterization. Patient was found to have no significant CAD.
However, she had an aneurysmal ascending aorta with a mass-like structure.
CT chest with contrast showed type A aortic dissection likely secondary to a
penetrating ulcer. It was also noted that her ascending aorta was severely dilated with a transverse diameter of 6.5 cm. Dissection starts just above the
sinuses of Valsalva and terminates prior to the great vessel origins, does
not extend into the great vessels or descending thoracic aorta.
444
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
tachypnea 28/min. Cardiopulmonary and neurologic examinations were initially unremarkable. Following a seizure, the patient was intubated and soon
thereafter was noted to be hypotensive requiring intravenous pressor agents.
Initial ECG revealed sinus tachycardia without ST segment or T wave
changes while laboratory panel did not indicate injury to renal, hepatic, muscular or hematologic systems. Antivenom treatment was initiated after consultation with Poison Control Center and continued for 3 days. However,
over the next 24h the patient developed multiorgan injury and failure: i) serum troponin rose to 396 ng/mL without ECG changes, while echocardiogram revealed apical hypokinesis and ejection fraction of 30%; ii)
rhabdomyolysis with peak creatinine phosphokinase of 8760 ng/mL; iii) hepatocyte necrosis with elevated serum transaminases; and iv) acute renal failure with elevated serum creatinine 3.5 mg/dL, which required hemodialysis.
Despite aggressive antivenom administration and other necessary
interventions, the patient succumbed days later.
Conclusions: Toxins contained in pit viper venom, such as that of the timber
rattlesnake, can cause dose-dependent injury to multiple organs. As occurred
in this patient with likely large-dose envenomation, there was putative ischemic necrosis of heart, muscle and liver parenchyma. Its pathophysiologic
origins would therefore likely include: a) endothelial cell damage and occlusion of the corresponding conduit vessels supplying these ischemic organs;
b) a thrombotic microangiopathy of involved organs; or c) a combination
of these pathologic entities.
125
An ascending aortic angiogram showing a large-sized contrast filled aneurysmal-like structure involving the proximal part of the ascending aorta.
CT image of ascending aortic aneurysm likely due to penetrating aortic
ulcer.
124
HEART, MUSCLE AND LIVER NECROSIS FOLLOWING
ENVENOMATION BY TIMBER RATTLE SNAKE
Askari R, Farah V, Koshy SK, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose: The timber rattle snake (Crotalus horridus) is considered North
America’s most venomous snake. After envenomation, attendant toxins
may be directed at nervous, hematologic and cardio/skeletal tissues. Herein,
we present the case of a farmer who developed necrosis and failure of multiple organs after being bitten on the right lower extremity by a timber rattle
snake.
Case Report: Upon transfer from another health care facility, this 61-yearold male presented with complaints of right leg and abdominal pain with
shortness of breath. Blood pressure 128/76; tachycardia 128 bpm; and
URINARY MAGNESIUM WASTING WITH
HYPOMAGNESEMA AND ATRIAL FIBRILLATION IN A
PATIENT WITH HYPERTROPHIC CARDIOMYOPATHY
McCullough RJ, Kabra R, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant
disorder with considerable phenotypic variability, including a less well-recognized association with altered Mg2+ homeostasis. Herein we present a
young woman with HCM accompanied by marked hypomagnesemia, secondary hypocalcemia, QTc prolongation and the appearance of symptomatic
atrial fibrillation (AF) found to be associated with pronounced urinary Mg2+
wasting.
Case Report: Over the past 12 months this 22-year-old female with known
HCM presented to the Emergency Department on multiple occasions with
paroxysms of rapid AF and complaints of chest tightness, dyspnea and
palpitations. Previously, she had received an implantable cardioverter (ICD)
and two ablation procedures for her AF. Positive family history included a
brother, who died suddenly while playing sports, and her sister diagnosed
with HCM. Pertinent physical findings included: an irregular, irregular
rhythm and pulse deficit with systolic murmur that decreased with handgrip
maneuver or leg raising. Laboratory studies: persistent normokalemia (>4.0
mEq/L); variable calcium ranging from low to normal (7.7-9.2 mg/dL); and
marked hypomagnesemia (≤1.5 mg/dL), which failed to correct despite intravenous and oral Mg2+ supplementation. ECG revealed rapid AF with
QTc prolongation (468 ms). Fractional urinary Mg2+ excretion was 27%
(normal 2%), consistent with marked renal wasting. Spironolactone, an aldosterone receptor antagonist, corrected her hypomagnesemia. Serum Mg2+
and Ca2+ in her sister were 1.9 and 8.6 mg/dL, respectively.
Conclusions: Mutations of the transient receptor potential melastatin 6 and 7
(TRPM 6 and 7) lead to dysfunctional epithelial cell ion channel Mg2+ resorption in kidney and colon and which is further downregulated by aldosterone released in response to reduced intracellular Mg2+. Ensuing marked
urinary and fecal Mg2+ losses with profound hypomagnesemia can lead to
AF and where these losses can be abrogated by spironolactone. Further interrogation of these Mg2+ transporters in HCM is merited.
126
AN UNUSUAL CAUSE OF PERICARDITIS
Waespe CK, Saxon J, Lopez-Ruiz A, Ashley K, Alexander M. University of
Mississippi Medical Center, Jackson, MS.
Case Report: Kelly Waespe MD, John Saxon MD, Arnaldo Lopez-Ruiz
MD, Kellan Ashley MD, Myrna Alexander MD
© 2014 The American Federation for Medical Research
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445
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
University of Mississippi Medical Center Department of Cardiology.
Pericarditis is a known cause of chest pain. Diagnostic clues are history,
diffuse ST elevation on Electrocardiogram (ECG), friction rub, or pericardial
effusion. Causes include aortic dissection, malignancy, or trauma, however
most cases are viral or idiopathic. Effusions can lead to tamponade with
anticoagulation and require observation. We describe an unusual cause of
pericarditis, the management, and implications.
A 68-year-old female presented with positional chest pain and ST elevation in II, III, and aVF on ECG. She had no prodrome. Cardiac biomarkers
were negative. Echocardiogram demonstrated a moderate pericardial effusion. A year prior she underwent a pancreatoduodenectomy, complicated
by post-operative day 10 development of Deep Vein Thrombosis (DVT).
An inferior vena cava (IVC) filter was placed. Later filter removal was
thwarted by thrombus on the filter and anticoagulation was initiated.
Her unusual presentation led to further work up. Computed Tomography
demonstrated a fractured fragment of the IVC filter in the right ventricle, and
hemopericardium. The remaining filter was in its original position, without
thrombus. Several services were involved in a hybrid approach to retrieval.
After sternotomy, the fractured fragment was visibly protruding from the
right ventricle and removed with a clamp. Via the right internal jugular vein,
complex techniques were required to free the filter from the IVC. There were
no apparent complications.
Diffuse ST elevation is pathognomonic for pericarditis, and segmental
ECG changes are infrequent. In this case the ECG changes may be related
to the cause of pericarditis, a rare complication of IVC filters. Filters are indicated for DVT with anticoagulation contraindications, however their use
remains controversial. Complications are infrequent and fracture of a filter
is reported in 1% of cases. Several filter types are retrievable, but attempts
are made in only 34% of cases. Here, embolization of a fractured strut required surgical removal, followed by percutaneous retrieval of the filter. We
present an unusual cause of pericarditis, complications of IVC filters, and
the need for filter retrieval.
cephalosporin or ampicillin-sulbactam for 4-8 weeks as the first line of therapy for
HACEK endocarditis. There were only two reports of extensive tissue invasion adjacent to the site of valve infection. This case demonstrates the importance of appropriate antibiotic therapy at the initial diagnosis of infective endocarditis to
improve clinical outcomes.
128
MASSIVE PULMONARY EMBOLUS IN TRANSIT
Marcial JM1,2, Jimenez L1,2, Calderon R1,2, Grovas D2, Kareh J2. 1University
of Puerto Rico School of Medicine, San Juan and 2Cardiovascular Center of
Puerto Rico and the Caribbean, San Juan.
Case Report: A 73 year-old man with hypertension, dyslipidemia and history of smoking was evaluated in the emergency department for an acute episode of near syncope and chest pain associated to a week of progressive
exertional dyspnea and recurrent dizziness upon standing. Transthoracic
echocardiography demonstrated an echodense structure with origin in the inferior vena cava and protruding through the right heart chambers.
Transesophageal echocardiography further characterized extension of the
thrombus into the pulmonary artery. Chest computerized tomography and
pulmonary arterial angiography confirmed multiple bilateral pulmonary emboli. Bilateral lower extremity ultrasound Doppler and duplex established
culprit deep vein thrombosis in the right common femoral, superficial femoral
and popliteal veins. In view of persistent hypotension despite anticoagulation,
intravenous thrombolytics were administered. Although there was initial relief
of cardiopulmonary symptoms with fibrinolytic therapy, the patient developed
sudden cardiac arrest due to massive intracerebral hemorrhage with brainstem
compression and ultimate demise. Discussion: Acute pulmonary embolism
with visible right-heart thrombi is a rare phenomenon and is associated with a
poor prognosis. Unstable patients should be considered for thrombolysis as
an emergency, life-saving treatment. However, fibrinolytic treatment is not without its risk for complications, as was demonstrated in this case.
127
A RARE OCCURRENCE OF HAEMEPHILUS
PARAINFLUENZAE AORTIC VALVE ENDOCARDITIS
WITH EXTENSIVE DISRUPTION OF THE AORTIC ROOT
Swaminath D, Soape M, Singh P, Narayanan R, Desai V, Jenkins L.
TexasTech University Health Science Center, Lubbock, TX.
Case Report: We present an unusual presentation of aortic valve endocarditis caused by H. parainfluenzae.
A 53-yr old male with PMH of alcohol abuse, illicit drug use, and DM
presented with symptoms of dyspnea, peripheral edema, and productive
cough for a month. A month ago he was diagnosed with Haemophilus
parainfluenzae aortic valve endocarditis and was receiving intravenous (IV)
antibiotics including ampicillin and vancomycin. Patient was admitted with
diagnosis of CHF secondary to severe aortic regurgitation. A large aortic
valve vegetation measuring 1.3 × 1.1cm, severe aortic regurgitation, a
perivalvular abscess encircling the aortic root, and a fistula to the left ventricular outflow tract were noted on the TEE. At this point, surgery was contemplated, and the patient underwent a coronary artery angiogram showing a 4050% stenosis in the right coronary artery. Patient was started on IV
tobramycin and ceftriaxone. Emergent surgery was performed to replace
the aortic valve. Post operatively patient recovered without complication.
HACEK group of organisms accounts for 3% of all endocarditis case.
Endocarditis caused by Haemophilus species is even rarer: about 0.8% to
1.3% of all cases in adults. Risk factors for development of H. parainfluenzae
endocarditis therefore include dental work, cleaning of teeth, nasopharyngeal
infection, tongue pierg, and the use of tongue scrapers. Indication for surgical intervention in bacterial endocarditis is major or recurrent septic embolization, congestive heart failure due to valve stenosis or regurgitation, false
aneurysms, large mobile vegetations, and persistent infection. HACEK endocarditis was treated by beta-lactam antibiotics such as ampicillin, with or
without the addition of an aminoglycoside. However, because of resistance to
betalactam current guidelines recommend empiric treatment with a 3rd-generation
446
Image A: Transthoracic echocardiography demonstrating elongated
thrombus traversing the right heart chambers. Image B: Transesophageal
echocardiographic visualization of the embolus in transit through the
right ventricular outflow tract and into the pulmonary artery.
129
NATIVE MITRAL VALVE ENDOCARDITIS COMPLICATED
AFTER
SURGICAL REPLACEMENT
Garcia D, Nascimento Cardoso R, Young L. University of Miami, Miami, FL.
Case Report
Introduction: Bacterial endocarditis can lead to serious complications such
as valve leaflet rupture requiring replacement or repair. Mycobacterium
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
abscessus and Kocuria species are rare causes of infections in humans. We
report a case of native mitral valve bacterial endocarditis caused by both
M. abscessus and Kocuria species complicated by mitral valve rupture.
Case Description: A forty-eight year old male intravenous drug abuser was
admitted for 1 week of fevers, shortness of breath and abdominal pain. Upon
initial evaluation he presented with thrombosis of the proximal celiac artery
due to septic emboli. Blood cultures initially revealed Enterococus fecaelis
and he was treated with ampicillin and gentamicin. The transesophageal
echocardiogram disclosed a two centimeter vegetation on the posterior mitral
valve leaflet with associated perforation leading to severe mitral regurgitation. Repeated blood cultures disclosed no organisms and therefore surgical
mitral valve replacement was performed using a bioprosthetic valve. Postsurgical blood cultures and mitral valve tissue cultures revealed Kocuria species and M. abscessus. At this point the patient was not a candidate for further surgery and therefore he was treated for 6 months with clarithromycin,
imipenem and amikacin, with resolution of symptoms. Repeated blood
cultures were negative.
Discussion: Infective endocarditis caused by one of these agents has only
been reported in 11 cases and there are no prior reports of combined infection. Prosthetic or native mitral valve can be affected equally; valve repair
is the treatment of choice although in cases where the patient is not a surgical
candidate, long-term antibiotic therapy is a feasible option. In our particular
case initial negative cultures or in atypical cases of sub acute endocarditis,
particularly if antibiotic resistance is an issue, acid-fast staining should be
done in order to increase the diagnosis of this infection.
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131
HYPERTROPHIC CARDIOMYOPATHY
Laura S, Singh M, Jain N. LSU Health Sciences Center, New Orleans, LA.
Case Report: A 47 year old woman female with family medical history
(Figure 1), of sudden death at the age of 60-70 was diagnosed with hypertrophic cardiomyopathy after presenting for 3 weeks of chest pain described
as left sided, dull/achy, without radiation, and occurring both at rest and
with exertion. The pain was associated with shortness of breath, dyspnea
on exertion and palpitations which had progressed to occurrence after a single flight of stairs and light housework over the past week. An echocardiogram on admission revealed normal LV function of >55% with severe
asymmetric septal hypertrophy, septal thickness of 20-29mm, moderate
systolic anterior motion of the mitral valve, with peak left ventricular outflow tract (LVOT) velocity of 6m/s and peak gradient of 144 mm Hg. The
patient underwent left heart catheterization which revealed normal coronaries with a large first septal branch demonstrating dynamic systolic compression. Hemodynamics revealed LV body pressure of 200/15 mm Hg with a
late-peaking dagger shape as in dynamic LVOT obstruction, LVOT pressure
of 100/15 mm Hg, aortic pressure of 100/61 mm Hg. No aortic valve obstruction was present. Cardiac MRI was performed which revealed an interventricular septum thickness of 31mm, LVOT obstruction, and a mid
myocardial scar in the inferoseptal segment. The patient underwent placement of an ICD for primary prevention.
Discussion: Hypertrophic Cardiomyopathy (HCM) is defined as left ventricular hypertrophy without an identifiable cause such as hypertension, aortic
stenosis, or chronic renal failure that usually develops in the second decade
of life. Prevalence in the general population is 1:500. HCM is inherited in
an autosomal dominant pattern in approximately 50% of cases with the
remaining due to new mutations. HCM, in the vast majority of cases, is associated with hypertrophy of the proximal anteroseptal wall, leading to obstruction of the left ventricular outflow tract in approximately 25% of cases.
130
RECIPE FOR ADMISSION: ATYPICAL CHEST PAIN AND
INCORRECT LEAD PLACEMENT
Spinks K, Arbour A, Engel LS, Amoss J. LSU Health Sciences Center, New
Orleans, LA.
Case Report: Chest pain is one of the most common primary complaints in
patients presenting to the Emergency Department, and electrocardiographic
findings are often the cornerstone in making a diagnosis. We present a case
where ECG lead misplacement significantly altered management.
Case: A 41 year old African American male presented to the emergency department with a chief complaint of chest pain, 5/10 in severity that began
while at rest and had been constant since the prior evening. His chest pain
was located in his left anterior chest and radiated to his shoulder and left
arm. The pain was not associated with activity, shortness of breath, nausea,
or vomiting. He had noted similar chest pain intermittently over the past
two months. On arrival to the emergency department, the patient appeared
comfortable, vital signs and his physical exam were unremarkable. The first
set of cardiac enzymes was negative. His initial electrocardiogram demonstrated T-wave inversions in leads V3-V6 causing concern for myocardial ischemia and the need for possible admission to rule out acute coronary
syndrome. Upon further examination of the patient, the precordial leads were
noted to be placed incorrectly, extending laterally from the xiphoid process at
the seventh intercostal space. A new set of leads were placed in the proper
positions for leads V1-V6 in the fourth and fifth intercostal spaces, and a repeat electrocardiogram showed no T-wave inversions. A subsequent electrocardiogram was repeated with leads placed incorrectly, which again
demonstrated the T-wave inversions. Based upon his history, physical, and
corrected diagnostic evaluation, the patient was determined to be very low
risk for having an acute coronary syndrome, and he was released home with
outpatient follow-up.
Discussion: Chest pain prompts many emergency department visits requiring clinicians to risk stratify patients based upon history, physical, and initial
diagnostic studies to determine the need for hospitalization to rule out acute
coronary syndrome. Clinicians make important patient care decisions based
upon electrocardiographic findings; therefore, clinicians must be aware of the
potential pitfalls of incorrect lead placement in order to avoid potential
mismanagement.
132
SARCOID CARDIOMYOPATHY
Laura S, McShurley T, Jain N. LSU Health Sciences Center, New Orleans, LA.
Case Report: Sarcoidosis is a systemic disease with the formation of noncaseating granulomas in a variety of tissues. Cardiac involvement is one of
the least common manifestations, however it has been suggested that it
may be present in up to 25% of those individuals diagnosed with systemic
sarcoidosis. Cardiac sarcoidosis may affect both the conduction system and
myocardial function through granuloma deposition and fibrosis leading to
ventricular dysfunction, congestive heart failure, atrioventricular block, atrial
or ventricular arrhythmias, and even sudden death.
Case: 56 y/o obese female with past medical history of untreated sarcoidosis
with pulmonary and neurological involvement (diagnosed by lumbar puncture several years prior), depression, anxiety, hypertension, hyperlipidemia,
migraines, GERD, and asthma was referred for evaluation of chest pain
and dyspnea on exertion. Her chest pain was described as sharp and left
sided with radiation to the jaw. Patient stated pain lasted approximately 1015 minutes, occurring 2-3 times per week for the past 3-4 months with exertion, rest, and coughing. Patient also described dyspnea when ambulating approximately 100 yards that was relieved with brief periods of rest, minimal
intermittent pedal edema worsened by prolonged standing, and orthopnea
for many years. Electrocardiogram showed normal sinus rhythm with occasional premature ventricular complexes. Two dimensional echocardiogram
revealed left ventricular enlargement (LVIDd: 5.6 cm, LVIDs: 4.9cm) with
an ejection fraction of approximately 45% and a thin, akinetic inferior wall
consistent with scarring. Estimated pulmonary artery pressure was less than
35mmHg. Diastology revealed an abnormal relaxation pattern with normal
left atrial pressure. The patient underwent coronary angiogram which revealed non-obstructive coronary artery disease. Chest radiography remained
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447
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
unchanged from computed tomography a few months prior which showed
perihilar and suprahilar lymphadenopathy. Patient was discharged with pulmonary and cardiac follow up for continued management of her sarcoidosis.
echocardiography. Two sub-types have been described. A pinhole-shaped
UAV typically presents in early infancy while a slit-shaped unicuspid aortic
valve commonly presents later in early adulthood.
133
135
LBBB ASSOCIATED WITH SPONTANEOUS
PNEUMOTHORAX: A CASE REPORT
Johnson P, Paccione R, Burwell J, Lo B. LSU Health Sciences Center, New
Orleans, LA.
Case Report: There are multiple reports in the literature of EKG changes associated with spontaneous pneumothoraces. However, there is no documentation
of isolated left bundle branch block (LBBB) changes due to a right-sided pneumothorax. We will discuss a patient with a right-sided spontaneous pneumothorax with a new LBBB that resolved post treatment.
Case: Our patient is a 62-year old African-American male with a history of
chronic atrial fibrillation, gout, diabetes, hyperlipidemia, and hypertension
who presented to the ED with a chief complaint of “chest pain and shortness
of breath x 10 hours.” His atrial fibrillation was rate-controlled without
medications. Home medications included warfarin, allopurinol, atorvastatin,
furosemide, lisinopril, and metformin. In the emergency department, the patient was found to have new LBBB and atrial fibrillation with rate of 90 bpm
on EKG, moderate-sized right pneumothorax on CXR, and normal cardiac
enzymes. After FFP administration, CT surgery placed a catheter in the right
chest. Repeat CXR showed complete re-expansion of the right lung. The patient had multiple EKGs in the ER that showed LBBB prior to chest tube insertion. However, serial cardiac enzymes were negative and serial EKGs
showed lateral T-wave inversions and LBBB. The patient remained chestpain free after the chest tube placement, and the tube was removed by the
third hospital day. The final EKG on the day of discharge showed AFIB with
resolution of the LBBB and improvement of T-wave inversions. Cardiac
enzymes remained negative.
Discussion: Common EKG changes that have been documented with
pneumothoraces include ST-segment elevation, T-wave changes, axis deviation
and incomplete right bundle branch block regardless of the side of the pneumothorax. Theoretically, pneumothoraces increase intrapleural pressures and decrease venous return and stroke volume; tachycardia then increases oxygen
demand resulting in ischemic changes on EKG. However, this does not seem
to be the case in our patient. Multiple sets of cardiac enzymes were normal despite the new LBBB and chest pain. This case emphasizes the importance of
recognition of LBBB directly related to a pneumothorax rather than to myocardial ischemia thus avoiding unnecessary invasive procedures i.e. coronary
angiograms.
TRICUSPID VALVE AVULSION: A CHALLENGING
DIAGNOSIS ASSOCIATED WITH BLUNT CHEST WALL
AND CARDIAC TRAUMA
Bomb R, Sethi PR, Garg N, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose: Posttraumatic avulsion of the tricuspid valve (TV) causing acute
tricuspid regurgitation is a rare, often overlooked and potentially fatal complication of blunt chest wall trauma with attendant cardiac injury. Although
it has been reported, acute tricuspid regurgitation can often be missed due
to its subtle presentation in the setting of more obvious traumatic injuries.
Unrecognized persistent tricuspid regurgitation will lead to right ventricular
volume overload and dysfunction with poor prognosis.
Case Report: A 34-year-old obese man presented to the Trauma Unit of the
Regional Medical Center with blunt trauma to his chest after being hit by a
car. Notable injuries included fracture of left hemithorax involving 4 ribs, femur and tibia. A few hours after admission, his blood pressure (BP) fell to
shock levels requiring intravenous pressor agents. On examination: BP 70/
42 mmHg; and pulse 100/min. Neck veins were difficult to assess due to
body habitus. A faint systolic murmur was noted. Urgent transthoracic echocardiogram was performed to assess the etiology of his hemodynamic instability: a flail anterior TV leaflet was seen with avulsion of the papillary
muscle accounting for severe eccentric acute tricuspid regurgitation; and systolic flow reversal was noted in the hepatic veins. Transesophageal echocardiogram confirmed these findings, during which time his Swan-Ganz
catheter, used for hemodynamic monitoring, was retracted to better visualize
the TV. Unconventional views at mid-esophagus provided complete assessment of the TV. Consultation with cardiothoracic surgery was obtained for
consideration of TV repair.
Conclusions: In patients with blunt chest trauma a high index of suspicion,
careful physical examination and echocardiography can lead to prompt recognition of this rare but potentially fatal complication. If TV and its leaflets
cannot be readily visualized, image optimization using non-conventional
views and removal of any right heart catheters should be considered. Early
surgical intervention may be necessary for prevention of long-term
complications attendant with persistent tricuspid regurgitation.
134
136
A RARE CAUSE OF AORTIC STENOSIS
TRACHEOSTOMY-RELATED SINUS BRADYCARDIA. A CASE
OF INTERNAL CAROTID MASSAGE
McShurley T, Laura S, Jain N. LSU Health Sciences Center, New Orleans, LA.
Case Report: A 33-year-old male presented with progressively worsening
substernal chest pressure and dyspnea on exertion relieved with rest over three
months duration. Physical examination revealed diminished carotid pulse with a
II/VI systolic crescendo murmur radiating to both carotids. Transthoracic echocardiogram showed a heavily calcified aortic valve with limited motion consistent with aortic stenosis. Peak gradient across the aortic valve was 110 mmHg
with a calculated aortic valve area of 0.6 cm2 consistent with severe aortic stenosis. The other valves appeared normal in structure and function. The patient
underwent surgical aortic valve replacement. Gross examination revealed a
heavily calcified, slit-shaped unicommissural aortic valve. Unicuspid aortic
valve is a rare congenital malformation seen in 0.019% of patients undergoing echocardiography and in 5.59% of patients undergoing aortic valve replacement. Approximately 60% of unicuspid aortic valves in adults are diagnosed
after surgical resection or at autopsy while only 20% are found on
448
Bomb R, Koshy SK, Weber KT. University of Tennessee Health Science
Center, Memphis, TN.
Purpose: The baroreceptor reflex emanating from the carotid sinus is integral to cardiovascular homeostasis, including the regulation of heart rate
and blood pressure. Stretching the carotid sinus activates the parasympathetic
nervous system with ensuing vagal stimulation causing sinus bradycardia
and even hypotension. External compression of the neck over the carotid sinus, located at the bifurcation of the internal and external carotid arteries, has
been used to raise vagal tone and convert tachyarrhythmias. Herein we present a unique case of inappropriate sinus bradycardia attributed to internal
compression of the carotid sinus.
Case Report: A 57-year-old man was hospitalized at Regional Medical Center, Memphis where he was intubated after multiple rib fractures and pulmonary contusion following a motor vehicle accident. A tracheostomy was
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
ultimately performed and several days later he was found to have developed
asymptomatic sinus bradycardia (rate 30-40 bpm) without hypotension or arrhythmia. Serum K+ and Mg2+ were 4.6 mmol/L and 2.0 mg/dL, respectively; and arterial O2 saturation 95%. QTc interval remained normal and
he was not receiving medications that would prolong it. No cause of his bradycardia could be ascertained other than the patient noting these episodes occurred with eating or moving his neck. This prompted careful manipulation
of his tracheostomy tube, including withdrawal with reversion to normal sinus rhythm and increment in heart rate to 70-80 bpm. The tube was
ultimately removed on day 5 and the patient discharged home with no further
episodes of bradycardia.
Conclusions: Tracheal suction has been reported to cause vagal stimulation
with ensuing sinus bradycardia. Herein, we report a hitherto unreported
cause of intermittent, asymptomatic sinus bradycardia induced by internal
carotid sinus massage by the tracheostomy tube. Awareness of this pathophysiologic response to unintended or intended manipulation of a tracheostomy tube can minimize its occurrence and prevent unnecessary evaluation.
137
BLUNT CHEST WALL TRAUMA WITH ST SEGMENT
ELEVATION: A CASE OF MYOCARDIAL CONTUSION
MIMICKING INFARCTION
Bomb R, Nayyar M, Garg N, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose: Damage to the heart following blunt chest wall trauma can range
from myocardial contusion to rupture with death. ECG changes vary from
minimal conduction defects to life threatening ventricular arrhythmias. Unfortunately there are no definitive diagnostic criteria for myocardial contusion except postmortem histopathologic findings. Herein, we present a case
of a young man who presented after blunt chest wall trauma with significant
ST elevation and minimal rise in serum troponin due to myocardial contusion
mimicking an acute myocardial infarction.
Case Report: A 32-year-old man with no significant medical history
presented to the Trauma Unit of the Regional Medical Center in Memphis after sustaining blunt chest wall trauma during a motor vehicle accident. He
was also found to have multiple rib fractures along with lower extremity
fractures and reported generalized chest pain that varied with respiration
and posture. No other risk factors for coronary artery disease were identified
in this otherwise healthy young male. Diffuse ST segment elevation was
noted on his initial ECG compatible with an acute myocardial infarction:
0.5-1 mm ST segment elevation in leads I, II, III, aVF and V2-V6.
Biomarkers of cardiomyocyte necrosis, including serum troponin, were
minimally elevated with no significant further rise over the next 24 hours.
Serum electrolytes were normal. A bedside transthoracic echo revealed a
normal left ventricle with no regional wall motion abnormalities or pericardial effusion. The patient was managed conservatively and ST elevation resolved over the next 24 hours.
Conclusions: We conclude that ST elevation on initial ECG with minimal
troponin release following blunt chest wall trauma with myocardial contusion can mimic an acute myocardial infarction and where anticoagulants
would be contraindicated.
138
INSULIN-LIKE GROWTH FACTOR-1 INCREASES
COLLAGEN TYPE I SYNTHESIS VIA INDUCING
EXPRESSION OF THE MRNA BINDING PROTEIN
LARP6 AND ITS BINDING TO THE 5′STEM-LOOP OF
COL1A1 AND COL1A2 MRNA
Blackstock C, Higashi Y, Sukhanov S, Delafontaine P. Tulane University
School of Medicine, New Orleans, LA.
Purpose of Study: Collagen content in atherosclerotic plaque is important in
prevention of plaque-rupture and is established as a hallmark of plaque stability. Our earlier studies showed that insulin-like growth factor-1 (IGF-1)
increases collagen content in atherosclerotic plaques of Apoe-/- mice. To
Southern Regional Meeting Abstracts
identify mechanisms, we investigated the effect of IGF-1 on the la ribonucleoprotein domain family member 6 (LARP6). LARP6 binds a stem-loop
motif in the 5’UTR of the mRNAs encoding the collagen type I alpha
subunits (α1(I) and α2(I)), and coordinates their translation into the
heterotrimeric collagen type I molecule.
Methods Used: Human aortic smooth muscle cells (SMCs) and mouse embryonic fibroblasts (harboring a 5’stem-loop mutation) were cultured and collagen expression in response to IGF-1 was measured via western blot and
3H-proline accumulation. LARP6 expression and association with COL1a1
and COL1a2 mRNA was measured via western blot and immunoprecipitation of LARP6, followed by qPCR. Additionally, an adenovirus was used
to overexpress a 5′Stem-loop RNA that served as a molecular decoy to sequester LARP6. IGF-1 upregulation of LARP6 and collagen expression was also
assessed in aortic tissue in an Apoe-/- mouse model of atherosclerosis.
Summary of Results: IGF-1 rapidly increased LARP6 expression and the
rate of collagen synthesis and extracellular accumulation. IGF-1 increased
the association of LARP6 with COL1a1 and COL1a2 mRNA, and mutation
of the 5’stem-loop of Col1a1 mRNA, which inhibits binding of LARP6,
abolished the ability of IGF-1 to increase synthesis of collagen type I. Furthermore, overexpression of a 5’stem-loop RNA decoy prevented the ability
of IGF-1 to increase pro-α1(I) and mature α1(I) expression in cultured medium. IGF-1 infusion in Apoe-/- mice increased expression of LARP6 and
pro-α1(I) in aortic lysates, and SMC-specific IGF-1-overexpression robustly
increased collagen fibrillogenesis in atherosclerotic plaque.
Conclusions: We identify the mRNA binding protein LARP6 as a critical
mediator by which IGF-1 augments synthesis of collagen type I in vascular
smooth muscle, which may play an important role in promoting atherosclerotic plaque stability.
139
SAPHENOUS VEIN GRAFT RUPTURE PRESENTING WITH
CARDIAC TAMPONADE EARLY AFTER CORONARY ARTERY
BYPASS SURGERY
Ababneh B, McShurley T, Gaines T, Jain N. LSUHSC, New Orleans, LA.
Case Report: Post coronary artery bypass surgery (CABG) cardiac
tamponade due to spontaneous rupture of saphenous vein graft (SVG) as
early as the third post-operative day has never been described before to our
knowledge. We report a case of early rupture of SVG on the third day after
CABG resulting in cardiac tamponade.
Case: A 70-year-old male with multiple risk factors was admitted for evaluation of accelerating angina and dyspnea. Physical examination was unremarkable. Electrocardiogram (ECG) was unchanged from one year prior.
Serum chemistries including serial cardiac biomarkers were negative. Coronary angiography showed severe multivessel disease for which CABG including 3 SVG grafts was subsequently performed.
On post operative day 3, the pericardial drain was removed. Upon transfer to a chair five hours later, he suddenly collapsed. Pulse was absent and
telemetry showed asystole. Resuscitation was performed and return of spontaneous circulation was achieved rapidly. Exam revealed muffled heart
sounds and elevated jugular venous pressure consistent with tamponade.
Emergent surgical exploration revealed hemopericardium and active bleeding from a tear in the SVG to the right coronary artery. The hemopericardium
was evacuated and the SVG tear was repaired with marked improvement in
hemodynamics.
Discussion: SVG rupture is a rare complication of CABG. It is typically associated with pathophysiological changes in the graft such as an aneurysm,
pseudoaneurysm, or as a consequence of mediastinitis or infection. Spontaneous rupture of vein grafts has been reported in literature however nearly
all presented years after surgery.
This case to our knowledge is the earliest occurring case of spontaneous
SVG rupture, occurring on the third post-operative day. It is unclear what
predisposing factors were present. Given the quiescent nature of the patient’s
initial hospital course, it is unlikely that the observed rupture occurred as a
manifestation of surgical technique mishap. Adding to that, there was no suggestion of graft aneurysm, mediastinitis or infection during this period. We
cannot fully exclude SVG trauma resulting in rupture due to removal of the
pericardial drain.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
449
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
140
AORTIC PSEUDOANEURYSM CAUSING LEFT ATRIAL
OBSTRUCTION
Aiello D, Lathia V, Jain N, Helmcke FR. LSUHSC–New Orleans, New
Orleans, LA.
Case Report: A 26 year-old white male with heroin abuse presented to the
emergency department with complaints of fever and malaise for a few days’
duration. His past medical history is significant for chest gun shot wound associated with a dime sized ascending aortic tear requiring emergent suture repair in 2007. A residual aortic pseudoaneurysm became apparent after his
initial repair but he refused surgical correction. Since then, the pseudoaneurysm was not followed as intended with scheduled serial aortic imaging
due to non-compliance.
During the current admission, he was diagnosed with pneumonia, sepsis,
and newly diagnosed Acquired Immunodeficiency Syndrome. Despite adequate management of sepsis, his respiratory status continued to decline and
the patient was intubated. A transthoracic echocardiogram was obtained revealing a very large aortic pseudoaneurysm, measuring 7.6cm x 5.5cm with
subtotal obliteration of the left atrial cavity. In addition, severe tricuspid regurgitation and pulmonary hypertension were seen. Three-dimensional
transesophageal echocardiography revealed compression of the bilateral pulmonary veins and erosion of the left atrial wall by the pseudoaneurysm
resulting in thrombus formation in the left atrium. A CT angiogram of the
patient’s chest confirmed these findings with additional compression noted
of the right pulmonary artery by the pseudoaneurysm.
The cardiothoracic surgery service was consulted for expedited repair of
the pseudoaneurysm. A bovine pericardial patch repair of the aortic wall defect was performed. After careful intraoperative evaluation, due to marked
pseudoaneurysm erosion of the left atrial wall this lesion was left undisturbed
as a repair complication would be life threatening. The patient’s immediate
hospital course was uncomplicated.
This case illustrates the potential long-term complications associated
with repaired aortic rupture and the need for regular surveillance.
141
MASKED VENTRICULAR TACHYCARDIA ETIOLOGY
ATTRIBUTED TO SURREPETITIOUS DRUG INGESTION
Aiello D1, Jain N1, Celebi M2. 1LSUHSC–New Orleans, New Orleans, LA
and 2Touro Infirmary, New Orleans, LA.
Case Report: A 55 year-old white male with no past medical history was
brought into the emergency department (ED) in cardiac arrest after being found
unresponsive. Bystander cardiopulmonary resuscitation was not immediately
initiated and the patient was unconscious for over 12 minutes before emergency
medical services (EMS) arrived to find the patient in pulseless ventricular tachycardia. Advanced cardiac life support measures were initiated by EMS services
personnel and continued in the ED. Once resuscitated, his electrocardiogram
showed a sinus rhythm with QRS widening, a prolonged QTc interval, and
ST elevations in lateral leads. Hypothermia protocol was initiated and urgent
cardiac work up revealed severe global left ventricular systolic dysfunction
without obstructive coronary artery disease. His serum chemistries drawn upon
arrival to the ED during active resuscitation revealed a normal serum potassium
level. On examination, the patient had marked gynecomastia and absence of
some male secondary sexual characteristics such as facial and axillary hair. After passively re-warming the patient, he was found to have clinical brain death
and subsequently expired.
Given the patient’s examination findings as well as the ventricular tachycardia arrest and electrogardiographic signs of hyperkalemia, it was
suspected that he was taking an aldosterone antagonist. The patient’s wife
was able to confirm this after finding a stash of spironolactone and estradiol
at home. He was taking spironolactone 100mg and 100 micrograms of transdermal estradiol daily. She informed the providers that the patient was a
transsexual who was taking hormones for feminization purposes. She stated
450
that he was not seeing a physician for this, but was obtaining the medications
through an overseas online source.
It is postulated that some of the medications administered during the initial resuscitation efforts masked the hyperkalemia that caused this patient’s
cardiac arrest. This case illustrates the fact that hyperkalemia should still
be considered in those with normal serum potassium levels drawn during a
cardiac arrest after “code drugs” are administered. Moreover, clinical
findings such as electrocardiographic changes consistent with hyperkalemia
should not be ignored.
142
A DEADLY “DUAL”
Foster MB, Glancy DL, Jain N. LSU-New Orleans, New Orleans, LA.
Case Report: Dual AV node physiology is a common finding during routine
electrophysiology studies. Observing this phenomenon on a single surface
lead electrocardiogram, however, is a rare occurrence. AV nodal reentrance
is a common mechanism of paroxysmal supraventricular tachycardia. We report the case of a patient who developed AV nodal re-entrant tachycardia
(AVNRT) as the first instance of dual conduction pathways. Subsequent surface lead recordings confirmed two distinct PR intervals consistent with dual
AV-nodal physiology. A 56 year-old female with a significant medical history
of nonischemic cardiomyopathy (EF = 30%), hypertension, paroxysmal
atrial flutter, diabetes mellitus, tobacco abuse, severe COPD, and cor
pulmonale presented to the ED complaining of productive cough, dyspnea,
orthopnea, nightly PND, and worsening lower extremity edema for two
weeks. She denied chest pain, palpitations, nausea, vomiting, fevers, or
chills. Her past history was significant for a syncopal episode within the last
year attributed to atrial flutter. Her initial vital signs revealed a blood pressure
of 149/108 mm Hg, pulse of 115 beats per minute, respiratory rate of 20,
temperature of 98.6°F, oxygen saturation of 97% on room air, and BMI of
45kg/sq m. Her initial labs revealed a BNP of 848 pg/mL and a potassium
level of 3.4 mmol/L. Chest radiography revealed cardiomegaly and pulmonary vascular engorgement. Her initial electrocardiogram revealed sinus
tachycardia with a rate of 112 and a PR interval of 164 milliseconds. The patient was admitted and started on intravenous furosemide. On hospital day 4
she suddenly developed supraventricular tachycardia with a rate of 160 to
170 beats/min with a pattern consistent with AVNRT. The patient’s QRS duration was 82 milliseconds on surface electrocardiogram. Prior to treatment
the patient converted to a sinus rhythm with a curious finding. The following
surface electrocardiograms demonstrate PR intervals prolonged to 340 to
360 milliseconds. One particular electrocardiogram demonstrated alternating
1:1 PR intervals. The fast conduction pathway showed a PR interval of
160 milliseconds. The slow conduction pathway showed a PR interval of
360 milliseconds. She was started on digoxin. The patient had no more
episodes of supraventricular tachycardia during her hospital stay.
143
PURULENT PERICARDITIS: AN UNCOMMON CONDITION
IN AN UNLIKELY PATIENT
Isaac J1, Saxon JT2. 1University of Mississippi Medical Center, Jackson, MS
and 2University of Mississippi Medical Center, Jackson, MS.
Case Report: A 50 year old African American man with type 2 diabetes
mellitus presented with positional chest pain of a few days duration. The initial electrocardiogram demonstrated diffuse ST segment elevation, most consistent with acute pericarditis. Transthoracic echocardiogram revealed
normal left ventricular systolic function with a small pericardial effusion.
The patient had a mild leukocytosis and normal cardiac biomarkers. He
was discharged on colchicine and ibuprofen for treatment of acute pericarditis. However, his symptoms persisted following hospital discharge, and he
had several subsequent visits to urgent care facilities, without a significant
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
change in clinical status. One week later, he developed severe dyspnea at
rest, and worsening severity of positional chest pain. He presented to the
emergency room for evaluation. A pericardial friction rub was present on
physical examination. Repeat transthoracic echocardiogram revealed a large
circumferential pericardial effusion with echocardiographic evidence of cardiac tamponade. A pericardiocentesis was performed, with aspiration of thick
purulent fluid. A pericardial drain was left in place at that time. Cultures of
the pericardial fluid ultimately revealed methicillin-sensitive Staphylococcus
aureus. The patient was initially treated with intravenous vancomycin and
oxacillin, which was narrowed to intravenous cefriaxone alone once bacterial
sensitivities became available. The pericardial space was lavaged daily with
thrombolytic agents and sterile saline, to promote lysis of fibrin formation
and discourage formation of pericardial adhesions. The patient’s symptoms
improved dramatically following pericardiocentesis. Prior to discharge, right
heart catheterization was performed, which demonstrated constrictive pericardial physiology.
Purulent pericarditis is an uncommon condition characterized by bacterial infection in the pericardial space. The condition most often affects immunocompromised patients. Pericardiocentesis is the initial diagnostic and
therapeutic intervention. Late in the disease course, pericardial constriction
due to fibrin formation is a common finding and usually necessitates operative pericardiectomy.
Endocrinology and Metabolism
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
144
46 XX PHENOTYPIC MALE WITH AMBIGUOUS GENITALIA
AND MULLERIAN DUCT REMNANT
Ahrari B1, Diaz-Thomas A2, Sinha SK2. 1University of Tennessee Health
Science Center, Memphis, TN and 2University of Tennessee Health Science
Center, Memphis, TN.
Case Report: Although ambiguity of external genitalia is seen in 1 out of
5000 births, 46 XX disorder of sexual development (DSD) has an incidence
of 1:20000. Discovering the etiology of the DSD is challenging. Lack of
standard guidelines for evaluation and management of 46XX DSD makes
this a particularly difficult condition to address.
14yr and 2mo old ex 31 week premature African American phenotypic
male is referred for hormonal evaluation. The patient had been born with ambiguous genitalia. Initial evaluation included routine karyotype which
revealed 46XX. Primed in situ labeling after birth had shown presence of
the sex-determining region on the Y (SRY) at chromosome X P22.3, however fluorescent in-situ hybridization and chromosomal microarray later in
life did not show any evidence of SRY.
At 14yr and 4mo patient underwent pelvic and scrotal ultrasound that
revealed: 9-cm right paramedian elongated tubular structure likely a utricular
Mullerian structure as well as bilateral gonads in the scrotum (right
2.8×1.1×1.9cm with a volume of 3.15 mL and the left 3.3×0.8×2.1 cm with
a volume of 3.10 mL). Hormonal evaluation at 14yrs 2mo was sent.
Testosterone, Total 249 (>180 ng/dl)
Testosterone, Free 2.2 (pg/ml N/A)
DHT 43 (24-65 ng/dL)
AMH 19 (3-5.4 ng/mL)
17-OH-Progesterone 23 (24-175 ng/dL)
Discussion: More often than not, the etiology of 46 XX DSD is unknown. In
cases where the molecular defect can be identified SRY translocation is the
most common mutation with other possibilities including SOX-9 duplication, copy number variation of SOX3, loss of function mutations in RSPO1,
and mixed gonadal dysgenesis. Our patient reveals an inconsistent SRY analysis and testosterone levels that are disproportionately high for prepubertal
gonads. Gonadectomy is the standard of care in 46XX DSD given the increased risk of malignant transformation, however our patient’s gonads appear to be well functioning and easily palpable. The presence of a
Mullerian remnant makes the possibility of mixed gonadal dysgenesis high
and could explain the inconsistent SRY result. Testicular biopsy is planned
Southern Regional Meeting Abstracts
and perhaps this in conjunction with molecular analysis can assist with risk
stratification and long term management.
145
HIDE AND GO SEEK - A CASE OF INTRATHYROIDAL
PARATHYROID ADENOMA IN A PATIENT WITH PRIMARY
HYPERPARATHYROIDISM
Awoniyi O1, Samant S2, Gosmanov A1. 1University of Tennessee HSC,
Memphis, TN and 2University of Tennessee HSC, Memphis, TN.
Case Report: Background: In patients with primary hyperparathyroidism
(PHPT), parathyroid adenoma can rarely be located within the thyroid. Preoperative localization studies are not very reliable to allow accurate localization of intrathyroidal parathyroid adenoma; therefore proper identification is
frequently performed during surgery.
Clinical Case: 83-year-old female with a history of hypertension, hypothyroidism and osteoporosis was referred for evaluation of hypercalcemia. Laboratory
evaluation revealed total calcium level of 11.1 (8.6-10.2 mg/dl), ionized calcium
level of 6.3 (4.5-5.6 mg/dl) and PTH level of 69 (10-65 pg/mL) with normal serum 25-OH vitamin D and 24-hr urine calcium levels. Repeat investigations
showed persistently elevated calcium and PTH levels consistent with PHPT.
Thyroid ultrasound revealed multinodular goiter and a 9x3x4 mm hypoechoic
ovoid structure in the right thyroid lobe consistent with a lymph node. Sestamibi
scan demonstrated asymmetric increase in activity in the right thyroid lobe without specific location of signal. Patient agreed for neck exploration and parathyroidectomy. Extensive neck exploration including the retroesophageal groove
revealed no parathyroid adenoma and perioperative PTH levels remained elevated. After review of the sestamibi scan, right thyroid lobectomy was
performed that resulted in marked decrease in intraoperative PTH level from
131 to 21 pg/mL. Pathology evaluation showed a parathyroid adenoma within
the inferior portion of the thyroid lobe supporting diagnosis of intrathyroidal
parathyroid adenoma.
Discussion: In PHPT, about 6-16% of parathyroid glands can be located in
ectopic positions making localization and surgical procedures challenging.
The incidence of intrathyroid parathyroid glands is rare ranging between
0.7-3.6%. While the positive predictive value of routine imaging modalities
to localize parathyroid adenoma is between 90-93%, in the setting of
multinodular goiter like in our case localization can be difficult. Studies suggest that sestamibi scan is superior to ultrasound in such cases. We propose
that localization studies together with the expertise of the surgeon are required for successful resection of intrathyroidal parathyroid adenoma.
146
TNF-Α INDUCED INSULIN RESISTANCE: USE OF SIRNA TO
DEMONSTRATE ROLE OF PTEN
Bulger D1,2, Conley J2, Conner S1,2, Majumdar G1,2, Solomon S1,2. 1University of Tennessee Health Science Center, Memphis, TN and 2Veterans Association Medical Center, Memphis, TN.
Purpose of Study: The incidence of Type 2 Diabetes Mellitus (DM2) has
sharply increased, primarily due to obesity. Adipose tissue in obesity
produces higher amounts of TNF-α, which leads to chronic low-grade inflammation and insulin resistance (IR). The mechanism is still unclear. Previous genomic and proteomic studies by us on this IR have revealed
significant differences in PTEN. PTEN dephosphorylates PIP3 and induces
IR. Herein we utilize siRNA knockdown of PTEN to elucidate its role in
TNF-α induced IR.
Methods Used: With and without PTEN siRNA (25 nM)/Dharmafect 1,
H411E rat hepatoma cells were treated with insulin (10,000 μU/ml), TNFα (1.0 nM), and insulin + TNF-α. We also tested 3-hydroxypicolinate vanadium (IV) complex (VO-OHpic) (37.5 nM) and metformin (10 μM). Protein
and mRNA were extracted from the cells and analyzed by Western blot (Wb)
and Real Time RT-PCR. Levels of PTEN in treated cells were compared to
untreated control. Sensitivity or resistance to insulin were reflected by results
of p-Akt Wb.
Summary of Results: PTEN/β-actin protein levels as measured by quantitation of Wb bands are calculated as percentages of control: insulin (80%, ↓);
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
451
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
TNF-α (197%, ↑↑, p < 0.05); and insulin + TNF-α (122%, ↑). For PTEN/βactin mRNA levels as measured by Real Time RT-PCR, percentages of control were the following: insulin (65.8%, ↓↓); TNF-α (198%, ↑↑); and insulin
+ TNF-α (98%, ↓). Insulin inhibited and TNF-α stimulated PTEN levels.
Phospho-Akt/Total-Akt protein levels: insulin (230%, ↑↑, p < 0.05); TNFα (95%, ↓); and insulin + TNF-α (300%, ↑↑↑, P < 0.05). Both insulin and
VO-OHpic each prevented TNF-α induced stimulation of PTEN (p <
0.05), and the combination was additive (p < 0.05). Metformin, previously
shown to inhibit TNF-α, showed no significant effects in the current
experiments.
Conclusions: Use of siRNA PTEN provides proof of concept of a critical
role of this phosphatase in insulin signaling. Insulin inhibits and TNF-α
stimulates PTEN. An increase in PTEN inhibits insulin signaling. Unexpectedly, TNF-α stimulation of PTEN did not alleviate IR (p-Akt). Despite this
last inconsistency, our overall data supports a central role for PTEN in IR,
making it a potential therapeutic target in DM2.
147
METABOLIC BONE DISORDER IN THE
ELECTROPHYSIOLOGY LAB: AN UNUSUAL DIAGNOSIS OF
PAGET’S DISEASE
de Silva TN, Ahmed J, LeLorier P. LSU Health Sciences Center, New
Orleans, LA.
Case Report: A 59 year old man with non-ischemic cardiomyopathy underwent implantation of an implantable cardioverter defibrillator (ICD). He had
New York Heart Association class II symptoms. Echocardiogram revealed
ejection fraction of 15% and aortic valve sclerosis. Post-operative chest xray revealed coarsening of the trabecula involving the glenoid and lateral left
scapula consistent with possible Paget’s disease. The patient was referred to
Endocrinology. He reported having mild left shoulder pain for 1 year, but did
not have other bony pain or history of fractures. His children reported that he
had been recently increasing the volume on his television, but he denied
symptoms of any cranial nerve deficit. He had multiple dental caries. Skull
x-ray was negative for changes consistent with Paget’s disease. Alkaline
phosphatase and bone specific alkaline phosphatase were found to be elevated at 226 U/L and 59.6 mcg/L, respectively, consistent with Paget’s disease. Treatment with a bisphosphonate was not initiated due to his poor
dentition and risk of jaw osteonecrosis.
Paget’s disease is a disorder of bone metabolism characterized by abnormal bone remodeling. Focal areas of the skeleton undergo increased osteoclastic resorption followed by subnormal repair by osteoblasts. The axial
skeleton is preferentially affected. Approximately 30-40% of patients are
symptomatic at the time of diagnosis. Bone pain is the most common
presenting symptom. Some patients with skull involvement may present with
deafness. Most patients who are diagnosed with Paget's disease are asymptomatic and the disorder becomes apparent due to findings of an elevated serum alkaline phosphatase level or abnormal radiograph obtained for other
reasons. Cardiac involvement can include calcific valvular disease, AV
block, and high output heart failure, due to increased cardiac output secondary to high blood flow as disease becomes more widespread. Cardiac stroke
volume has been found to be higher in patients with Paget’s disease and has
been correlated with severity of bone resorption. It is possible that Paget’s
disease in our patient is contributing to his poorly controlled heart failure;
however as the extent of his disease is limited it is not likely to be the primary
cause of his heart failure.
148
PARATHYROID ADENOMA WITH SEVERE SYMPTOMATIC
HYPERCALCEMIA AND IMPAIRED KIDNEY FUNCTION
Edriss H, Mazek H, Aly M, Berdine G, Lado-Abeal J. Texas Tech University
Health Science Center, Lubbock, TX.
Case Report: We report a 49-year-old man with no past medical problems
who presented with polyuria, polydipsia, nausea, fatigue, muscle weakness,
weight loss and dehydration. His serum calcium was 15.9 mg/dl, PTH
was 568 pg/ml (nl: 15-65 pg/ml), 24-hour urinary calcium level of 460 mg
452
(nl: <320mg), blood urea nitrogen 21mg/dl, creatinine 1.2mg/dl, eGFR
43ml/min/1.73m2, alkaline phosphatase 152 (nl: 29-129IU/L), and AST 51
(nl: 5-40IU/L). His chest x-ray was normal. Sestamibi-scintigraphy showed
increased signal in the right side of the thyroid lobe. CT neck showed soft tissue
nodule just posterior to right lobe of the thyroid measuring 1.5 × 0.8 cm.
The patient was initially treated with rehydration, calcitonin, and pamidronate.
His clinical symptoms improved, serum calcium level decreased to 11.2mg/dl,
and kidney function improved within 48 hours. He underwent parathyroidectomy, and the histopathology showed a parathyroid adenoma. His PTH decreased to 7 pg/ml a few hours after the surgery.
Hypercalcemia is defined as a serum calcium level greater than 10.3 mg/dl.
Primary hyperparathyroidism (PHPT) is the autonomous overproduction of
parathyroid hormone (PTH) and often causes hypercalcemia. The most common cause of hypercalcemia of PHPT is a single parathyroid adenoma (85%).
Most patients with PHPT are asymptomatic and identified during routine care;
these patients have calcium levels that fluctuate between 9.7-11.5 mg/dl.
Severe symptomatic hypercalcemia of >14 mg/dl is a rare presenting symptom of parathyroid adenoma. PHPT or malignancy causes more than 90% of
cases of hypercalcemia. PHPT accounts for up to more than 90% in the outpatient setting and about 20% in hospitalized patients; malignancy accounts
for up to 70% of hypercalcemia in hospitalized patients.
We report this case whose presentation initially suggested malignancy
since severe symptomatic hypercalcemia of more than15mg/dl in the inpatient setting is uncommon presentation of primary parathyroid adenoma
and is usually associated with malignancy. Severe symptoms of PHPT,
known as parathyroid storm, require immediate management of the hypercalcemic crisis to reduce mortality. However, mortality remains high, even with
surgical treatment which is the definitive therapy.
149
METHIMAZOLE-INDUCED GOITEROGENESIS IN A
PATIENT WITH THE SYNDROME OF RESISTANCE TO
THYROID HORMONE
Glymph K, Gosmanov A. UT Health Science Center, Memphis, TN.
Case Report: Introduction: The syndrome of pituitary-selective resistance to
thyroid hormone (RTH) is a rare disease characterized by an increased
threshold of pituitary thyroid stimulating hormone (TSH) suppression by
thyroid hormone due to mutation in the TSH receptor gene. Antithyroid
medications were not shown to improve clinical manifestations of hyperthyroxinemia in these patients.
Case: A 63 year-old African American female presented to our endocrinology clinic in April of 2012 for second opinion on management of goiter. In
2005, she was diagnosed with hyperthyroidism due to pituitary-selective
RTH and started on therapy consisting of methimazole 15mg daily and
atenolol. On initial evaluation in 2005, TSH level was elevated at 6.964
(0.35-5.50 mIU/L) and over period of treatment with methimazole TSH
slowly increased with a peak of 24.88 mIU/L. Free thyroxine (FT4) initially
was 1.55 ng/dL (0.61-1.76 ng/dL) and normalized after initiation of
methimazole. Having achieved biochemical euthyroxinemia, she noted thyroid gland enlargement associated with progressive symptoms of dysphagia
and dyspnea. On our examination, she had a globally enlarged firm thyroid
gland with areas of nodularity in both lobes. She had TSH of 16.57 mIU/L,
FT4 of 1.10 ng/dL, and free T3 of 173 ng/dL (85-205 mg/dL). A computed tomography of the neck showed enlarged thyroid goiter with extension around bilateral sternocleidomastoid muscles and compression onto the trachea. We
thought that chronic elevations in TSH level resulted in goiterogenesis and
discontinued methimazole while continuing atenolol. In 12 months, she experienced marked clinical and radiographic improvement of the goiter size associated with TSH reduction to 1.26 mIU/L and modest FT4 increase to 2.31 ng/dL.
Discussion: Patients with the RTH develop symptoms of thyrotoxicosis due
to elevated thyroid hormone levels; therefore, it seems appealing to treat these
patients with antithyroid medications. However, in these patients thionomides
will decrease thyroxine level, which will in turn stimulate TSH production.
TSH is a growth factor whose chronic elevation predisposes to goiter formation. In our case, discontinuation of methimazole caused decrease in TSH production with resultant improvement in patient symptoms and decrease in
thyroid goiter size.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
150
A CASE OF THIAMINE DEFICIENCY PRESENTING AS
HYPOTHERMIA IN AN AIDS PATIENT
Graebert A, McDougald L, Engel LS, Masri N. LSU Health Sciences Center,
New Orleans, LA.
Case Report: Hypothermia is a condition in which the core body temperature is decreased to below 35°C (95°F).
Case: A 45 year old Hispanic male was brought to our facility after being
found lying outside on a sidewalk. According to EMS reports, the patient initially had a blood pressure of 106/60 with a pulse of 48, respirations of 18,
100% oxygen saturation on room air, a blood glucose level of 125, and
had admitted to alcohol use that day. Upon arrival to our facility, he was
found to have a core body temperature of 84.2°F. On our exam, the patient
was drowsy but arousable. He spoke but did not answer questions appropriately. He did not have any evidence of head trauma and pupils were sluggish
but reactive to light, otherwise his physical exam was unremarkable. Initial
laboratory studies revealed hemoglobin of 11.7 gm/dL and an MCV of
111.2 fL. Toxicology screen was negative. EKG changes were consistent
with Osborn waves seen with hypothermia. Blood, urine, and sputum
cultures were obtained to evaluate for possible sepsis as the cause of the
patients hypothermia and he was started on broad spectrum antibiotic therapy. TSH normal and both random and 8:00am cortisol levels were not depressed. He had a normal vitamin B12 level and a borderline deficient
folate level. The patients’ hypothermia was gradually corrected with active
external re-warming techniques. After about 12 hours of monitoring in the
ICU his core body temperature reached 96°F. Further history was then able
to be obtained and it was learned that the patient was infected with HIV.
An immunodeficiency panel was checked revealing an absolute CD4 count
of 2 cells/uL. As thiamine deficiency was supported by a thiamine level
of 22 ug/L. The patient was discharged with thiamine and folate
supplementation
Discussion: The moderate hypothermia observed in our patient is most
likely secondary to thiamine deficiency secondary to multiple etiologies including his malnourished state, AIDS and his alcohol abuse. Deregulation
at the level of the hypothalamus is specifically responsible for the hypothermia seen in thiamine deficient individuals. Currently, there are no recommendations for administration of thiamine to patients with hypothermia.
151
REFRACTORY HYPONATREMIA IN AN INFANT
Hiebert R, Marin M, Krishnan S. University of Oklahoma Health Science
Center, Oklahoma City, OK.
Purpose of Study: To investigate an uncommon cause of refractory
hyponatremia in an infant.
Methods Used: Literature and chart review.
Summary of Results: Isolated hyponatremia is an uncommon presentation in neonates. When hyponatremia is associated with hyperkalemia, it is
concerning for mineralocorticoid abnormalities. While congenital adrenal
hyperplasia is the most common cause of such a presentation, rarely a neonate may have aldosterone deficiency or resistance.
A 1 month old child was admitted to the hospital with hyponatremia
(131 mEq/L) and hyperkalemia (7.3 mEq/L) noted during a visit to her primary care physician. She was born at 33 weeks gestation and required a
2 week NICU stay for hyponatremia since birth and prematurity. At presentation she was clinically asymptomatic with adequate oral intake. Physical
examination was completely normal and did not reveal any ambiguous genitalia. She was started on florinef with sodium chloride supplements which
were titrated up to achieve eunatremia pending endocrine work up. Laboratory
work up included plasma renin activity, corticosterone, ACTH, 17 hydroxyprogesterone, thyroid function tests, and an ACTH stimulation test, all of
which were normal. Aldosterone level was elevated consistent with pseudohypoaldosteronism. She was discharged on sodium chloride supplementation
at a dose of 21 mEq/kg/day. She has experienced stable growth and development
since then.
Conclusions: Pseudohypoaldosteronism (PHA1) or mineralocorticoid resistance is an uncommon syndrome which can be further subdivided into two
forms. The autosomal recessive form is associated with severe generalized
salt loss in the skin and saliva and results from a mutation in the gene coding
Southern Regional Meeting Abstracts
the epithelial sodium channel. The autosomal dominant, renal form is less severe and results from a mutation in the mineralocorticoid receptor gene
NR3C2. The less severe renal PHA1 presents with salt loss, insufficient
weight gain due to chronic dehydration, hyperkalemia, and elevated plasma
renin and aldosterone levels. Our patient’s clinical picture is highly suggestive of the renal form of PHA1. Although unlikely given our patient’s negative sweat chloride test, the generalized form of PHA1 with a milder clinical
course cannot be ruled out. Premature infants also have physiologic aldosterone resistance, which may have been exaggerated in our case, but must be
a diagnosis of exclusion.
152
HYPOGLYCEMIA IN AN ELDERLY PATIENT-DON’T
IGNORE INSULINOMA
Jacovino CA, Powell S, Gosmanov A. University of Tennessee Health Science Center, Memphis, TN.
Case Report: Insulinoma is a rare neuroendocrine tumor deriving mainly
from pancreatic islet cells that secrete insulin. Prior studies suggested that
the diagnosis of insulinoma is difficult in elderly and it is thought that the incidence of insulinoma in the elderly persons presenting with hypoglycemia
can be underestimated.
An 81-year old African American female with a history of obesity, hypertension and hyperlipidemia and no history of diabetes presented to the emergency room with weakness. She was found to have blood glucose (BG) of
40mg/dL; hypoglycemic episode resolved after glucagon injection. After admission for short-term observation, she had another episode of hypoglycemia
with a BG of 43 mg/dL associated with C-peptide of 3.44 ng/mL (0.8-3.80)
and insulin of 73.0 μU/mL (4.2-27.9). Our evaluation showed normal adrenal
and thyroid function, hemoglobin A1c of 5.4% (<5.7%), and no evidence of
liver or kidney failure. A 72-hour fasting was initiated to exclude insulinoma.
Symptomatic hypoglycemia with BG level of 49 mg/dL occurred 18 hours
after initiation of fasting accompanied by inappropriately elevated C-peptide
of 2.63 ng/mL and insulin of 43.7 μU/mL. Her sulphonylurea panel, chromogranin A, gastrin, and calcium levels were normal. A computed tomography of abdomen showed an 18 mm hyperdense focus within the uncinate
process of pancreatic head. Patient underwent surgical resection of the tumor.
The final pathological diagnosis demonstrated insulin-secreting islet cell
tumor. During our follow up 6 months after surgery, the patient no longer
had hypoglycemic episodes and was found to have mild type 2 diabetes.
The diagnosis of insulinoma depends on the exclusion of the more common causes of hypoglycemia and based on the demonstration of raised
plasma insulin and C-peptide levels in the presence of hypoglycemia
in fasting state. Few cases have been reported atypical presentation of
insulinoma. In our patient, blood glucose levels were taken during fast confirmed biochemical diagnosis of insulinoma which was subsequently
supported by the pathology report of the excised tumor. Though insulinoma
is predominantly seen in young and middle age patients, it should be
suspected in the elderly presenting with hypoglycemia that is not explained
by other clinical processes.
153
RECURRENT MIXED-TYPE PAPILLARY AND FOLLICULAR
THYROID CARCINOMA IN A PATIENT WITH
DIGEORGE SYNDROME
Mikail J, Rincon-Subtirelu M. University of Tennessee College of Medicine
at Chattanooga, Chattanooga, TN.
Case Report: DiGeorge syndrome is a 22q11.2 deletion syndrome that
has characteristic signs and symptoms that often include neuromuscular
problems, congenital heart defects, defects in the palate and recurrent
infections. Recurrent infections are associated with a hypoplastic thymus.
Initial clinical signs include hypocalcemia at birth due to malfunctioning
parathyroid glands. Late-occurring consequences include hypothyroidism
and hyperparathyroidism. There is no literature documenting patients with
DiGeorge syndrome who develop thyroid carcinoma.
Case Report: This 13 year old female was diagnosed at three months of age
with DiGeorge syndrome. In association with her DiGeorge syndrome, she
had cleft palate and ventricular septal defect. Though, she never had
symptoms of hypocalcaemia or any immunological deficits.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
453
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Her pediatrician noted a thyroid nodule during an office visit when she
was 4 years old. An ultrasound study found the mass to be solid. She had
a left thyroidectomy performed one month later. Histology on the tissue sample was found to contain papillary carcinoma, follicular variant. There was
invasion of the capsule by the tumor and the tumor extended to the surgical margins in several areas. She subsequently had a total thyroidectomy
performed one month later. No tumor was seen in the specimen. At least
one parathyroid gland was preserved at the time of surgery. She was started
on long term thyroid hormone replacement.
After an elevated thyroglobulin level was noted in her yearly follow up at
age 13, she had a repeated scan that showed small areas of radioactivity in
the left lung and right lung. The patient received a second I131 treatment
with a follow-up scam showing small areas of radioactivity in the lower
neck and left chest wall. Following this scan, the patient has remained stable
despite new evidence of recurrence.
Conclusions: We report a new case of thyroid carcinoma in a female patient
with DiGeorge syndrome. As both DiGeorge syndrome and papillary thyroid
carcinoma, follicular variant are rare, it raises the question of whether this is
the result of a genetic tumor suppressor gene that is affected in certain
variants of DiGeorge syndrome.
thymus hypoplasia and hypoparathyroidism. A subgroup of these patients
develop some degree of psychiatry, the most frequent of which is schizophrenia. Hyperprolinemia type 1 (HPI) is caused by mutations in the PRODH
gene, which can be included in the deleted region. Here, we present a patient
diagnosed with a 2,91 Mb deletion in 22q11.2 region. The patient started
regressing at the age of 24 and had memory loss, seizures despite medication
and psychiatry. We hypothesized that hyperprolinemia, which is known to
cause psychiatry in PRODH related metabolic patients, could cause the psychiatry and seizures in our patient.
Methods Used: We did Array CGH to see whether PRODH was deleted in
this patient, we measured proline levels and searched the literature for
patients diagnosed with 22q11.2 deletion syndrome involving PRODH, to
see whether they have hyperprolinemia.
Summary of Results: ArrayCGH showed that PRODH was deleted in our
patient. The patients’ proline levels were variable but consistently elevated.
We found other patients with deletions in 22q11.2 and hyperprolinemia.
Thus, we confirmed our hypothesis.
Conclusions: Haploid insufficiency of PRODH can cause hyperprolinemia,
which might play a role in psychiatry seen in 22q11.2 deletion patients. Symptoms
of such psychiatry can be late onset. We suggest protein restriction as therapy in
these patients, to lower proline levels and therefore patient outcome.
154
A CASE OF COEXISTING DIABETES INSIPIDUS AND
CEREBRAL SALT WASTING SYNDROME SUCCESSFULLY
TREATED WITH DESMOPRESSIN AND FLUDROCORTISONE
Rivas AM, Laengvejkal P, Sotello D, Lado-Abeal J. Texas Tech University
Health Science Center, Lubbock, TX.
Case Report: Patients with central nervous system surgery or trauma can
have complex electrolyte disorders.
A 76-year-old man with a history of essential hypertension on olmesartan
presented with decreased level of consciousness caused by spontaneous
bleeding inside a non-functioning pituitary macroadenoma diagnosed with
head MRI. He underwent emergent evacuation of sella turcica. Sixteen hours
after surgery he developed diabetes insipidus (DI) with good response to
desmopressin (DDAVP). However, forty-eight hours later, he developed recurrent polyuria (128cc/hour) that didn’t respond to an increased dose of
DDVAP. Subsequently, he became volume contracted and developed severe
hyponatremia (129 mmol/L) secondary to high urine sodium excretion
(186 mmol/L). Further workup showed serum aldosterone levels <1ng/dL
and brain natriuretic peptide of 4581pg/mL; no signs of congestive heart failure or cardiac ischemia were present. Cerebral salt wasting syndrome (CSW)
was suspected, and he was started fluid hydration with physiologic saline solution without improvement. Oral fludrocortisone (0.1mg twice daily) was
added and corrected the polyuria and hyponatremia.
DI and CSW are well-known complications of intracranial surgery, but
their coexistence is unusual. In our case, the recurrent polyuria was initially
attributed to DDAVP resistance, but the lack of response to higher dosages of
DDAVP together with hyponatremia, natriuresis, high BNP and aldosterone
deficiency lead us to suspect CSW.
CSW is characterized by an abnormal release of natriuretic factors by injured brain tissue. These patients present with aldosterone depletion secondary
to an inhibitory effect of brain natriuretic peptides on the renin-angiotensinaldosterone system and decreased sympathetic stimulation of renin secretion
after acute central nervous system injury. Repletion of mineralocorticoid was
successful in the treatment of CSW in this patient. We recognize that our patient
could have had some baseline aldosterone deficiency from chronic use of
olmesaratan, which should not be overlooked. Even so, considering what is understood of the physiopathology of CSW, it is likely that fludrocortisone had an
important role on its treatment.
156
PERSISTENT HYPERTENSION FOLLOWING SUCCESSFUL
TREATMENT OF BILATERAL RENAL ARTERY STENOSIS:
A CASE OF TERTIARY HYPERALDOSTERONISM
Varughese Ag, Uwaifo G. Louisiana State University, New Orleans, LA.
Case Report: Introduction: Secondary hypertension (HTN) is known to be
common and its recognition is growing in importance due to potentially reversible causes. Renovascular HTN is the most common cause of secondary
HTN. Persistent HTN after treatment of renal artery stenosis (RAS) can be an
alerting sign. The most common etiology in this setting is coexistent essential HTN but the possibility of another secondary etiology due to renovascular HTN needs to be sought.
Case: A 46 YO Black American woman with obstructive sleep apnea (OSA)
presented with sixteen years of HTN. Patient developed hypertensive emergency and was found to have bilateral renal artery stenosis (RAS) with elevated serum aldosterone 30 ng/dl (1-21) and a plasma renin activity (PRA)
5ng/ml/hr (0.6-4.3). The RAS was due to fibromuscular dysplasia (FD)
and she underwent bilateral renal artery angioplasty without improvement
in HTN. Further work up showed an aldosterone of 12 ng/dl and PRA of
0.41 (ratio: 30). 24 hour urine aldosterone with oral sodium loading yielded
an aldosterone of 12.9ng/dl and sodium of 314 ng/dl. Adrenal computerized
tomography (CT) was normal and biochemical features typical of primary
hyperaldosteronism (PAH) post successful RAS repair. Patient started on
eplerenone 50mg daily resulting in reversal of HTN but higher aldosterone
levels (44ng/dl). These finding are consistent with PAH following prior secondary hyperaldosteronism (SAH) caused by bilateral RAS. This suggests
progression from prior SAH to autonomous tertiary hyperaldosteronism (TAH)
probably due to bilateral adrenal hyperplasia.
Discussion: PAH is one of the common causes of Secondary HTN. In PAH,
aldosterone production is inappropriately high and non-suppressible by sodium loading. Persistent HTN after treating the reversible causes of SAH
suggests zona glomerulosa autonomy called TAH. TAH is uncommon and
most commonly occurs in older patients with prior SAH due to atherosclerotic plaques of renal arteries. Her untreated OSA may have contributed to
the development of TAH following SAH.
Conclusion: Recognition of this entity, TAH, is important to enable effective
blood pressure control in patients with persistent HTN despite apparent hemodynamic correction of renovascular HTN.
155
HYPERPROLINEMIA AND PSYCHIATRY IN 22Q11.2
DELETION SYNDROME
van Asbeck E1,2, Morava-Kozicz E1,2. 1Radboud University, NIjmegen,
Netherlands and 2Tulane University, New Orleans, LA.
Purpose of Study: 22q11.2 deletion syndrome has a highly variable phenotype, despite the uniformity of the deleted region. It is associated with cleft
palate, congenital cardiac abnormalities and learning disabilities, as well as
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157
PROFOUND MUSCLE WASTING AS A PRESENTATION OF
HYPERTHYROIDISM IN AN ADOLESCENT
Walkup JD, Carlucci M, Rincon-Subtirelu M. University of Tennessee at
Chattanooga College of Medicine, Chattanooga, TN.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Case Report: A 14-year-old male presented with a progressive symmetrical
weakness of the upper and lower extremities over the course of approximately
3 weeks. This culminated in an event in which he was unable to rise out of a
movie theater seat, and had enough strength only to fall forward and crawl.
The patient reported that over the 3 weeks he had been unable to stand up from
a sitting position and was falling forward, then pulling himself to stand. Prior to
this, he had a weight loss of 74 lbs. over the preceding 7 months. This was not
initially considered problematic by the family because he was undergoing a
workout regimen to correct his obesity. They had noticed the patient was able
to eat smaller meals and were proud of this accomplishment. However, from
the patient’s perspective he was experiencing progressive early satiety and had
no real increase in muscle mass.
On exam, the only positive finding was significant muscle wasting in the
proximal muscles of the upper and lower extremities. The differential diagnosis for this patient’s muscle weakness and wasting included polymyositis,
muscular dystrophy, neurological causes, psychiatric causes (due to a history
of bullying because of obesity), and nutritional deficiency. Over the course of
his hospital admission, he was found to have abnormal thyroid function test
with a final diagnosis of hyperthyroidism, secondary to Graves’ disease. In
retrospect, he did not have vital signs abnormalities, nor thyroid enlargement
or ophthalmopathy to suggest the disease.
This case is unique because of the presentation of hyperthyroidism as
rapid development of muscle wasting without the most classical signs and
symptoms of the disease. Pediatricians should include thyroid dysfunction
when evaluating severe myopathy in the context of weight loss.
Gastroenterology
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
158
MASSIVE UPPER GASTROINTESTINAL BLEED
FOLLOWING FOUR-DAY THERAPY OF NAPROXEN AND
DOXYCYCLINE: IS IT A SYNERGISTIC EFFECT?
Gharaibeh K1,2, Brewer M1,2, Patnana S1,2, Cook-Glenn C2, Bhagat R1,2.
1
University of Mississippi Medical Center, Jackson, MS and 2G.V. Sonny
Montgomery VA Medical Center, Jackson, MS.
Case Report: Introduction: Chronic prolonged naproxen-induced gastrointestinal (GI) mucosal injury/bleeding and doxycycline pill-induced esophagitis
are well documented in literature. There is no information on the GI effects of
the two together. We report a case of massive upper GI bleeding secondary
to gastric ulcers after only 4 days of doxycycline and naproxen therapy.
Case-report: A 53-year-old male was admitted with 8 hours of bleeding
per rectum. Patient reported mild epigastric pain with nausea but denied
vomiting, hematemesis, or melena. Four days prior he was initiated on naproxen
500 mg and doxycycline 100 mg twice daily for presumed Lyme disease. On
exam the patient had tachycardia (108/min), hypotension (90/60 mmHg) and
mild epigastric tenderness. His admit hemoglobin was 7.1 g/dL (4 days prior
was 15.6). Nasogastric lavage confirmed fresh blood. In the intensive care unit
he was fluid resuscitated and initiated on pantoprazole and octreotide infusions.
He required transfusion with 8 units of blood. Esophagogastroduodenoscopy
revealed multiple ulcers and the bleeding gastric ulcer vessel was treated with
a clip. H. pylori work up was negative. Patient recovered well off doxycycline
and naproxen.
Discussion: Naproxen induced upper GI bleeding with <14 days of therapy
is rare (0.026%) while doxycycline causes pill esophagitis and rarely gastritis. Our patient had received both together for only 4 days when he developed a massive upper GI bleed. Our report raises the possibility of
synergistic effect between naproxen and doxycycline leading to “accelerated”
GI injury. Naproxen, besides inhibiting cyclooxygenase, also disrupts the hydrophobic phospholipid protective mucosal barrier. In our patient we hypothesize this loss of protective barrier with caustic effect of acidic doxycycline
lead to upper GI bleed with only 4 days of therapy.
Conclusion: The combination of naproxen and doxycycline should be prescribed with care. The possibility of the two drugs synergistically increasing
the risk of accelerated upper gastrointestinal injury associated bleeding needs
further exploration.
Southern Regional Meeting Abstracts
159
A RARE CAUSE OF CHRONIC DIARRHEA IN AN
ENDOSCOPIC PERCUTANEOUS GASTROSTOMY PATIENT:
A CASE REPORT AND LITERATURE REVIEW
Mankongpaisarnrung C, Dumrongmongcolgul N, Phisitkul S, Roberts J, Rakvit A.
Texas Tech University Health Sciences Center (TTUHSC), Lubbock, TX.
Case Report: Introduction
Oropharyngeal dysphagia can be caused by external compression, such
as cervical spondylosis, by luminal disorders in upper aerodigestive tract,
and by neuromuscular weakness, such as previous stroke. The long-term
management goal is to avoid parenteral nutrition and to maintain enteral
feeding by placing a feeding tube surgically or endoscopically. However,
complications, such as bleeding, infection, peritonitis, transverse colon injury, and buried bumper syndrome, can occur.
Case report: A 66-year-old man with COPD had oropharyngeal dysphagia
from cervical myelopathy. He was status post 360° cervical fusion of C3-C7
with C4 corpectomy and required percutaneous endoscopic gastrostomy
(PEG) tube placement due to difficulty swallowing. He was transferred from
an outside facility with 30 pound involuntary weight loss over 6 months and
chronic diarrhea 30-40 times a day after PEG tube placement. He described
the tube feeding as “running right out of him”. He had adhered strictly to dietary
recommendations. He denied nausea, vomiting, and fever. Physical examination
was unremarkable except for cachexia. Abdominal examination showed PEG
tube in place; no erosion or infection was seen. Initial labs revealed mild anemia
with Hb of 11.5 gm/dl. He underwent computed tomography of abdomen for
initial evaluation to assure the integrity and the position of the PEG tube. CT
scan abdomen revealed PEG tube balloon tip was mal-positioned in the
transverse colon; this was confirmed with gastrografin fistulogram.
Intraoperative findings showed dislodged and misplaced PEG tube in mid-distal
transverse colon.
Discussion: We are reporting a rare complication with a PEG tube, which
resulted in profuse and intractable diarrhea. Recalcitrant diarrhea after PEG
tube placement should alert the clinician to this complication. The PEG tube
probably traversed and migrated into an adjacent structure, and the profuse
diarrhea was caused by colonic irritation. Aspirating feculent material from
the PEG tube can help recognize this complication early. CT scan and
fistulogram can make this diagnosis. The only treatment option is surgical
correction.
159A
PANCREATIC BURKITT’S LYMPHOMA SHOULD ALERT
PROMPT TREATMENT?
Mankongpaisarnrung C1, Kijsirichareanchai K2, Soape M1, Rassameehiran S1,
Rakvit A1. 1Texas Tech University Health Sciences Center (TTUHSC),
Lubbock, TX and 2University of Nebraska, Omaha, NE.
Case Report: Burkitt’s lymphoma (BL) is an aggressive NHL with a rapid
doubling time of 24-48 hours. Its primary location is within the abdomen
(60-80%), followed by the head and neck. The histopathology is classically
described as a starry sky appearance. Pancreatic lymphoma (PL) can be divided into primary and secondary PL by the involvement of other lymph
nodes (secondary). PL as an extra-nodal involvement can present without
symptoms, with painless jaundice, or with pancreatitis.
A 45-year-old healthy Hispanic man presented with a 2-week history of
LLQ pain and a 20-pound weight loss. He also reported a progressive swelling of neck. History of heavy smoking and IVDU were noted. Physical exam
found a 5 × 5 cm matted right anterior cervical and supraclavicular LAD and
LLQ tenderness. His labs were normal except elevated amylase (78 U/L), lipase
(177 U/L), LDH (2201 U/L), and positive HIV. CEA and CA19-9 were normal.
MRCP of the abdomen confirmed a 2.5-cm pancreatic head mass causing mild
distal PD dilatation. CT scan also found an 8.6-cm mass in the right paratracheal
node. Subsequent EUS showed a round, poorly defined, hypoechoic mass in the
posterior mediastinum, 3.7 cm irregular and hypo-anechoic mass in the pancreatic head with portal vein invasion but no CBD dilatation. The dilated 2.4mmPD was noted in the body and tail of pancreas. Pancreatic adenocarcinoma
(PA) was preliminarily suspected from EUS. FNA showed anaplastic cancer
cells, favoring a hematopoietic tumor. CT-guided lung BX confirmed BL. He
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
455
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
lost to follow up but presented two weeks later with SVC syndrome and pericardial effusion. Cyclophosphamide-based chemotherapy (CMT) regimen was
started but he died of tumor lysis syndrome soon after CMT began.
BL can grow rapidly if left untreated. Since pancreatic BL and PA require
different therapeutic approaches, accurate diagnosis is crucial for prompt
treatment. With the use of EUS, the findings may mimick PA. However, clinical correlation and cytology increase the diagnostic yield. We underscore the
importance of detecting secondary PL from BL, which may reflect a significant tumor burden and lead to poorer outcomes. Hence, prompt aggressive
workup and treatment once the definite diagnosis confirmed is essential.
160
A CASE OF PLUNGING GIST PRESENTING AS
INTERMITTENT DYSPHAGIA
Nakshabendi R1,2, Clark M1,2, Munoz JC2,1, Nakshabendi I2. 1University of
Florida, Jacksonville, FL and 2University of Florida, Jacksonville, FL.
Case Report: The patient is a 71-year-old woman with a past medical history of melenoma who presented to the hospital with progressively worsening intermittent dysphagia mainly for solid foods over the past year.
Associated symptoms included reflux without significant weight loss. She
typically had to chase solid food down with liquids in order to prevent any
type of impaction. She has never had any food impaction which has required
a food bolus removal. EGD 6 months prior was unremarkable.
Endoscopy revealed a large 4 to 5 cm subepithelial lesion in the midesophagus at around 30 cm. The lesion appears smooth with normal overlying smooth tissue. There were no obvious ulcerations. Biopsies were not
taken at that time. CT scan of the chest confirmed the presence of a large submucosal mass in the distal esophagus. Repeat biopsy was performed with
EUS and biopsy. No extra-esophageal masses were identified and biopsy
was proven for the gastrointestinal stromal tumor. The patient was started on
neoadjuvant chemotherapy with Gleevec with future plans for surgical resection.
EGD Biopsy result
Esophageal mass, FNA: Spindle cell neoplasm consistent with a gastrointestinal stromal tumor with necrosis. Comments: An immunohistochemical
stain for CD117 (C-Kit) is positive within the spindle cells. An immunohistochemical stain for HMB-45 is negative. This pattern of staining is consistent with a gastrointestinal stromal tumor.
Tumor is visible when it is plunged into the esophagus.
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161
EOSINOPHILIC GASTROENTERITIS AND SYSTEMIC LUPUS
ERYTHEMATOSIS; A RARE ASSOCIATION
Omer AA, Abdel Karim T, Akhter SR, Islam A, Ahmed M, Ali A. Texas
Tech Univ Health Sciences Center, Amarillo, TX.
Case Report: A 40 year old male presented to our hospital with a 3-month
history of nausea, vomiting, abdominal distention and mild diarrhea. He denied
any GI symptoms prior to his current illness. On physical exam, he was found
to have positive shifting dullness. Laboratory data revealed a white count of
3100 cells/mm3 with eosinophils 0.4% and serum albumin of 2.4 g/dL. Positive serological markers were: ANA at 1:320; anti-dsDNA at 1:1280; antiSSA antibodies at 144 AU/ml; anti-SSB antibodies at 68 AU/ml; a low C3
(54mg/dL) and C4 (14mg/dL) levels .Stool culture, ova and parasites were
negative. Abdomino-pelvic CT scan showed diffuse small and large bowel
wall thickening and ascites. Endoscopic evaluation revealed an edematous
gastric wall with many areas of submucosal hemorrhage, edematous gastric
antrum and edema of the colonic wall. Nonspecific chronic gastritis, chronic
duodenitis and normal colonic mucosa were found on endoscopic biopsies. Exploratory laparotomy yielded 3 liters of ascitic fluid with 24% eosinophils. An
excisional biopsy of the appendix revealed chronic serositis changes and focal
eosinophilic infiltrates in the lamina propria and the submucosa. Liver biopsy
demonstrated an increase in sinusoidal eosinophilis. When methylprednisilone
was given, his symptoms improved dramatically and his ascites resolved.
Discussion: Eosinophilic gastroenteritis (EGE) is categorized as either primary (idiopathic) or secondary. In the former type, an allergy basis is very likely.
This is derived from the positive allergic diathesis history in some patients, peripheral and tissue eosinophilia and elevated total and food-specific IgE levels.
Secondary EGE has been associated with inflammatory bowel disease (IBD),
hypereosinophilic syndrome, celiac disease, vasculitic disorders, drugs and parasitic infestations. More recently, an association of EGE with SLE has been
reported. The relationship between these two disease entities is unclear, but an
underlying immunological link is conceivable. Corticosteroids remain the cornerstone of treatment for EGE, with a reported response rate of 90%. In conclusion physicians should not ignore the differential diagnosis of EGE in SLE
patients with unexplained gastrointestinal symptoms.
162
AN UNCOMMON CAUSE OF DRUG-INDUCED
PANCREATITIS: DOXYCYCLINE
Rassameehiran S, Mankongpaisarnrung C, Rakvit A. Texas Tech University
Health Sciences Center, Lubbock, Texas, Lubbock, TX.
Case Report: Acute pancreatitis is one of the common gastrointestinal
causes of hospitalization. Although the incidence of drug-induced pancreatitis is low, this disease is associated with substantial morbidity and mortality.
Identification of the offending medication and history taking are very important. Common offending medications include asparaginase, azathioprine,
didanosine, pentamidine, and valproic acid. This is a case of an uncommon
medication causing acute pancreatitis.
An 82-year-old African American woman with previous cholecystectomy presented with severe epigastric and right upper quadrant abdominal
pain associated with nausea for 1 day, happened after 12-day usage of doxycycline for cellulitis. The rest of her medication list remained unchanged.
She was hemodynamically stable. Physical examination revealed mild tenderness over the epigastric area without peritoneal signs. Laboratory findings
revealed an elevated lipase level of 2,222 IU/L, elevated amylase level of
1,122 IU/L, unremarkable triglyceride, unremarkable total bilirubin level of
0.3 mg/dl, normal alkaline phosphatase level, and normal calcium level. Computed tomography of abdomen and pelvis revealed enlargement of head and uncinate process of the pancreas with peripancreatic stranding. Ultrasonography
of abdomen showed normal size of common bile duct at 3.7 mm. She denied
any alcohol use. She was diagnosed with acute pancreatitis. Doxycycline was
stopped and she was treated conservatively with uneventful hospital stay.
Drug-induced pancreatitis from doxycycline is uncommon. There have
been conflicting data about the effect of doxycycline on the pancreatitis.
Some reports describe a protective effect of doxycycline by inhibiting
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
pancreatic phospholipase A2, which reduces the tissue damage in acute pancreatitis. However, doxycycline has also been reported as a causative agent in
drug-induced pancreatitis. In our case, we considered doxycycline-induced
pancreatitis after eliminating other causes of pancreatitis. Antibiotic-associated
pancreatitis usually has a mild course, and discontinuation of the offending
medication is the mainstay of treatment.
Health Care Research and Quality Improvement
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
163
THE SAFEMED PROGRAM: EARLY LESSONS FROM
A CARE TRANSITIONS PROGRAM WITH A FOCUS ON
MEDICATION MANAGEMENT
Bailey JE1,2, Nault J1, Waters T2, Speck P3, Rashed S4, Ugwueke M5. 1University of Tennessee Health Science Center (UTHSC), Memphis, TN; 2UTHSC,
Memphis, TN; 3UTHSC, Memphis, TN; 4UTHSC, Memphis, TN and 5Methodist
Le Bonheur Healthcare, Memphis, TN.
Purpose of Study: Previous research suggests that patients with multiple
chronic conditions (MCC), high inpatient utilization, and polypharmacy
are at high risk for readmissions and adverse outcomes. Early research
suggests that care transitions programs with an emphasis on medication management have potential to result in better care and outcomes at lower cost.
This study seeks to determine whether the SafeMed Program, a care
transitions program emphasizing medication management funded through
the Center for Medicare and Medicaid Innovation, significantly impacted readmission rate in the vulnerable population served.
Methods Used: This quasi-experimental prospective study assessed impact
of the SafeMed Program on hospital readmission rates during first six
months of program implementation. Patients were required to meet full eligibility criteria including: 1) hospital admission to either of two hospitals serving readmission hotspots, 2) two or more chronic health conditions, 3) high
previous inpatient utilization, 4) Medicare and/or Medicaid, and 5) age > 18.
Comparison population included individuals who were screened as eligible
for participation. Main outcome measure was hospital readmission rate calculated using the UnitedHealth Group (UHG) counting methodology and
utilization reports generated using an internal data management system for
the region-wide hospital network.
Summary of Results: 57 enrolled patients had a 34% readmission rate
whereas 76 comparison patients had a 46% readmission rate, however, this difference was not statistically significant at p<.05. These findings suggest that
enhanced medication management is necessary but not sufficient to significantly impact the readmission rate.
Conclusions: Preliminary results suggest that enhanced medication management is necessary but not sufficient to significantly impact readmission rates.
Care transitions programs emphasizing medication management have great potential but will likely need to provide robust symptom triage and care coordination
services to address complex social needs to have major impact on readmissions.
164
QUALITY IMPROVEMENT IN DIABETES FOOT AND EYE
CARE: A MULTIDISCIPLINARY APPROACH
Day B, Dreyer S, Furr A, Hill J, Myrick WM, Obih U, Price S, Tenini J, Saha A.
Carolinas Medical Center, Charlotte, NC.
Purpose of Study: The purpose of our study is to improve the care of
patients with diabetes through two quality improvement (QI) initiatives:
one designed to increase the rate of annual diabetic foot examinations (goal
of 80% in one year), and another to increase the rate of annual retinopathy
screening (goal of 50% in one year).
Methods Used: We first formed a multidisciplinary team dedicated to ambulatory QI initiatives. We employed frequent Plan-Do-Study-Act (PDSA) cycles
and Lean methodology as our main tools for improvement. Weekly electronic
data capture with run chart tracking helped guide the effectiveness of our
Southern Regional Meeting Abstracts
interventions. For the foot exam project, successful interventions included:
having patients remove their shoes, having monofilaments visible, resident education at conferences, transparency in data publishing, and nursing-physician
communication via a laminated card. For our newer eye exam project, our first
PDSA intervention involved a nursing-driven protocol for documenting and
requesting eye exams.
Summary of Results: We began with an estimated baseline rate of 28% of
patients up to date on their annual diabetic foot exam. Within two months we
were able to significantly increase our weekly foot exam rate, achieving our
stated goal of 80%. However, success proved difficult to sustain, and along with
incoming interns in July our rates dropped to 55.1%. With continued efforts, we
have slowly and reliably increased our weekly rate back towards our stated goal
once again. Initial eye exam data shows a baseline of 36% of patients documented as up to date. After our first PDSA cycle, we have not yet increased
our rate of complete eye exams and are planning the next PDSA cycle.
Conclusions: Improving diabetic health maintenance is a challenging but vital task in primary care. In our study, we focused on improving rates of annual foot examinations and screening for retinopathy. Our foot exam project
showcases successful quality improvement interventions which are currently
proving sustainable and are likely easily replicable in similar primary care
settings. Our eye exam project is still underway although we have not
shown improvement here yet, we hope to apply the lessons learned from
the foot exam project to achieve sustainable success in this area as well.
165
STANDARDIZATION OF WRITTEN HANDOFFS ON AN
INTERNAL MEDICINE SERVICE
Edwards AL1, Shewchuk R2, Taylor B1, Rodriguez M1, Kraemer R1, Curry W1.
1
University of Alabama at Birmingham, Birmingham, AL and 2University of
Alabama at Birmingham, Birmingham, AL.
Purpose of Study: New duty hour standards have raised concern regarding
increased numbers and quality of handoffs. We examined whether a standardized
written handoff would improve resident satisfaction, perceived utility, and subjective efficiency of the handoff process.
Methods Used: Quasi-experimental design (pre-/post-intervention). Single
academic internal medicine service (1,000-bed hospital) (Mar-May 2013).
Residents used a standardized, word processor-based, written handoff template. A five-item survey assessed resident satisfaction, utility, and subjective
efficiency of the handoff process at baseline (pre-) and at 3-month follow-up
(post-). Respondents selected agreement with written handoff: 1) utility to
accomplish daily duties, 2) ability to accomplish daily duties in a timely
manner, 3) utility when answering cross-cover questions, 4) satisfaction,
and 5) use of the standardized template almost always (last item, only at follow-up survey)(5-point Likert scale; 5 = strongly disagree, 1 = strongly agree).
Respondents also estimated time spent composing written handoffs. We compared pre- and post-intervention responses with the Mann–Whitney U test.
Summary of Results: Of 48 individuals surveyed, 15 responded at baseline
(31%) and 20 at 3-month follow-up (42%). The standardized handoff template was used almost always by 95% of respondents. Respondents reported
increased written handoff utility in accomplishing daily duties, increased
utility when answering cross-cover questions, and increased overall satisfaction with the written handoff process post intervention (Table). Unchanged
were accomplishing daily tasks in a timely manner (P = 0.11) and time spent
composing written handoffs (P = 0.36).
Conclusions: Standardization of the written handoff improved satisfaction
and perceived utility with the handoff process without decreasing efficiency.
Assessing and measuring perceived effectiveness at the local level is an important step to engage stakeholders for implementation and dissemination of
national handoff recommendations.
Comparison of Outcomes
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
457
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
166
WHAT DO COMPLAINTS TEACH US?
Foreman E2, Monroe K1, King A4, Bradley B3, Miller R4. 1Univ of Alabama,
Birmingham, AL; 2University of Alabama, Bham, AL; 3Childrens of Alabama,
Bham, AL and 4Childrens of Alabama, Bham, AL.
Purpose of Study: Pediatric Emergency Departments routinely receive
complaints from families. Reviewing these complaints can serve as a guide
to improve services.
Methods Used: A convenience sample of complaints received by patient
relations for an 11 month period (august 1,2012 through july 14th 2013)
were reviewed. The reviewer read each complaint and extracted data from
patient relations database and hospital computer databases. Complaints were
categorized as relating to wait times, attitudes of staff, diagnosis, quality of
care, system errors and “nothing was done.” All data were entered into Excel.
Summary of Results: 82 complaints were reviewed. There were 49 Caucasian (59%)31 African American, and 1 Latino families represented. Insurance distribution was 61% (n=52) private insurance, 33% (n=27) medicaid;
2 no insurance,1 missing data. Most complaints occurred from patients seen
during the evening shift 49% (n=42) (day 29%(n=24) and overnight 18%
(16)). 18 were admitted 55 discharged and 9 left without treatment. We
employ a five level triage category system with triage level 1 being the highest
acuity and level 5 being the lowest. Only 2 of the complaints came from the
families of patients triage level 1, nine from level 2, twenty one from level 3 with
the highest percentage coming from level 4 (42) and only nine from level 5.
Mondays and Thursdays accounted for 39% of total complaints. Of the 82
complaints, the categories were “rudeness/attitude of staff ” (n=24); parents felt
“nothing was done ”(n= 13),complaint about our system of care (NP/residents
involved, follow up nurse, signage)(n=12) prolonged wait (n=10); parents felt
quality of care was poor (n=8)insurance issues (n=5) and other such as TV
didn’t work, angry that child was admitted to hospital, couldn’t find chewable
prescription medication (n=7) and parent felt diagnosis was incorrect (n=3).
Conclusions: Evening shift accounted for most of complaints- adequate
staffing during these times and having patient relations available is key. Better communication with families regarding diagnosis and treatment plan
should be emphasized. The fewest complaints came from the highest and
lowest levels of triage categories.
167
SKIN CANCER PROTECTION: WHAT DO PATIENTS KNOW
AND DO?
Gutierrez M, Ross R, Harris K, Arnold C, Davis T. Louisiana State University Health Sciences Center, Shreveport, LA.
Purpose of Study: Identify patient skin cancer protection knowledge and
practices and determine if this varies by income.
Methods Used: Adult patients in a private dermatology clinic were given a
structured interview evaluating skin cancer protection knowledge and behavior, demographic information and literacy level.
Summary of Results: Of 230 patients enrolled, 68% were female, 14% were
African American, 84% Caucasian, and 2% other. Patients ranged in age
from 18 to 86, mean age 48; 14% had low literacy (<9th grade reading level).
63% of patients had a yearly household income of >$50,000, 26% had
≤$50,000, 11% declined to answer. Almost all patients (94%) had heard of
melanoma, 85% knew signs indicating a person might have skin cancer.
78% remembered having a sunburn that caused them to blister or peel as a
child. Only 60% reported a doctor had ever talked to them about skin cancer.
Over half of white patients (54%) reported they had used a tanning bed.
Among all patients, those with higher income were more likely to believe
that it is possible to get skin cancer from tanning beds (95.7% vs. 86.8%;
p=0.003) and that tanning beds are not safer than tanning in direct sunlight
(90.1% vs. 79.4%; p=0.023). Lower income patients were more likely to indicate they were unsure if tanning beds were safer than direct sunlight
(14.7% vs. 4.3%). 92.9% higher income patients believed individuals can
get skin damage in cold weather versus 80.6% with lower income
(p=0.024). The majority of patients with both higher income (99.4%) and
lower income (94.1%) indicated they knew ways to protect themselves from
skin cancer. The most commonly known methods were wearing sunscreen
(91%), avoiding the sun (45%), and using protective clothing (44%). About
half indicated that they had sunscreen in a moisturizer (54%) or Chap Stick
458
(51%). There was no significant difference between income groups in sunprotection behavior. 47% indicated they “often to always” wear sunscreen
when in the sun for an hour and 75% indicated using sunscreen this often
on their children.
Conclusions: Most patients were aware of skin cancer risk and protection
methods but less likely to engage in protective behavior. Higher income
patients were more aware of risks than their low income counterparts.
168
COMMUNITY HEALTH WORKER’S PERSPECTIVE ON
BARRIERS TO CARE DELIVERY AMONG LATINOS WITH
POORLY CONTROLLED DIABETES
Koshy L, Kenya S, Okoro I, Carrasquillo O. University of Miami Miller
School of Medicine, Miami, FL.
Purpose of Study: Community health workers (CHWs) are a promising approach to improve care delivery to minority populations. Among Latinos, diabetes is an area where the role of CHWs has been identified as an important
strategy. While many studies describe barriers to care that CHWs can help
overcome, few have focused on challenges that CHWs may experience when
caring for such populations.
Methods Used: We conducted key informant interviews with three CHWs
who were delivering care to Latino patients enrolled in a NHLBI sponsored
randomized trial. Each CHW managed a caseload of about 30 patients. We
used semi-structured interviews that included 10 open-ended questions on
barriers and challenges experienced by CHWs. Interviews were recorded,
transcribed and coded to help us identify recurrent themes mentioned by
all three CHWs.
Summary of Results: We were able to categorize responses into three overarching themes that centered on barriers at the patient, CHW and health system level. At the patient level, CHWs noted that despite being of a similar
background as their patients, some cultural ideologies such as machismo,
ageism and sexism at times hindered their interactions. In addition, when
patients did not follow through on agreed plans for diet, exercise, glucose
self-monitoring, and taking medications as prescribed, the CHWs personally
felt discouraged and disappointed. When patients remained non-adherent to
basic diabetes care, CHWs realized this elicited feelings and attitudes within
themselves that were not conducive to successful interactions with that patient or others. Lastly, at the health system level, the primary barrier mentioned was the disrespectful attitudes in the hospitals and clinics by some
the physicians and staff towards the CHWs. One example was providers asking
CHWs to remain quiet and not participate in any portion of the clinic visit.
Conclusions: These key informant interviews provide us with a richer understanding of the complex barriers faced by CHWs caring for Latino
patients. By identifying such barriers, we can develop improved training
and protocols for CHWs that can better address these patient, CHW and
health system level challenges.
169
NEONATAL PATIENT SAFETY
McFalls K1, Ambalavanan N2, Bidez M3, Burke D3. 1UASOM, Homewood,
AL; 2UAB, Birmingham, AL and 3UAB, Birmingham, AL.
Purpose of Study: Medical errors are a major cause of morbidity and mortality, and little advancement has been made in eliminating serious medical
errors. This research study was designed to explore and expose medical
errors within the setting of a regional neonatal intensive care unit (NICU).
Methods Used: The first step of this project was to create a list of common
medical errors occurring within the NICU using a random patient safety audit process. This was accomplished using 2 standard questionnaires, one for
nurses and one for physicians, listing possible causes of adverse outcomes.
Summary of Results: Data consisted of responses collected from 50 of each
survey. We found that 30% of the nurse surveys and 66% of the physician
surveys identified potential or actual patient safety issues. The main safety
issues were radiology studies and equipment/medical devices for nurses
and intermittent suction not set to < 80 as well as inability to locate consultations for physicians.
Conclusions: Future steps are to construct a plan of action to eliminate the
potential for errors happening in the NICU and improve patient safety. This
technique may be useful in other health-care settings.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
170
BACTERIAL ISOLATES IN COMMERCIAL YOGURT
COMPARED TO PROBIOTICS
Panchavati PK2, Hassoun A1. 1Alabama Infectious Diseases Center, Huntsville,
AL and 2Huntsville Hospital, Huntsville, AL.
Purpose of Study: Clostridium difficile associated diarrhea (CDAD) is one
of the most common causes of healthcare associated infection. It’s associated
with significant mortality and morbidity. Antimicrobial use is the main risk
factor for CDAD. If taken with Antibiotics, Probiotics have been shown to
reduce the risk of CDAD.
Methods Used: Sixteen different brands of commercial yogurts were cultured for 5 days to asses’ amount of bacterial growth and type of isolate of
each type. We choose different brands and fat content, those with and without probiotics, those frozen, popsicle and regular as well as flavored. Also,
we reviewed bacterial isolate of 16 commercially available probiotics capsule. In addition, we reviewed chart of patients from 1/2012- 4/2013 in our
practice that have been on intravenous or oral antibiotic therapy and advised
to take daily cup of yogurt of any type, to assess their rate of CDAD.
Summary of Results: All commercial yogurt grew at least one bacterial isolate except frozen yogurt popsicle did not grow any organisms. Each one had
significant growth of more than 100,000 cfu/ml. the following organisms
were found: Lactobacillus acidophilus, Enterococcus durans, Streptococcus
thermophilus, Streptococcus viridians, other Lactobacillus species as well.
Seven of these grew 2 different organisms and one grew 3 different organisms.
There was no difference between those with or without probiotics, and no significant difference between brands. No gram negative organisms grew in any of them.
Buttermilk showed similar growth. In comparison, Commercially sold probiotics
randomly selected as we did with yogurt, 11 of them contained species of Lactobacillus and Bifidobacterium. Four had Saccharomyces boulardi and one contained
Streptococcus thermophilus. Patient charts reviewed who discussed with them
use of yogurt while on antimicrobial therapy showed only five developed CDAD.
Conclusions: Commercially available yogurts have at least one organism
and can be considered as probiotic. Further studies are warranted to test the
effectiveness of yogurt for prevention of CDAD.
171
PATIENT SATISFACTION WITH HURRICANE PLAN GIVEN
TO HEMATOLOGY-ONCOLOGY PATIENTS AT CHILDREN’S
HOSPITAL IN NEW ORLEANS
Raulji C, Velez M, Gardner R. LSUHSC/Children’s hospital of New orleans,
New Orlenas, LA.
Purpose of Study: Because of increased frequency and intensity of hurricanes in the New Orleans region, many patients being followed at HematologyOncology clinic at Children’s Hospital (CHNOLA) are relocated temporarily
during these periods. Each year at the beginning of Hurricane season patients
are given a hurricane plan in form of a questionnaire, which asks them about
pertinent patient identification data; their evacuation plan in case of a hurricane; if there is a hospital nearby; and if they know which pharmacy they will
be using. They are also asked to have an updated copy of their roadmap with
them. This information is then scanned to a flash drive along with their roadmap for treatment and is available to the staff in case of evacuation. The families are given information about where the clinic will be held in case of evacuation.
Our goal was to assess the barriers to providing healthcare to HematologyOncology patients by evaluating the effectiveness of our hurricane plan given
to the families.
Methods Used: We distributed a survey to patient’s families at the time of
clinic visits. The patient or the family representatives were asked to participate in the study. We excluded patients who started following up with us after
the hurricane season ended. The questionnaire included questions about diagnosis, previous evacuations, evacuation plan, factors that figured in their decision to
evacuate, and if they had treatment plan and medicine available to them, etc.
Summary of Results: The majority of patients (73%) evacuated during previous hurricane seasons and most (77%) had a plan for where they were going
to evacuate. The most important factor influencing that decision was availability of a children’s hospital nearby. Although a majority (71%) had medicines with them to last for 2 weeks, a significant number (43%) did not have
treatment plans available with them and many (28%) did not have emergency
Southern Regional Meeting Abstracts
contact information for staff as well. Most patients (78%) found information
given to them by staff helpful.
Conclusions: Most of our patients found the hurricane action plan implemented at CHNOLA helpful. We suggest implementation of similar plans to
other departments as well. We are in process of comparing our present data to
data available before implementation of the action plan.
172
ADULT SICKLE CELL DISEASE AND DEPRESSION
Schumann SO, Brownfield E, Glass R, Khuu T, McDermott W. Medical University of South Carolina, Charleston, SC.
Purpose of Study: Sickle cell disease burdens patients with significant morbidity and mortality from birth, unlike other chronic medical conditions such
as many forms of cancer and heart disease. Compounded by a lack of access
to resources, social support systems, and coping skills, sickle cell disease
often results in high utilization of emergency medical resources and prolonged
inpatient stays. Small, non-randomized studies have correlated comorbid depression with severity of sickle cell disease. Other studies have shown the rate
of depression in sickle cell disease to be similar to that of other chronic medical
conditions (18% - 44%) compared to that of the general public (9%). There
are currently no recommendations for the routine screening or treatment
of depression in the care of adult sickle cell disease. Our project aims were
to determine the rate of depression in MUSC’s sickle cell clinic and identify
undiagnosed or undertreated depression.
Methods Used: In August 2012 a dedicated “Sickle Cell Clinic” was opened
at MUSC to treat the acute and chronic complications of sickle cell disease
in an effort to provide comprehensive care for this population. The fifty
patients who were MUSC’s highest utilizers of emergency and inpatient
medical services were enrolled. Upon enrollment the following were completed: past psychiatry history, medication history and PHQ-9 questionnaire.
A score of > 9 on the PHQ-9 scale was used to identify undiagnosed and under
treated depression.
Summary of Results:
- 35 of the 50 patients enrolled underwent a formal intake
- 31 of these 35 (88.5%) patients completed the PHQ-9 questionnaire
- 34.3% (12/35) of these patients carried a past history of depression,
and 58.3% (7/12) of these patients were receiving treatment
- 22.9% (8/31) of patients scored > 9 on the initial PHQ - 9 scale and of
these patients 5 (62.5%) did not have a history of depression
Conclusions: 22.9% of patients known to be high utilizers of the health care
system and enrolled in an Internal Medicine Sickle Cell Clinic had
undertreated or undiagnosed depression. Over half of these patients had a
new diagnosis of depression. A significant portion (15/50 - 30%) did not
complete the initial intake. Screening for depression should be included in
treatment guidelines for patients with sickle cell disease.
Hematology and Oncology
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
173
EXTRAGONADAL GERM CELL TUMOR:
A RAPID GROWER
Anazia G, Jones N, Yu M, Billeaud C, Englert D, Engel LS. LSU Health
Sciences Center, New Orleans, LA.
Case Report: A 22-year-old man presented to the emergency room after
acute onset of cough 3 days prior associated with severe weakness. On initial
physical exam, he was afebrile, normotensive, but in mild respiratory distress, and mildly tachypneic at 32 respirations per minute. His oxygen saturation was 98% on room air. He had decreased breath sounds and dullness to
percussion on the right. Testicular exam was unremarkable. Labs were remarkable for a slight leukocytosis, mild normocytic anemia, and hypoalbuminemia. Alpha fetoprotein (AFP) drawn on initial presentation was elevated at
860. β-hCG and CEA were within normal limits. Chest X-ray revealed complete opacification of the right hemithorax. CT of the chest with IV contrast
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
459
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
demonstrated a large right-sided pleural effusion with right-to-left mediastinal
shift, and numerous heterogeneously-enhancing pleural based lesions, highly
concerning for neoplasm. An ultrasound of the scrotum and testicles was
obtained and showed a small left-sided hydrocele, but no masses. Thoracentesis was performed and revealed 1,800cc of bloody fluid and biopsy was
suggestive of either a synovial sarcoma or a poorly-differentiated germ cell
tumor. One week following discharge, the patient developed increasing pain
and shortness of breath. Repeat CT of the chest showed that the tumor had
grown to occupy the entire right hemithorax, with shift of the mediastinal
structures to the left. Due to his rapid clinical deterioration, he was intubated
and started on empiric chemotherapy with etoposide, ifosfamide, and cisplatin, even before a final diagnosis was made. His tumor responded well
to chemotherapy. Repeat biopsy was obtained and was consistent with a
germ cell tumor most compatible with yolk sac origin.
DISCUSSION: Mediastinal germ cell tumors are aggressive, rapidly growing tumors, typically found in younger men. Standard treatment consists of
platinum-based chemotherapy, usually followed by surgical resection of residual disease. Due to their rapidity of cell growth, these tumors are typically
very responsive to chemotherapy.
174
TUMOR OF THE GLOMUS APPARATUS, EASY
TO DIAGNOSE EASIER TO MISS
Bitar C, Sammour R. University of Balamand, Beirut, Lebanon.
Case Report: A 50 y.o male presents with a 5-year history of subungual lesion over the left thumb. The lesion was painful upon pressure and cold exposure, and failed multiple rounds of onychomycosis therapy. Physical
examination revealed a tender solitary subungual longitudinal macule with
a reddish-blue discoloration on a hyperkeratotic, dystrophic nail (A, B).
MRI showed 9×7.5×5 mm rounded, well circumscribed subungual lesion
with a characteristic low signal on T1 (D), hyperenhencement on T2 (C),
and intense enhancement following gadolinium (E).
Glomus tumor is a benign neurovascular tumor originating from the
neuromyoarterial glomus apparatus. It classically affects middle-aged women,
but can also occur in men and other age groups. Glomus bodies are mainly
localized in the fingernails were 75% of the tumors occur . The diagnosis is
clinical and based on the triad of pain, cold hypersensitivity and tenderness
on pressure. MRI can detect tumors as small as 2 mm, with a sensitivity of
90%. Treatment is surgical.
175
INTRAVASCULAR LARGE B-CELL LYMPHOMA
OF THE DUODENUM: AN UNEXPECTED DIAGNOSIS
Chowdry RP1, Janbain M1, Safah H1, Vander Velde N1, Schmieg J2, Mayhall G2.
1
Tulane, New Orleans, LA and 2Tulane, New Orleans, LA.
Case Report: Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal diffuse large B-cell lymphoma (DLBCL). It has an insidious presentation, often mimicking embolic or vascular pathology, and it
almost always has an aggressive course. The majority of cases are found incidentally at autopsy. IVLBCL is characterized by the presence of malignant
lymphocytes exclusively within the lumen of small vessels. It commonly involves the skin or central nervous system (CNS). Involvement of other organs
is exceedingly rare, with a paucity of reports described in the literature. Primary
gastrointestinal manifestations of IVLBCL are very rare. We present the first
reported case of IVLBCL involving the duodenum. A 59 year old previously
healthy African-American male presented with a one month history of increasing abdominal girth, nausea and vomiting. Computed tomography scan
of the abdomen showed thickening of the proximal duodenum. The patient
was taken to endoscopy for tissue biopsy. He was found to have a friable
duodenal mucosa with circumferential thickening. Microscopic examination
of the biopsied duodenal tissue revealed extensive necrosis, however no atypical or malignant duodenal cells were seen. Instead, an intravascular lymphoid
infiltrate was found with large, atypical, mononuclear B-cells expressing CD20
and CD79a. There was no evidence of adjacent DLBCL within the biopsied
tissue. These findings were most consistent with intravascular large B-cell
lymphoma. No other suspicious lymph nodes were found on imaging. The
patient underwent staging workup with bone marrow biopsy and lumbar
puncture and there was no evidence of lymphoma involvement of the bone
marrow or CNS. Chemotherapy with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone (R-CHOP) was initiated after review of the
literature and guidelines. The patient underwent 3 cycles of R-CHOP after
which he had radiographic evidence of response with decreased duodenal
thickening. He also received prophylactic high dose methotrexate for a high
risk of CNS progression. The subtle manifestations of this rare entity make
it a diagnostic and therapeutic challenge and we hope to increase awareness
with this unique case.
176
GERM CELL TUMOR
Dhillon N, Castillo E, Puligothram S, Loch M. LSU New Orleans, New
Orleans, LA.
Case Report: 22-year-old African American male presented to the ER with
SOB and abdominal pain. He was recently found to have a right lung/mediastinal
mass with biopsy result as spindle cell sarcoma versus germ cell tumor in the
other facility. For further evaluation the path was forwarded to Mayo clinic pathologist as well as referral was made to our oncology clinic.
Before patient could make it to the clinic, he presented to the ED. He was
found to be tachycardic and hypoxic, had a rapid response in the ED and was
admitted to ICU.
CT chest showed marked mass effect on the great arteries displacing
them into the right hemithorax secondary to extensive right lung mass. This
mass crosses the midline and contacts the anterior margins of the aorta. The
right main pulmonary artery is encased by mass. Additionally, there is question of opacity within the lumen of the right pulmonary artery which is suspicious for tumor thrombus.
Oncology was consulted. Though the pathology was not very clear at that
point as well patient had very poor performance status. It was discussed with
the patient and his mother that the outcome is death without any treatment
but there is a chance that his tumor is germ cell, and they are usually very
chemo responsive. Knowing that the chemotherapy itself can likely kill
him, and he may not have a treatable tumor. He and his mom were willing
to proceed.Patient was started on VIP chemotherapy, per multidisciplinary
consensus it was decided not to intubate patient electively. While on chemotherapy patient was given tumor lysis prophylaxis. He did have SVTs as well
as became more hypercapnic resulting in intubation on day 13. He was
extubated within a week. His condition started to improve and was stepped
down to floor. He was given second cycle of VIP on the floor. While on
the floor patient started tolerating PO intake as well as physical therapy
460
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
worked with him. He was discharged home soon after that. Before cycle 3,
patient had staging scan that showed dramatic response to therapy and
marked improvement in performance status.
The point of presenting this case to put emphasis on the fact that germ
cell tumors are very chemo responsive and even when patient’s performance
status is low; trial of chemotherapy should be given.
177
DOUBLE HIT LYMPHOMA
Dhillon N, Castillo E, Puligothram S, Reske T. LSU New Orleans, New
Orleans, LA.
Case Report: 59 years old female was transferred from outside facility to
neurosurgery as she presented there with lower extremity weakness. MRI
of the lumbar and thoracic spine showed disc bulge at the level of L3-4, 4-5,
L5 and S1, with compression of the cord at the level of L3 and 4. The patient
also has a large mediastinal mass involving multiple structures and invading
the spinal cord with extension into the foramen compression of the spinal cord
at multiple levels. Neurosurgery did decompression laminectomy as well as tissue biopsy of the mass Patient was discharged soon. Pathology came back as
high grade B cell lymphoma, favor B cell lymphoma unclassifiable, with
features intermediate between diffuse large B cell lymphoma and Burkitt lymphoma. Patient was called back in the hospital. FISH studies detected both an
IGH/BCL2 gene rearrangement and a MYC gene rearrangement in the malignant B cells, as well a gain of one to two copies of the BCL6 gene in a subset
of the malignant B cells (but no BCL6 gene rearrangement). Chemotherapy
with R-EPOCH was initiated. After cycle 1, patient started to have progressive
cough especially whenever she will swallow. On imaging studies it was found
that, as lymphoma started to resolve it resulted in development of large tracheoesophageal fistula. Eventually 12cm x 23mm fully covered self-expanding metal
stent was placed. Patient so far has received total of 3 cycles of R - EPOACH
and is doing well.
The point of presenting this case is to put emphasis on the new WHO
classification, which now includes unclassifiable lymphoma. Double hit lymphomas represent a subset of B cell lymphoma, unclassifiable, with features
intermediate between diffuse large B cell lymphoma and Burkitt lymphoma,
and usually harbor both an IGH/BCL2 gene rearrangement and MYC gene
rearrangement. In addition to these two gene rearrangements, double hit
lymphomas frequently demonstrate a complex karyotype, which might account for the gains of the BCL6 gene seen in a subset of the malignant B
cells. Double hit lymphomas are usually aggressive neoplasm, and most
cases are associated with a poor prognosis regardless of treatment. Based
on clinical significance and the fact that no other solid diagnostic tools are
available to identify DH lymphomas, it seems advisable to test all diffuse
large B-cell and related lymphomas for MYC and other breakpoints.
178
IMMUNE THROMBOCYTOPENIC PURPURA DURING
MAINTENANACE THERAPY OF PRE-B ACUTE
LYMPHOCYTIC LEUKEMIA
Erker CA, Sandler E. University of Florida, Jacksonville, FL.
Purpose of Study: Immune Thrombocytopenic Purpura is a common hematologic disorder in children, but is uncommon in the setting of malignancy.
There is almost no literature on ALL therapy leading to a compounding diagnosis of ITP. We share our unique experience of 3 rare patient courses
who develop ITP during their maintenance therapy of ALL, including the
suspect causes and difficulty treating these patients for both ALL and ITP.
Methods Used: Retrospective case series using pediatric patients who developed ITP during their therapy for ALL.
Summary of Results: Patient one’s ITP resolved when he finished ALL therapy. However, patient’s two and three have had success with standard ITP
therapy with IVIG, but they continue to have intermittent low platelets and
are currently still undergoing ALL therapy.
Conclusions: Although rare, patients who have persistently low platelet
counts during treatment for ALL must have ITP in the differential and their
treatment will have to be adjusted.
Southern Regional Meeting Abstracts
179
PRIMARY THYROID LYMPHOMA: DIFFUSE LARGE B-CELL
TYPE WITH INCIDENTAL PAPILLARY THYROID
CARCINOMA IN A PATIENT WITH HISTORY OF CHRONIC
LYMPHOCYTIC THYROIDITIS
Hosiriluck N, Mankongpaisarnrung C, Dumrongmongcolgul N, Nugent K.
Texas Tech Health and Sciences Center, Lubbock, TX.
Case Report: Diffuse large B-cell lymphoma of thyroid is a rare aggressive
thyroid malignancy. Papillary thyroid carcinoma is the most common type of
thyroid cancer and carries an excellent prognosis. The synchronous development of both malignancies is extremely rare.
A 79-year-old man with history of hypothyroidism presented with an enlarging neck mass; a thyroid ultrasound revealed hypoechoic soft tissue
distorting the size and configuration of both lobes (8.9 x 4.4 x 4.6 cm of right
lobe and 7.1 x 3.6 x 3.6 cm of left lobe) and thickening of isthmus. Fine needle
biopsy showed atypical cells. A subtotal thyroidectomy was performed without lymph node dissection. Pathological studies showed diffuse large B cell
lymphoma with background of chronic lymphocytic thyroiditis and papillary
carcinoma with negative margins and no capsular invasion. CT scan showed
multiple subcentimeter lymph nodes in the mediastinum and multiple retroperitoneal lymph nodes, the largest 1.5 cm. Bone marrow aspiration and biopsy did
not reveal any tumor. He was staged as IIIE, received rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, but he could only tolerated five
cycles of therapy. Follow up imaging demonstrated clinical remission.
Primary thyroid lymphoma is very rare, especially advanced stage which
occurs in only 2-7% of cases. Simultaneous occurrence of primary thyroid
lymphoma and well differentiated thyroid carcinoma is uncommon, but the
association of both type of cancer with Hashimoto’s thyroiditis, can lead to
this situation. The prognosis of the two diseases is different because of poor
prognostic factors for B-cell lymphoma including male sex, advanced age
and stage, diffuse large B-cell type, ECOG score, greater tumor size, rapid
clinical growth, and intolerance to chemotherapy. Due to its aggressive
course, patients with Hashimoto thyroiditis who present with thyroid
nodules/masses must have prompt imaging and tissue diagnosis. Treatment
must be prioritized by the diagnosis that carry the worst prognosis.
180
MALE GYNECOMASTIA DUE TO BLADDER CANCER
Kanji A, Raizada M, Islam A, Ahmed M. Texas Tech University Health
Sciences Center, Amarillo, TX.
Case Report: Bladder cancer is the most common malignancy involving the
urinary system. Urothelial (transitional cell) carcinoma is the predominant
histologic type in the United States, where it accounts for 90 percent of all
bladder cancers. Painless, gross, and episodic hematuria is the most frequent
presenting manifestation of bladder cancer. Hyperestrogenemia and gynecomastia in males due to bladder cancer is rare. In this report, we present a case
of an adult male with gynecomastia and nipple pain due to high-grade
urothelial carcinoma of the bladder neck.
A 74 year old male presented with right nipple pain and gynecomastia.
An ultrasound of the breast was done which reported normal tissue. Several
blood tests conducted at the same time revealed a beta-hCG of 95 mIU/mL
and an estradiol of 90.4 pg/mL. He developed hematuria shortly after, which
prompted CT scans of the head and abdomen, as well as a prolactin level. CT
scans showed a normal brain and empty sella but blood test revealed an elevated prolactin of 29.1 ng/mL. No abdominal abnormalities were noted on
CT scan. Further investigation of the hematuria via cystoscopy revealed a
bladder tumor in the superior aspect of the bladder neck. A transurethral resection of the bladder and pathological examination showed high-grade
urothelial carcinoma with invasion into muscularis propria, lymphovascular
and perineural invasion. Although symptoms of hematuria improved following resection, his gynecomastia progressed, and he developed increasing
right nipple pain. Repeat imaging for staging showed pulmonary metastasis.
Bladder cancer typically does not present with a hyperestrogen state.
Hyperestrogenemia and gynecomastia in men related to cancer are usually
due to a paraneoplasm of testicular cancers of choriocarcinoma, teratomas,
and embryonal carcinomas. An ectopic production of hCG from a urinary
bladder urothelial carcinoma is a rare cause of gynecomastia. The ability
to differentiate in the direction of syncytial trophoblasts in a urothelial
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Southern Regional Meeting Abstracts
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carcinoma may have a genetic background. Though there have been few
cases in the past of a bladder cancer with high levels of hCG, it leaves a possibility of treatment aimed towards blocking hCG via pharmacotherapy.
181
BREAKING BAD: A CASE OF MALIGNANT MELANOTIC
SCHWANNOMA
Milner CP, Hamilton R. University of Mississippi Medical Center, Jackson, MS.
Case Report: Melanotic schwannoma is a very rare tumor, accounting for
less than 1% of primary nerve sheath tumors. These tumors, once thought
to behave benign, originate from the neuroectoderm and are characterized
by melanotic pigmentation in the cytoplasm of neoplastic Schwann cells.
A 55-year-old African American man, with a history of a partially
resected spinal melanotic schwannoma, presented to his neurosurgeon with
complaints of left upper extremity weakness. Given his history of a previous
incomplete resection of a C5-6 mass approximately 1.5 years prior, patient
underwent spinal imaging which confirmed the presence of a cervical mass.
He subsequently underwent partial tumor resection of the C5-C7 spinal tumor in conjunction with posterior cervicothoracic stabilization with rib autograft. Pathology was reviewed by Mayo Clinic, and found to be positive for
Vimentin and S-100 immunoperoxidase staining and negative for GFAP and
Cytokeratin-A immunoperoxidase staining. Pathology was confirmed to be a
recurrent melanotic schwannoma. These tumors have not been associated
with neurofibromatosis, and therefore NF1 and NF2 gene testing was not
obtained. Further imaging revealed areas of metastatic disease in the lung,
liver, and bones. He underwent radiation therapy to his cervical spine, but
had a poor response to therapy. While awaiting systemic chemotherapy, patient underwent another surgical debulking due to further symptoms of pain
with recurrent left upper extremity weakness and evidence of impending
cord compression on imaging. Systemic chemotherapy was initiated with
doxorubicin, ifosphamide, dacarbazine, and mesna to attempt to gain disease
control. During the second cycle, patient developed ifosphamide neurotoxicity, and is currently preparing to start a cycle of gemcitabine and docetaxel.
These tumors have been described as slow growing and previously benign,
but recent case reports emphasize the malignant behavior of this disease. The
rarity of these tumors leaves much to be understood about the treatment and
management of this malignancy. The standard of care is complete surgical resection, but little is known about the roles of radiation and chemotherapy in
the management of unresectable disease. This case highlights a rare tumor
and its malignant behavior while presenting a treatment dilemma.
182
183
THE GIST OF IT
Nagireddy S, Khansur T. University of Mississippi Medical Center, Jackson, MS.
Case Report: A 48 year old African American male with no significant past
medical history presented with diffuse abdominal pain to the ER. A palpable
mass, lead to CT scan of the abdomen, which showed a 28 X 20 cm mass
arising from the stomach. He underwent image guided biopsy revealing a
spindle cell neoplasm strongly positive for CD 117 (C-KIT), negative for
smooth muscle antigen and S-100, consistent with GIST. The initial staging
PET/CT showed a necrotic 15 x 23 x 30 cm mass in the left upper quadrant
with SUV of 10.9 and a 1.5 cm metastatic lesion in the liver with SUV of 8.
Following multi-disciplinary review, the mass was deemed marginally resectable as it was encroaching the liver hilum. He was started on neoadjuvant
therapy with Imatinib. A repeat PET/CT obtained at 3 weeks to assess the response showed decrease in the size of the mass to 11 x 18 x 27 cm and a drop
in SUV to 1.6. After 3 months of the Imatinib therapy he underwent partial
gastrectomy. He is continuing on Imatinib and tolerating it well. There are
plans to reassess the liver and ablate any residual disease if it fails to completely regress.
GIST represents 85% of mesenchymal neoplasms of gastrointestinal
tract. Approximately 85-90% of GIST harbor C-KIT mutation that result in
activation of the tyrosine kinase. 3-5% carry PDGFRA proto oncogene.
GISTs are common in stomach followed by small intestine. They commonly
present with abdominal pain, GI bleeding or palpable mass. GISTs are highly
462
PET avid as tyrosine kinase augments the glucose transport protein signaling.
Acute changes in the metabolic activity precede the anatomical changes, so
PET is useful to assess response to Tyrosine kinase inhibitors. Surgery
remains the main stay of therapy for primary GIST. In borderline resectable
states the use of neoadjuvant Imatinib with down staging increases the likelihood of complete resection. If the tumor proves resistant to Imatinib, second
line therapy with Sunitinib and Regorafenib is available. Here we present a rare
giant GIST, that was borderline resectable at diagnosis. We assessed the response to pre-operative TKI therapy with a PET scan. The down-staged tumor
was amenable to surgical resection. This case illustrates the potential use of
neoadjuvant imatinib therapy and PET/CT to assess the response to this drug.
184
DIFFUSE LARGE B CELL LYMPHOMA OF
LATISSIMUS DORSI
Nagireddy S, Elkins S. University of Mississippi, Jackson, MS.
Case Report: A 46 year old African American female came to the ER with
complaints of back pain and mass in the right flank area for duration of
4 weeks. It lead us to do CT of the chest and abdomen which showed a mass
of size 9 × 6.5 × 13.5 cm arising from latissimus dorsi and MRI was recommended for further delineation of the mass. MRI of the back showed 9.6 cm heterogeneously enhancing mass with area of necrosis. A soft tissue sarcoma
was suspected and a core biopsy was done. Pathology came back as high grade
B-cell lymphoma consistent with diffuse large B cell lymphoma (DLBCL). Immunohistochemistry showed positive CD20, CD 79a, PAX 5, Ki 67 was 90%
positive. She also underwent a PET/CT, which showed a hypermetabolic mass
in the right flank with SUVof 10.6 with no other abnormality, so she was staged
as IA extra nodal. Then she was started on chemotherapy with CHOP-R (Cyclophosphamide, Vincrsitine, Prednisone, Doxorubicin and Rituximab). A PET/
CT scan was done after 4 cycles, which showed decrease in the size of the mass
to 5 × 2 × 8 cm with a decrease SUV of 1.1. Chemotherapy with CHOP-R was
continued and PET/CT was done after 6 cycles which showed a mass with size
of 7.4 × 2.7 cm with no hypermetabolic activity. After completion of chemotherapy she underwent radiation to the mass, total dose of 4000cGy in 20 fractions.
She finished chemotherapy & radiation with out any difficulty and now she is
under observation.
Non Hodgkin lymphoma (NHL) is the common form of lymphoma and
DLBCL being more common form of NHL. Although DLBCL is common
in the lymph nodes, it can arise in the extra nodal sites like intestine, bone,
breast, liver, skin and central nervous system. Primary skeletal muscle non
Hodgkin Lymphoma is a rare tumor accounting for only 0.5% of all lymphoma. When skeletal muscle is involved, common sites are upper arms, pelvis and glutei. MRI and PET/CT are the main modes of imaging. Standard
treatment is chemotherapy combined with radiation. Tissue diagnosis with
histopathology and immunohistochemistry is still crucial in making exact diagnosis which can avoid unnecessary surgeries. Here we present a rare case
of DLBCL which involved latissimus dorsi muscle and treated her successfully with chemotherapy and radiation.
185
BREAST CANCER PATIENT ON CHEMOTHERAPHY
PRESENTING WITH PARTIAL SMALL BOWEL
OBSTRUCTION: CAN IT BE CLOSTRIDIUM DIFFICILE?
Neppalli AK1, Devarakonda SS1, Raley M2, Marion J1. 1Feist Weiller Cancer
Center / LSU Health Shreveport, Shreveport, LA and 2LSU Health Shreveport,
Shreveport, LA.
Case Report: Introduction: Clostridium difficile colitis is a well-known disease entity with typical presentation of acute watery diarrhea with lower abdominal pain, low-grade fever and leukocytosis, which commences during or
shortly after antibiotic administration due to alteration in normal gut flora.
We present a case of chemotherapy associated clostridium difficile colitis,
in a patient with no antibiotic use in the previous 3 months, presenting with partial small bowel obstruction possibly aggravated by the use of antidiarrheal
medications.
Case: A 76-year-old Caucasian female with stage IV Breast cancer, who has
been on oral Capecitabine (Xeloda) for 3 months, presented to the
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
emergency room at outside hospital with nausea, vomiting and abdominal
pain. Plain radiograph and computerized tomography of the abdomen were
consistent with partial small bowel obstruction, and she was transferred to
LSU for further management. Further questioning revealed that the patient
had several weeks of diarrhea since starting Capecitabine and had been taking Imodium as needed at home. She denied any diarrhea for 2 days prior to
presentation. Vital signs showed that she was normotensive, tachycardic, and
afebrile. On exam she was mildly distressed with signs of dehydration and had
normal bowel sounds. Labs were significant for leukocytosis (WBC count of
16.87 with left shift), AKI (Creatinine 2.2, with baseline of 0.7). Nasogastric
tube was placed due to signs of partial small bowel obstruction, along with
aggressive IV hydration and electrolytes replacement. Given the history of
chronic chemotherapy associated diarrhea, use of Imodium and Leukocytosis,
Clostridium difficile assay was ordered. This was positive for both C. Diff antigen and toxin. She was started on Metronidazole and her symptoms subsided
over next few days.
Discussion: Clostridium difficile associated diarrhea can be seen in patients
receiving chemotherapy due to disruption of normal mucosal barriers, even
with out prior antibiotic use. It is rare with a few cases reported in literature.
A high index of suspicion is warranted to make a timely diagnosis in patients
receiving chemotherapy.
186
LIKELY GIANT CELL CARCINOMA OF THE LUNG BY
PLEURAL FLUID CYTOLOGY EVALUATION
Oncale M1,2, Kimbrell H3,1, Barnhill M2,1. 1Tulane, New Orleans, LA;
2
Tulane, New Orleans, LA and 3Tulane, New Orleans, LA.
Case Report: A 75-year-old female ex-smoker with diabetes, hypertension,
and renal disease presented with dyspnea and weakness. She noted weight
loss, dry cough, and back pain for one month.
Acute respiratory failure and hemodynamic collapse prompted intensive
care unit admission. Chest computed tomography noted right lung field/
bronchial collapse, pleural effusion, and a 3x1.5cm hilar mass. There were
lytic bone lesions and abnormal foci in the liver and adrenal gland concerning
for metastases. Pleural fluid cytology revealed metastatic carcinoma consistent
with pulmonary sarcomatoid carcinoma, likely giant cell subtype.
She required ventilatory and vasopressor support; tissue diagnosis or staging
could not be done. She expired four days after admission. Autopsy was declined.
Sarcomatoid carcinomas (SC) are defined as poorly differentiated nonsmall cell lung carcinomas (NSCLC) with sarcomatoid differentiation or true
sarcoma components. SC comprises 3% of lung cancers, is more common in
males and smokers, and presents at a mean age of 59 years. Lesions are peripheral and metastases are similar to other NSCLC. SC have a poor prognosis; 6-month survival of less than 27%.
Giant cell carcinoma (GCCL) is a sub-category of SC composed only of
giant tumor cells and can only be diagnosed on excisions or autopsy. Without
autopsy, it is unknown whether our patient truly had GCCL. However, her
pleural fluid had a pure population of discohesive, bizarre cells, an inflammatory background, and neutrophil emperipolesis by tumor cells, characteristic
of GCCL. The lack of tumor cells with an epithelioid appearance suggests
that the giant cells likely made up a significant proportion of the tumor.
Little information on SC treatment exists. Cases with platinum-based
chemotherapy noted poor response. There are cases of 800-day survival with
gefitinib in females with epidermal growth factor receptor mutations. Even
with surgery in early disease, most patients die within 18 months. Radiation
has no role in SC treatment.
We present a case of the rare entity of SC, likely GCCL that is unique as
our patient was elderly, was female, and had a central lesion. As in prior
cases, our patient presented at diagnosis with distant metastases and rapidly
decompensated.
187
CHORIOCARCINOMA WITH METASTATIC BRAIN LESIONS
Paccione R, Hutchins K, Johnson D, Engel LS, Lillis R. LSU Health Sciences
Center, New Orleans, LA.
Case Report: We describe a case of metastatic choriocarcinoma with brain
lesions and the importance of its inclusion in the differential diagnosis of
young men with testicular masses and neurological symptoms.
Southern Regional Meeting Abstracts
CASE: A 28 year old Hispanic man transferred to our hospital after initial
evaluation demonstrated an intracranial mass with midline shift. He complained of worsening frontal headaches with concurrent nausea and vomiting
for four days prior to presentation. He also reported a painless testicular mass
which had increased in size over the last four months. At the time of our evaluation, his vital signs were: Blood pressure 104/42 mmHg, Pulse 88 bpm,
Temp 98.4 °F, Resp 16 /min, BMI 25.18 kg/m2. Pertinent findings on physical
exam included a 10 cm x 10 cm firm, non-tender left testicular mass. Gait
abnormalities included a slight limp with a dragged left foot. Visual acuity
was 20/20 OS, 20/40 OD. He had a peripheral field defects at his left lateral
and lower visual fields. MRI of his brain showed a single lesion, 3.4 cm ×
3.7 cm × 3.8cm with 12 mm midline shift. Initial testicular US showed a
13.5 cm × 12.3 cm × 10.5 cm heterogenous hypoechoic testicular mass. Further
imaging with cat scan revealed multiple pulmonary metastases bilaterally
and liver metastases. Initial beta-hCG and AFP were both significantly elevated. Craniotomy with resection of his brain metastases was performed and
pathology revealed a malignant neoplasm with features consistent with choriocarcinoma. He was diagnosed with a nonseminomatous germ cell tumor stage
IIIC, poor risk. Chemotherapy with Bleomycin, Etoposide, and Cisplatin
(BEP) was initiated. His Beta-hCG, AFP, LDH decreased. An orchiectomy
was scheduled.
DISCUSSION: Choriocarcinoma is the most malignant tumor of gestational
trophoblast origin. Although a traditionally curable neoplasm, metastatic brain
lesions significantly worsen prognosis. However, if there is a high index of suspicion for this neoplasm, increased the overall survival has occurred with aggressive surgical intervention and advances in chemotherapy and radiotherapy.
Neurological symptoms in conjunction with a testicular mass should raise suspicion for this neoplasm with the possibility of metastatic disease.
188
ACQUIRED HEMOPHILIA TYPE A IN AN YOUNG PATIENT
WITH ACQUIRED IMMUNE DEFICIENCY SYNDROME AND
REMOTE HISTORY OF LYMPHOMA: A STEP-BY-STEP
APPROACH TO SUCCESSFUL MANAGEMENT
Pasam A, Devarakonda SS, Nair B. LSU Health/ Feist Weiller Cancer Center,
Shreveport, LA.
Case Report: Background: Acquired hemophilia is relatively rare in young
patients, usually seen in patients >65 years of age, with an incidence of
0.2 to 1 per million per year.
Case: We report a case of a 48 year old man who was diagnosed with acquired hemophilia type A. He was initially admitted with complaints of sudden onset swelling, bruising, ecchymoses and intractable pain in both legs.
Ultrasound doppler ruled out DVT, CT scan showed soft tissue swelling in
bilateral calves. PT was 13.1, INR was 1.04, and aPTT was 71.9 sec. Mixing
studies were suggestive of presence of a delayed inhibitor and factor VIII activity was <0.6 (50-150%). Factor VIII inhibitor levels were 432 Bethesda units
(very high, Normal value <1.0) Of note, patient has a history of HIV/AIDS,
on anti-retroviral therapy and has a history of anaplastic large B cell lymphoma
treated with R-CHOP in 1998 and has been in remission. With careful consideration towards his immune status and precariously high inhibitor levels, we
treated him with concurrent cyclophosphamide 150 mg/day for 2 weeks and
rituximab 375 mg/m2 weekly for 4 weeks and prednisone started at 1 mg/kg/
day initially and was tapered off slowly. Patient had remarkable improvement
and had no further bleeding signs, pain resolved and aPTT slowly improved towards normal, with most recent value 24.8.
Discussion: Acquired hemophilia involves spontaneous development of
autoantibodies against factor VIII molecule, leading to its inhibition. Etiology remains uncertain in most cases. It can be life threatening and should
be suspected in cases of soft-tissue and systemic bleeding episodes with no
apparent etiology. Hemarthroses are uncommon in this condition, unlike hereditary hemophilia. Workup includes mixing studies initially, followed by
factor assays and factor inhibitor levels. Treatment strategies are based on
the acuity and severity of bleeding manifestations, with goals of controlling
active bleeding and eliminating the inhibitors, to achieve remission. Activated Factor VIIa or prothrombin complex needs to be administered in case
of active bleeding. Prednisone, cyclophosphamide and rituximab can be used
in various combinations to cause selective B-cell depletion.
© 2014 The American Federation for Medical Research
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463
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
189
HEPATOCELLULAR CARCINOMA TWENTY YEARS AFTER
BONE MARROW TRANSPLANTATION FOR FANCONI
ANEMIA
Ramirez LE. Tulane University, New Orleans, LA.
Case Report: Fanconi Anemia (FA) is a rare genetic disorder leading to
bone marrow failure as well as cutaneous, musculoskeletal, and urogenital
congenital abnormalities. The FA phenotype is caused by molecular defects
in the pathway that regulates DNA repair. Patients with FA are at increased
risk for development of hematologic and solid tumor malignancies. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure for
FA. We present a case of hepatocellular carcinoma after HSCT for FA.
A 41 year old Caucasian man presented with right upper quadrant abdominal pain, 20 pound weight loss, nausea and vomiting, and decline in
functional status over the past seven months. At 21 years of age he had an
HSCT from his HLA identical brother for treatment of FA. Past medical history included hypospadias, severe chronic obstructive pulmonary disease,
and diabetes mellitus. He had an aunt with FA. He lived with his wife and
daughter, and previously worked in construction. He had a 30 pack year history of smoking, occasional alcohol consumption, and no illicit drug use.
Physical examination revealed short stature, bilateral brachydactyly, wheezing in bilateral lungs, abdomen firm to palpation in right upper quadrant
and diffusely tender with enlarged liver. Laboratory examination revealed
INR 1.3, AST 170, ALT 61, alkaline phosphatase 455, albumin 2.7. Computed tomography revealed an enhancing mass in the right hepatic lobe
(12x8x7cm) and numerous lesions scattered in the right and left hepatic
lobes, all consistent with hepatocellular carcinoma. There were numerous
pulmonary nodules consistent with metastatic disease, and portal vein thrombosis. Alpha-fetoprotein measured 9314. He was Child-Pugh class A at diagnosis, and was considered for palliative treatment with Sorafenib. His clinical
condition rapidly declined and he died within six weeks of diagnosis, prior to
initiation of treatment.
HSCT as treatment for FA is only curative for the associated hematologic
manifestations. Patients with FA are still at increased risk of liver tumors and
squamous cell carcinomas after HSCT. The risk of solid tumors increases
with age, and 30% of patients develop a solid tumor by age 45. This case
highlights the need for surveillance for solid tumor malignancies in patients
with FA, even after HSCT.
190
INTRACRANIAL HEMORRAGHE IN A PATIENT RECEIVING
ECULIZUMAB FOR TREATMENT OF ATYPICAL
HEMOLYTIC UREMIC SYNDROME
Ruiz MA. Louisiana State University Health Sciences Center, New Orleans, LA.
Case Report: Background: Eculizumab, a terminal complement inhibitor,
was recently approved for treatment of Atypical Hemolytic Uremic syndrome (aHUS) in adults. This drug has revolutionized treatment with improvement in clinical symptoms and laboratory abnormalities associated
with aHUS (thrombocytopenia and microangiopathic hemolytic anemia).
This appears to be a safe drug with few side effects including, hypertension,
and mild infections
Case report: We report the case of a 26 year old patient from England with
diagnosis of atypical hemolytic uremic syndrome that was started on
Eculizumab six months prior to be hospitalized in our institution
Patient was admitted for sudden loss of conciousness Patient developed a
severe case of intracranial hemorrhage. Initial vital signs when patient was
admitted were stable and mild hypertension (145/85) was detected . No other
medical problems were diagnosed. No history of drug abuse was elicited.
There was not history of congenital vascular abnormalities or central nervous
system aneurysms in the family. Patient underwent testing which showed a
frontal lobe hemorrhage. MRA did not detect any vascular anomaly. Laboratory values were within normal limits including toxicology testing. In view of
the absence of any other causes, his severe intracranial hemorrhage was attributed to Eculizumab
Discussion: Side effects associated with Eculizumab have been reported and
include, headaches, hypertension, mild infections among others. To our
464
knowledge there are no reports in the literature of eculizumab and severe intracranial hemorrhage
Conclusion: Eculizumab may cause intracranial hemorrhage and patients
should be closely monitored. Further reports and studies are needed to demonstrate this association.
191
AN ATYPICAL PRESENTATION OF PRIMARY PULMONARY
ADENOCARCINOMA
Salih H1, Gomez-Sanchez C1,2, Frost J1,2. 1University of Mississippi, Jackson,
MS and 2G.V. Sonny Montgomery V.A. Medical Center, Jackson, MS.
Case Report: Adenocarcinoma cases represent 34% of primary lung cancers
in men. Of all primary lung adenocarcinoma metastasis cases, 35% are
reported to metastasize to the adrenal glands. However, only 2-4% of all cancer patients have a cancer in which primary location of origin can not be
identified. We present a case of a patient with negative imaging for pulmonary mass who presented with a large adrenal mass and pathology demonstrating adenocarcinoma of pulmonary origin.
A 70 year old black male with Hypertension, Diabetes Mellitus type 2
and Gout presented with unintentional weight loss of 70 lbs over 5 months.
An initial physical exam was unremarkable. Work-up was initiated with
chest/abdomen/pelvis contrast CT demonstrating a large right adrenal mass.
ACTH was not suppressed making Cushing’s unlikely. Aldosterone level was
normal. Urinary metanephrines were only slightly elevated and not in range
of a Pheochromocytoma. He underwent right adrenalectomy/nephrectomy
and a large 12 cm adrenal tumor was visualized. Histopathology revealed a
poorly differentiated carcinoma with TTF-1, CK-7, and CEA and negative
for MART-1, Synaptophysin and CK-20. Markers and histopathology demonstrated likely primary adenocarcinoma of the Lung. A CT Chest showed
no significant lung lesion. A follow up PET/CT demonstrated two retroperitoneal masses and no other sites of activity. The patient agreed to broad spectrum chemotherapy and has completed 6 cycles of Carboplatin/Taxol. The
patient has tolerated chemotherapy well and post-treatment imaging demonstrated slightly diminished soft tissue mass adjacent to surgical site and no
hepatic metastatic disease.
Adrenal gland metastasis are not uncommon for adenocarcinoma of the
lung, but it is unusual to be the sole location of metastasis and even more unusual to lack any evidence of a primary location. The pathology from the adrenalectomy/nephrectomy was positive for TTF-1, indicating tissue arising
from lung or thyroid, and CK-7, and CEA strongly indicating likely pulmonary origin. Broad spectrum chemotherapy stabilized the lesions in posttreatment imaging. In cases with an extra-pulmonary mass and no radiographic evidence of a pulmonary lesion, pulmonary cancer can not be ruled
out and immunostaining is necessary to prove origin.
192
PNEUMOBILIA ATTRIBUTED TO BILIARY DUCT
NEOPLASM
Umyarova E1, Suvorava N2, Rakvit A2. 1MUSC, Charleston, SC and 2TTUHSC,
Lubbock, TX.
Case Report: Pneumobilia is an accumulation of air in the biliary tree. We
report a case of a biliary duct neoplasm presented with pneumobilia. 75 yo
female presented with fever and hypotension. She was evaluated for anemia
and unintentional weight loss and had unremarkable colonoscopy results.
She was admitted with diagnosis of sepsis underwent CT abdomen/pelvis
which showed dilatation of bile ducts multiple intraabdominal and retroperitoneal LN, and pneumobilia (p1). Subsequent ERCP/EUS showed biliary
stricture with dilatation of main hepatic ducts and mass on a bifurcation of
hepatic ducts suspicious for adenocarcinoma. CBD brushing revealed atypical cells suspicious for well differentiated adenocarcinoma. Pneumobilia is
an uncommon finding on imaging studies. Causes include recent biliary instrumentation, incompetent sphincter Oddi, biliary-enteric spihincter anastomosis, spontaneous biliary-enteric fistulas, PUD, neoplasms, emphysematous
cholecystitis and liver abscess. Radiographically: large linear confluence of air
located within a central portion of the liver. It is important to distinguish
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
pneumobilia from portal and mesenteric vein gas since the mortality rate
reaches 90% for the later if left surgically untreated. Our patient presented with
signs of sepsis and was found to have pneumobilia on imaging studies. Unlike
the other patients with hilar cholangiocarcinoma patient did not have signs of
obstructive jaundice, abdominal pain or pruritus and experienced only refractory anemia and significant weight loss.
Southern Regional Meeting Abstracts
supports the diagnosis but is not necessary. Diagnosis requires the detection
of a clonal population of plasma cells present in the periphery which can be confirmed with flow cytometry.
194
ONLY THE STRONG SURVIVE: A CASE OF PRIMARY
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
193
GO WITH THE FLOW: PLASMA CELL LEUKEMIA IN A
PATIENT WITH LEUKOCYTOSIS, HYPERCALCEMIA,
AND RENAL FAILURE
Wilkinson KJ1, Herrin V1, Lam JT2. 1UMMC, Jackson, MS and 2UMMC,
Jackson, MS.
Case Report: Plasma cell leukemia is a rare variant of multiple myeloma defined by the presence of clonal plasma cells in the peripheral blood exceeding
2000/microL or comprising at least 20% of the peripheral leukocytes.
Presenting characteristics are similar to those seen in myeloma and in other
leukemias, and abnormal protein electrophoresis is not required for the
diagnosis.
An 80-year-old female presented to the emergency room with complaints
of flu-like symptoms. Laboratory studies revealed a leukocyte count of 85.5
x 109/L, hemoglobin of 7.7g/dL, calcium of 13.9 mg/dL, and creatinine of
4.10 mg/dL. Review of peripheral smear showed many smudge cells and atypical lymphocytic cells with scant basophilic cytoplasm, round nuclei with
homogenously condensed chromatin, and no nucleoli comprising approximately 50% of the leukocytes. A few of these cells had a vague plasmacytic
appearance with eccentrically placed nuclei, but this was very subtle. Chronic
lymphocytic leukemia was considered in the differential but did not explain her
other findings, thus a bone marrow aspiration and biopsy were performed. The
marrow aspirate revealed sheets of atypical lymphoplasmacytic cells similar to
those seen in the periphery. Aggregates of these cells encompassed 80-90% of
the cellularity on the biopsy. Flow cytometry detected a cell population which
expressed CD138, CD38, and myeloperoxidase with cytoplasmic kappa Ig
light chain restriction. This immunophenotype was consistent with plasma cell
leukemia. Interestingly, serum and urine protein electrophoresis did not reveal
an elevated M-protein, however free kappa light chains were significantly elevated as well as the kappa/lambda light chain ratio.
She and her family strongly desired treatment, and she received one cycle
of renally-adjusted VD PACE and liposomal doxorubicin. She developed
neutropenia, and despite antifungal therapy, she died from candidemia sepsis.
Plasma cell leukemia can be difficult to diagnose given its rarity and subtle
morphology. As this case demonstrates, an abnormal protein electrophoresis
Williams JC, Herrin V. University of Mississippi Medical Center, Jackson, MS.
Case Report: Hemophagocytosis is a phenomenon where by erythrocytes,
leukocytes, platelets and the bone marrow precursors are engulfed by
macrophages. It is an unspecified phenomenon found in several conditions
such as hemolytic anemia, malignant disease, infections, and hemophagocytic
syndrome. Hemophagocytic Syndrome, also known as Hemophagocytic Lymphohistiocytosis (HLH), is an uncommon; life-threatening hyperinflammatory syndrome caused by severe hypercytokinemia due to a highly stimulated but
ineffective immune process.
We present the case of a 37 year old African-American man diagnosed
with secondary HLH. The patient initially presented as an inpatient consult
to Hematology to identify the etiology for fever in the presence of pancytopenia. Review of patient’s history was unremarkable except for three week
history of fever as high as 103 degrees Fahrenheit. Other than mild tachycardia, the patient’s physical exam was normal. With respect to his initial lab
work, the patient had pancytopenia, transminitis, negative hepatitis panel,
EBV negative, HIV negative, Parvovirus negative, normal rheumatoid factor,
and normal ANA. Two bone marrow aspirates and biopsies revealed rare
hemophagocytosis. The patient was discharged with follow up in Hematology clinic. One month after his initial presentation in the hospital, the patient
was re-admitted with unresolved fever, worsening transminitis, ferritin of
76,000; ileus and weakness, and soluble IL-2 of 5453. The patient was diagnosed with secondary HLH. He was subsequently administered HLH 1994
protocol with intent for bone marrow transplant. Despite not having any bone
marrow match, the patient tolerated the HLH 1994 protocol without difficulty and remains in remission.
HLH is an uncommon life-threatening hyperinflammatory syndrome
caused by severe hypercytokinemia due to a highly stimulated but ineffective
immune process. This case illustrates that swift diagnosis and treatment with
immunosuppression and cytotoxic agents can prolong disease free survival
and overall survival in a disease with poor prognosis.
Infectious Disease, HIV, and AIDS
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
195
RAOULTELLA ORNITHINOLYTICA: A RARE HUMAN
INFECTION
Akhter SR, Smalligan RD, Islam A, Chandra R. Texas Tech Univ Health
Sciences Center, Amarillo, TX.
Case Report: A 46yo woman was admitted for fever and right flank pain
despite 7d of amox/clav for UTI. No hx of hematuria or nephrolithiasis. PMH:
Hepatitic C with cirrhosis, recurrent UTIs. No illicit drug use and no HIV risk
factors. PE: A&O, BP 140/78, HR 100, RR 17, T 103. Heart and lung exams
normal. Abdomen soft without fluid wave but CVA tenderness present on the
right. Neurologic exam without flapping tremor. Lab: WBC 2.8k, 80% segs,
Hgb 10.8, platelets 74k, bili 2.0, Cr 1.0, HIV neg. UA had WBCs and nitrite
pos and cx: Raoultella ornithinolytica resistant to amp but sensitive to quinolones and carbapenems. Abd CT: right pyelonephritis without stones or obstruction. She responded to levofloxacin and was discharged home within 3 days.
Discussion: Internists encounter complicated UTI and pyelonephritis on a
daily basis. The vast majority are due to E. coli with the less common appearance of Proteus, Klebsiella and Enterobacteriaceae species. This case was
caused by an uncommon Enterobacter. family organism: Raoultella ornithinolytica. In the past Raoultella was thought to be a Klebsiella organism but molecular techniques have shown it to be a separate genus consisting of R.
ornithinolyticia, R. planticola, and R. terrigena. This is a gram-negative encapsulated aerobic bacillus that is found in aquatic environments and soil. It is
© 2014 The American Federation for Medical Research
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Southern Regional Meeting Abstracts
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found in the gut of fish, and that of soil insects like earthworms and millipedes,
hence contaminating the water and soil. Risk factors include eating contaminated fish, fecal-oral spread and contact with soil. Immunocompromised
patients are particularly susceptible, including cirrhotics like our patient. There
are reports of Raoultella genus causing skin and soft tissue infections, bacteremia, and UTIs. A distinctive feature of this bacterium is its ability to convert
histidine to histamine in scombroid fish causing redness and flushing on the
skin (histamine fish poisoning).
Raoultella spp can be quite resistant with some being resistant. Macrolides
are ineffective, as are rifampicin, glycopeptides and fusidic acid. Carbapenemases
have been found in Raoultella spp as well hence it is best to guide treatment by
current sensitivities. Effective choices often include cephalosporins, cotrimoxazole, fluoroquinolones, aminoglycosides, or nitrofurantoin.
196
ANOTHER DOMESTIC CHAGAS DISEASE IN THE US
AlBaalbaki F, Land M. University of Tennessee, Germantown, TN.
Case Report: 36 year Caucasian female with no significant past medical history, referred to our Infectious disease clinic for positive serology of
Trypanosoma cruzi. The patient works in Memphis, TN, and lived most of
her life in rural areas of northern Mississippi. She denied any cardiac or GI
symptoms. She had no history of blood transfusion, needle stick, or organ
transplantation. She had never traveled to an endemic area, had an insect bite,
or involved in outdoor hunting. Her mother had never lived in an endemic
area and had negative serology. Her physical exam was basically unremarkable. Electrocardiogram showed no evidence of conduction abnormality. Her
basic laboratory came back normal. Repeat T. Cruzi antibodies came back
positive for IgG with titer of 12 (positive 5-16). Another blood sample was
sent to the CDC and was IFA positive with titers of 1:32 (reactivity at 1-32
or greater sample dilution is defined as a reactive test). The patient was diagnosed with chronic indeterminate chagas disease and offered treatment with
Benznidazole according to CDC recommendation. She tolerated the medicine well with no significant side effect.
Chagas disease is most commonly transmitted through defecation by T
cruzi-infected triatomine insects after a blood meal. Chagas infection first
presents as an acute illness that can either be asymptomatic or a self-limiting
febrile illness. After a decrease in parasitemia, patients enter the indeterminate chronic stage. About 70% of patients in the indeterminate stage will
never experience symptoms. The remaining 30% of patients will develop
chagasic cardiomyopathy or digestive symptoms, including megaesophagus
or megacolon, often 10 to 30 years after the initial infection.
Chagas disease has long been known to be an important parasitic infection in Latin America, with 11 million people or more currently infected.
However, there is an increasing attention to the presence of the disease in
the US, with more than 300,000 chronically infected persons residing in
the country. It is well known that triatomine vector can be found across the
southern half of the country. It is assumed that most of the Chagas disease
cases in the US result from immigration; however, new evidence documents
at least 23 cases of autochthonous, vector-borne transmission within the US.
197
INTRACRANIAL TUBERCULOMA IN A PATIENT
PRESENTING WITH CONFUSION AND HEMIPARESIS
Bojanowski CM, Beck S, Krauland J, Gruber M, Engel LS, Amoss J. LSU
Health Sciences Center, New Orleans, LA.
Case Report: Central nervous system (CNS) tuberculosis is a rare severe extra-pulmonary form of Mycobacterium tuberculosis disease that manifests as
either tuberculoma or meningitis. The majority of CNS cases occur in children and immunocompromised patients. Common presenting symptoms include headache, seizures, and hemiparesis. Radiological diagnosis of a
brain tuberculoma is difficult as the imaging presentation is varied and can
be non-specific.
CASE: A 55 year old African American woman with end stage renal disease
on hemodialysis, anemia of chronic disease and severe de-conditioning presented with altered mental status and left sided hemiparesis. Initial brain CT
and sequential MRI brain with and without contrast showed a ring enhancing
lesion high in the right parietal lobe with restricted diffusion, calcifications,
466
and associated vasogenic edema. Also noted on admission imaging was the
presence of right supraclavicular and cervical chain lymphadenopathy. Excisional biopsy of a supraclavicular lymph node revealed granulomatous type inflammation and mycobacterium tuberculosis grew from culture at 2 weeks.
Serum Interferon gamma release assay (IGRA) was also positive. She was
started on dexamethasone and phenytoin for seizure prophylaxis. Empiric antimicrobial coverage for both brain abscess and tuberculoma was provided with
vancomycin, ceftazidime, and RIPE therapy.
DISCUSSION: Tuberculomas typically appear on imaging as either solitary
or multiple, round lesions often located in the frontal or parietal lobes with
irregular walls and with signs of ring enhancement after contrast. The “target
sign” (a ring enhancing lesion with a central area of enhancement or calcification) has been described as characteristic of tuberculomas. MRI findings
vary according to the stage of the lesion. Despite advancements in imaging
and laboratory diagnostics, tuberculomas of the central nervous system remain a diagnostic challenge. Early diagnosis is imperative, since clinical outcomes are largely dependent on timely treatment. Standard medical care
includes prolonged treatment (12–30 months) with rifampicin, isoniazid,
pyrazinamide, and streptomycin or ethambutol.
197A
DISSEMINATED HISTOPLASMOSIS WITH ORAL
AND CUTANEOUS MANIFESTATIONS IN AN
IMMUNOCOMPETENT PATIENT
Bojanowski CM, Johnson P, Salassi M, Mohan M, Engel LS. LSU Health
Sciences Center, New Orleans, LA.
Case Report: Occurrence of the disseminated histoplasmosis is rare in HIV
seronegative patients. Here we describe a case of disseminated histoplasmosis
with both oral and cutaneous manifestations in an immunocompetent patient.
CASE: A 40 year old construction and oil field worker from Louisiana
presented with a 4 month history of weight loss, night sweats, fever, nonproductive cough, shortness of breath and pleuritic chest pain after failing treatment for presumed bronchitis and pneumonia. The patient also reported
inguinal erythematous papules and ulcers and a recent onset of oral ulcerations. At the time of admission, he was not febrile. On physical exam, he
was described as a well-built thin Caucasian male in no acute distress who
had a right superior gingival 2mm ulceration. His skin had multiple (> 10)
small erythematous nodular papules located bilaterally in his inguinal folds
and a large erythematous and plaque-like lesion with rolled borders and central erosion in his left inguinal fold. Initial labs revealed microcytic anemia.
HIV, RPR, HSV, T-Spot, Blastomycosis antibody, Histoplasma urine antigen,
and blood culture were negative. CXR on admission showed a right upper
lobe opacified nodule with central lucency, a right lower lobe nodule. CT
guided fine needle aspiration of lower lobe lung mass demonstrated suppurative non-caseating granulomatous inflammation with organisms consistent
with Histoplasma identified within the macrophages. GMS staining also
revealed abundant fungal elements consistent with Histoplasma. Fungal culture grew Histoplasma capsulatum. Inguinal punch biopsy also showed abundant dermal histiocytes heavily loaded with fungal elements. The patient was
started on a 12 month course of itraconazole.
DISCUSSION: Manifestations of disseminated histoplasmosis include fever,
weakness, weight loss, hepatosplenomegaly, and mucocutaneous lesions. Systemic histoplasmosis has emerged as an important opportunistic infection
among immunocompromised patients as well as in immunocompetent patients
residing in endemic areas. Biopsy of lung, mucosal or cutaneous lesions is the
most rapid method of arriving to the correct diagnosis.
198
HISTOPLASMOSIS AND MYCOBACTERIUM KANSASII
PRESENTING AS A RECURRENT PLEURAL EFFUSION
Bojanowski CM, Krauland J, Engel LS, Amoss J. LSU Health Sciences Center,
New Orleans, LA.
Case Report: Mycobacterium kansasii is the second most common nontuberculous mycobacterium (NTM) species isolated from patients with HIV infection and is considered to be one of the most virulent. Confounding the
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
presentation of this disease is the high rate of coinfection with other pulmonary pathogens.
CASE: A 67 year old African American man with HIV/AIDS (CD 4 count 11)
presented with a six month history of unintentional weight loss and a one month
history of dyspnea on exertion and cough productive of yellow-brown sputum.
At the time of presentation, he denied any chest pain, fevers, or night sweats.
Social history was significant for a remote incarceration and a 50 pack year
smoking tobacco history. His last negative PPD was 2 years prior around the
same time he discontinued taking his HAART therapy. Pertinent exam findings
included a respiratory rate of 18 with 97% oxygen saturation on room air,
cachexia with temporal wasting, seborrheic keratosis, and a lung exam significant for increased fremitus, dullness to percussion to the level of scapula, and
decreased breath sounds on the right. Imaging (CXR and CT chest with contrast) showed a large right sided pleural effusion with a right to left cardiomediastinal shift. He was placed on respiratory isolation and AFB sputum samples
were obtained. The patient was started on RIPE therapy after AFB smears were
positive. Pleural fluid culture eventually grew Histoplasma capsulatum. He was
started on Ambisome IV and continued on RIPE therapy. Cerebrospinal fluid
was negative for histoplasmosis, AFB, and Cryptococcus. Gen-probe of both sputum and pleural effusion were positive for Mycobacterium kansasii as well. Treatment was tailored to include rifampin, INH, ethambutol and intraconazole.
DISCUSSION: The literature suggests that up to 69% of HIV positive
patients with pulmonary consolidations and M. kansasii, also had a another
pulmonary pathogen concurrently isolated from respiratory specimens. Due
to the highly variable clinical and radiographic presentation and frequent
occurrence of coexisting pathogens, all patients with HIV/AIDS and respiratory symptoms should be evaluated for mycobacterial disease and opportunistic infections such as Histoplasmosis.
199
NEONATAL CONGENITAL HIP DISLOCATION
COMPLICATED BY GROUP B STREPTOCOCCOS SEPTIC
ARTHRITIS AND OSTEOMYELITIS
Brosset Ugas M1, Balan A2, DeLeon S1. 1University of Oklahoma Health
Sciences Center, Oklahoma City, OK and 2University of Oklahoma Health
Sciences Center, Oklahoma City, OK.
Case Report: Septic arthritis and osteomyelitis are unusual in the newborn
period, which may cause a delay in diagnosis. Their presentation is variable
and can be associated with dislocation or physeal separation. We present
the case of a female neonate with a congenital hip dislocation found to have
septic arthritis complicated by osteomyelitis.
Our patient was an 18 day old infant born at term via spontaneous vaginal delivery after an uncomplicated pregnancy; her mother tested negative
for HIV and Group B Streptococcus (GBS), and she was discharged home
at 48 hours of life. Since discharge, her family reported decreased spontaneous movement and pain with passive movements of her right leg. She was
otherwise well, had no fevers, and demonstrated normal activities. On day
of life 17, she was seen at an Emergency Department due to persistent parental
concerns about her leg. On physical exam her right hip was non-erythematous
but did have tenderness with passive motion. Pelvis X-ray showed a right hip
dislocation, metaphyseal lucency, and signs of a septic right hip joint. Hip
ultrasound showed a large heterogeneous multiloculated joint effusion with
displacement of the femoral head laterally. Her initial CBC showed a WBC
count of 26,300 and a normal differential, normal hematocrit, and elevated
platelets of 725,000; ESR and CRP were also markedly elevated. Orthopedics performed an open drainage and washout and obtained grossly purulent synovial fluid with negative gram stain. She was placed in a Pavlik
harness and started on vancomycin and cefotaxime. Blood and spinal fluid
cultures were sent and ultimately were negative. The joint culture, however,
grew GBS. Our patient was switched to ampicillin, and ultimately penicillin, once sensitivities were known.
A literature search reveals few cases of neonatal osteomyelitis. This case
shows that osteomyelitis/septic arthritis in a newborn may present with only
decreased motion of an extremity, and physicians should maintain a high index of suspicion. In our case maternal and delivery risk factors for infection and hip dislocation were not present, which caused an even greater
delay in diagnosis.
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200
INFANT BOTULISM IN OKLAHOMA: A CASE REPORT
Daniels BS1, Nabavizadeh N1, Balan A1, Johnson M1, Ng Y2. 1University of
Oklahoma Health Sciences Center, Oklahoma City, OK and 2University of
Oklahoma Health Sciences Center, Oklahoma City, OK.
Case Report: Infant botulism is a rare disease caused by Clostridium botulinum toxin and characterized by descending symmetrical paralysis, cranial
nerve and bulbar dysfunction, and hyporeflexia.
In Oklahoma there were only 8 cases reported in the past 23 years. We
present a 1-month old patient with an unusual presentation of infant botulism.
A 1-month old infant presented with acute onset of poor feeding, fussiness and constipation. Parents reported weakness and a faint cry, but denied
fever, apnea, seizures, or rash. On physical exam, she was hypotonic with
head lag and poor suck reflex, but with normal deep tendon reflexes. She underwent a full sepsis work up which was negative. On hospital day 3, she had
increasing weakness and respiratory distress. A blood gas revealed severe
respiratory acidosis requiring intubation. Just prior to intubation she appeared
weak with a “ragdoll” appearance, and with poor gag but intact cough reflex.
Genetic and thyroid studies, EEG, brain and spine MRI were normal. With
no apparent cause, and in view of rapid respiratory failure and progressive paralysis, infant botulism became high on our differential. After discussion with
specialists at the California Department of Health, human botulism immune
globulin, BabyBig©, was given on hospital day 5, and a stool sample was sent
to the CDC for botulinum toxin bioassay.
Within two days of BabyBig© administration her strength improved. On
hospital day 10, she was reported to have positive botulinum toxin in stool,
extubated on hospital day 12, and discharged on day 19.
Infant botulism is rare in Oklahoma; the source of the spores remains
unknown but it likely was from the environment, as her house was in a construction zone near recent tornado damage. Our report describes an atypical presentation given the young age (median 2-8 months) and intact distal deep tendon
reflexes despite respiratory failure. Her strength seemed to improve after intubation, which confounded the diagnosis. Our case illustrates that in an infant with
acute weakness and rapid progressive respiratory failure, botulism must be considered, as prompt diagnosis and treatment ensures faster full recovery and
lower hospitalization costs.
201
THE CAT SOLVED IT: A CASE OF PULMONARY
HISTOPLASMOSIS IN A 58 YEAR- OLD FLORIST
Echendu CE1, Khasawneh FA1, Trybus J2. 1Texas Tech Univ Health Sciences
Center, Amarillo, TX and 2Texas Veterinary Medical Diagnostic Laboratory,
Amarillo, TX.
Case Report: Histoplasmosis is a fungal infection caused by Histoplasma
capsulatum. It is reported throughout the United States but is most endemic
along the Mississippi and Ohio River valleys. The primary source of infection is contaminated soil and most cases are acquired via inhalation of
microconidia, hence pulmonary infection is most common. Histoplasmosis
diagnosis relies on identifying the fungus in infected tissues by culture, histopathological examination, serology/antigen detection or new molecular diagnostic methods in combination with consistent history and imaging studies.
Pulmonary histoplasmosis can be successfully treated with amphotericin B or
itraconazole. A 58 year-old female florist was admitted with a small subdural
hematoma after falling down due to a vasovagal syncope. She was found to
be hypoxic. A computed tomography of the chest was done to rule out pulmonary embolism. It showed bilateral pulmonary nodules with hilar lymphadenopathy. On further questioning, the patient reported weight lose and night sweats
and she recalled losing her cat 2 months ago to a fungal infection. The veterinarian was contacted for the histopathology report, which showed feline histoplasmosis. The patient refused invasive testing. Histoplasma serology was positive.
She was discharged home on oral itraconazole to be followed up in clinic. Animal to human transmission of histoplasmosis has not been reported. In our
case, both cat and owner acquired their infection from same or similar source.
The patient lived just to the west of histoplasma endemic areas. Furthermore,
exposure to conidia by being a florist who gets flowers and plants from all over
the county could have been a second potential source of infection. Obtaining
detailed history aided in the management of our patient.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
467
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
202
NON-FATAL GASTRIC MUCORMYCOSIS
Ferrara AP Lewis T. Baptist Health Systems of Alabama, Birmingham, AL.
Case Report: Gastric mucormycosis is a rare form of fungal infection that
most commonly affects immunocompromised patients with high rates of
mortality. Predisposing conditions include metabolic acidosis and defects
in neutrophil or monocytic function. Patients generally present with fever,
abdominal pain, distention, nausea, vomiting, and hematochezia. The diagnosis is established with histopathologic specimens often acquired through
upper endoscopy. First line therapy of treatment is amphotericin B combined
with surgical debridement. Posaconazole additionally can be used in conjunction with these therapies or in the case of failure or inability to tolerate
amphotericin B. Our patient was a 56 year old African American woman
with a history of controlled diabetes mellitus type 2 and end-stage renal disease on peritoneal dialysis. She was found to have gastric mucormycosis
after hematemesis prompted upper endoscopy. She was successfully treated with
liposomal amphotericin B with total gastrectomy and a right hemicolectomy.
related to other causes of immune compromise such as cirrhosis. We present
a case of rapidly fatal disseminated Cryptococcus neoformans in a non-HIV
cirrhotic patient.
We present a 58-year-old male with alcoholic cirrhosis presented with
new onset gait disturbance followed by altered mental status, bladder and
bowel incontinence, and hallucinations favoring a diagnosis of hepatic encephalopathy. The patient’s family reported alcohol and tobacco use, and previous treatment of cancer without residual disease. Physical exam findings
included jaundice, abdominal distention, upper extremity fasciculations,
and disorientation. Studies included blood cultures, ammonia level, and hepatic function panels. HIV screen was negative, CT of the head showed no
lesions or enhancement, hepatic function panels revealed elevated ammonia
of 99 umol/L. On day 2, blood cultures were positive for fungus and empiric
treatment with Abelcet and flucytosine were started. Suspicion for cryptococcal meningitis was high despite decreasing ammonia levels and persistent
confusion. Tachycardia and anuria following initial dose of abelcet suggested acute renal failure with creatinine of 1.47 ng/dL from admission level
of 0.57 ng/dL and was subsequently discontinued. On day 4, further patient
decline lead to mechanical ventilation and vasopressors. Family decided to
withdraw support, and the patient expired 72 hours after diagnosis of disseminated cryptococcus without evidence of HIV.
Currently, there are limited reports of cryptococcal infections in non-HIV
patients; however, with immune dysfunction secondary to chronic diseases
like cirrhosis and diabetes, such infections are possible. With a low index
of suspicion, invasive fungal infections in non-HIV infected patient may delay testing and therapy. Early recognition and treatment of cryptococcal
infections can decrease mortality.
204
Ischemic ulcer at lesser curvature.
Hyphae fungal forms.
203
HEPATIC ENCEPHALOPATHY: A CASE OF
KILLER FUNGUS
Googe A, Grover I. University of Mississippi, Jackson, MS.
Case Report: Cryptococcal infections are unanimously associated with HIV
and are an AIDS defining illness. However, approximately 10% of cases are
468
WEST NILE VIRUS ENCEPHALITIS MASKED BY
ALCOHOL WITHDRAWAL
Graebert A, Jones N, Engel LS, Maffei J. LSU Health Sciences Center, New
Orleans, LA.
Case Report: West Nile virus is an arbovirus transmitted by mosquitoes most
commonly from a host bird. Often asymptomatic West Nile virus may present
as self-limited febrile illness or as a neuroinvasive disease manifested as meningitis, encephalitis, or flaccid paralysis.
CASE: A 57 year old Caucasian male with a medical history significant for
hypertension and alcohol use presented to the emergency department after a
witnessed episode of syncope. The patient admitted to a 2-3 week history of
generalized weakness. Upon presentation vital signs and physical exam were
unremarkable. Comprehensive metabolic panel revealed sodium of 127 mmol/L,
potassium of 2.8 mmol/L, creatinine of 0.97 mg/dL, and mildly elevated total
bilirubin. Overnight, the patient became febrile up to temperatures of 104 °F
and developed a tremor. Blood and urine cultures were drawn, and he was
started on empiric antibiotic therapy. He was also placed on a multivitamin,
thiamine, and folate, along with ativan as needed for suspected alcohol withdrawal. Head CT and MRI showed only chronic cerebral atrophy and
chronic ischemic changes. He continued to have febrile episodes. However,
multiple sets of blood and urine cultures did not reveal evidence of infection
and chest x-ray did not reveal an infiltrate. Lumbar puncture produced clear
and colorless cerebrospinal fluid with 41% white blood cells, glucose of
61 mg/dL, and protein of 48.1 mg/dL. Routine CSF cultures, acid fast bacillus, CSF VDRL, HSV PCR, nasal swabs for influenza A and B antigens,
and serum cryptococcal antigen were negative. His West Nile virus IgG and
IgM were positive and a diagnosis of West Nile virus was confirmed. Over
the course of the next week the patient’s mental status improved and strength
was regained through physical therapy.
DISCUSSION: The patient’s initial presentation was his manifestation of
West Nile fever characterized by fevers, chills, and generalized weakness.
The progression of disease to encephalopathy was likely secondary to his history of alcohol abuse, as older age, alcohol abuse, and diabetes in patients with
West Nile virus have been associated with encephalitis. There is no specific
treatment for West Nile virus, and patients are managed with supportive care.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
205
FAR MORE THAN A CYCLING INJURY
Gravolet R, Doan C, Engel LS, Guillory S. LSU Health Sciences Center,
New Orleans, LA.
Case Report: Approximately 300 episodes of miliary TB were reported in
the US in 2010, accounting for only 1-2% of all TB cases.
CASE: A 73 year old man presented with a 4 day history of hematuria and
a 4 week history of dysuria and lower back pain. The patient attributed his
problems to a recent biking injury in which his groin collided with the
bike’s handlebars. He was trearted with ciprofloxacin for 10 days for a suspected UTI. His symptoms continued to worsen over the next two weeks,
however, and his lower back pain intensified to a 9/10. On presentation to
our facility, further questioning revealed a 10 year history of cough that in
previous weeks was more productive of darker colored sputum. He reported
a 40 lb weight loss in the previous months. He affirmed a 20 pack year
smoking history, a history of incarceration five years prior, and current
homelessness. Vital signs were unremarkable. On physical exam, the patient
had decreased breath sounds in his lower lobes, tenderness to palpation
along his lower back, and a hyperpigmented hardened nodules on the skin
of his scrotum and behind his right shoulder. Urinalysis was positive for
blood (250/microliter) and protein (150/microliter), and PSA was 28.5. Chest
CT showed cavitary changes, miliary changes, and calcified nodules distributed throughout the lungs bilaterally. Abdominal/pelvic CT revealed L5-S1
discitis & osteomyelitis, an enlarged heterogenous prostate, and an 8 mm
UVJ stone; US of the testicle revealed a hydrocele and possible scarred fibrous tissue. The pt’s sputum tested AFB 3+ positive, and an IR biopsy of
the lumbar spine was AFB smear positive, both subsequently grew Mycobacterium tuberculosis. Urinary involvement was also confirmed with a
urine culture grew TB. The patient was diagnosed with disseminated TB,
and RIPE + B6 therapy was administered with plans to treat for 9 months.
DISCUSSION: Due to nonspecific signs and symptoms, miliary TB is often
missed (20% of cases diagnosed post mortem) and requires a high index of
suspicion. Our patient’s diverse problems including a long history of productive cough, weight loss, back pain, dysuria and hematuria are united under
one pathological process of disseminated TB.
206
A RARE ASSOCIATION:INFLUENZA TYPE B WITH
SEVERE THROMBOCYTOPENIA
Habib S, Moti D, Chandra R. Texas Tech Univ Health Science Center,
Amarillo, TX.
Case Report: A 32 year old male had acute presentation with 3 days of generalized bodyaches, headache and malaise, sore throat and fever. He also had
been coughing out blood for a day with shortness of breath. He had no past
medical history. He came to US from Cuba 1 month prior, but had been
healthy all along until this current presentation. He did not consume alcohol
or any medications. On examination BP was 120/70,P 80 R 22, temperature
100F. Heart, lung exam was clear. Skin had mild petechiae. Abdomen was
without any splenomegaly. No lymph node enlargement. Nonfocal neurologic exam. Labs showed WBC count of 3,000,normal Hb, platelet count
of 17,000. Renal and liver function was normal.CT scan of the chest showed
bilateral “tree in bud” infiltrates more consistent with diffuse bronchopneumonia rather than alveolar hemorrhage. Investigations for thrombocytopenia
noted for peripheral blood smear with a very low platelet count. No platelet
clumping. No schistocytes, no signs of hemolysis or microangiopathy. Dengue IgM was normal and malarial parasite was normal. Vitamin B12 and
Folic acid levels were normal. Rapid strep test,monospot test, HIV serology,
hepatitis panel, ANA, ADAMTS13 test, were all negative. Regarding his
pneumonia, his blood cultures were negative. Sputum cultures and AFB stain
were negative.Influenza antigen was positive for Influenza B. Patient was
treated for Influenza B pneumonia with oseltamivir. He clinically improved
and platelet count spontaneously recovered to 101,000/cmm after 6 days.
While moderate thrombocytopenia has been described with many viral
illness, a severe thrombocytopenia of this level is seen very rarely with Influenza type B. Less than 1.8 % of type B influenza will have thrombocytopenia
per one study.In general,mechanisms of thrombocytopenia in influenza are
mostly to do with transient viral suppression of platelets production in the bone
marrow. These patients would spontaneously recover without any specific
Southern Regional Meeting Abstracts
therapy as in our patient.Thrombocytopenia in TypeA influenza has been associated with higher mortality.RT PCR testing is not always readily available, low
platelet counts can be a clue to certain aggressive strains.Newer emerging potentially fatal Type A( H7N9) variants in 2013 have been associated with thrombocytopenia and alveolar hemorrhage.
207
TULAREMIA : A CAUSE OF LYMPHADENITIS IN A
PEDIATRIC PATIENT FROM OKLAHOMA
Hines L1, Lich A1, Balan A2, Ramji F3. 1University of Oklahoma Health Sciences
Center, Oklahoma City, OK; 2University of Oklahoma Health Sciences Center,
Oklahoma City, OK and 3University of Oklahoma Health Sciences Center,
Oklahoma City, OK.
Case Report: Tularemia is an uncommon cause of lymphadenitis in
Oklahoma. It is a disease caused by the bacteria Francisella tularensis, most
commonly transmitted through the bite of an infected tick. Approximately
120 cases of tularemia are reported each year in the United States. In 2010,
states with the highest number of cases were Arkansas, Missouri, Kansas
and South Dakota, with a range of 11-19 cases per year; Oklahoma was
the 4th state, with only 8 reported cases, all patients were 17 years and older.
A 9-year-old female presented with fever, tenderness, erythema and
lymphadenopathy of left inguinal area. Her symptoms began 5 days after a
tick bite to the area. She was started on trimethoprim/sulfamethoxazole,
but symptoms worsened and she was admitted for further management.
She received intravenous clindamycin. Doxycycline was added due to history of a tick bite and presence of thrombocytopenia. She developed a small
ulceration at the site. Her fever improved and she completed a 10-day course
of oral clindamycin and doxycycline. The ulceration healed and lymphadenitis improved. One week after discontinuation of antibiotics, she experienced
recurrence of fever and worsening of lymphadenitis, at which point tularemia
was considered and serology was obtained; oral doxycycline and clindamycin
were given. A week into restarting her antibiotics treatment tularemia titer was
reported at 1:1280 (positive >1:160). She developed a large, tender, erythematous and fluctuant mass in her inguinal region; incision and drainage, and therapy with gentamicin was initiated. She completed a 2-week course of gentamicin
without relapse of her symptoms.
We present a pediatric case of ulceroglandular tularemia. While this is an
uncommon cause of lymphadenitis, it has to be considered in states with relatively high prevalence, like Oklahoma, especially in patients reporting a tick
bite. Our case illustrates relapse with therapy with doxycycline. The recommended treatment of choice remains an aminoglycoside like gentamicin; doxycycline is associated with a high relapse rate especially with a short duration of
therapy.
208
WHO’S TO BLAME?
Jethwa SD, Thomas WH, Bocchini J. LSU Health Sciences Center,
Shreveport, LA.
Case Report: 17 year old female with extensive past medical history,
presented with facial and lip edema. Previously, she developed a left nostril
abscess, was drained, and treated with 10 days of doxycycline. Upon completion of her antibiotics, she noted facial swelling and pain. She received
Rocephin, Keflex and Bactrim at another facility. She presented to clinic with
increased pain and swelling. She was admitted with facial cellulitis. IV
Clindamycin was started. Lab values were within normal limits; sed rate elevated at 36. Sinus CT showed only soft tissue swelling. Blood and facial exudate cultures were negative. Her facial swelling and drainage worsened. She
was changed to Vancomycin with continued worsening of symptoms. High
dose IV steroids were initiated. By 24 hours, she showed vast improvement
of facial swelling and pain. During her initial course of steroids no changes
were made to her home medication including: lisinopril, risperdal,
metformin, insulin. Within 48 hours of discontinuing steroids, her symptoms
reoccurred. All medications with potential to cause angioedema were
stopped. Despite changing her medications, lisinopril to diltiazem and
risperdal to fluoxetine, she continued to swell. IV steroids were restarted
and continued with a 2 week taper. She was discharged with no facial swelling. The differential diagnosis of this her pain and swelling are the following:
drug induced hypersensitivity reaction, infection, or C1 esterase deficiency.
© 2014 The American Federation for Medical Research
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Her C4 complement level was slightly elevated at 44.6 mg/dl (range 10-40
mg/dl); therefore, C1 esterase deficiency has not been completely ruled
out. Classic patients with C1 esterase deficiency do not respond to steroids
as our patient did.
209
CHYLOUS ASCITES IN KAPOSI SARCOMA:
A CASE REPORT
Johnson P, Chang E, Smith E, Lo B. LSU Health Sciences Center, New
Orleans, LA.
Case Report: A 26 year old Caucasian male with a history of AIDS (CD4 123), KS, pleural effusion, and pulmonary emboli was admitted for diffuse
abdominal pain and scrotal edema. Physical exam was notable for tachycardia, pallor, decreased basilar breath sounds, diffuse abdominal pain with distention, positive fluid wave and shifting dullness without rebound or
guarding, tender scrotal edema, inguinal adenopathy, and purple lesions on
his upper palate and torso consistent with KS. A prior EGD and colonoscopy
showed Kaposi’s lesions throughout the GI tract. An abdominal CT revealed
diffuse adenopathy. A paracentesis was performed and 1510mL of milky, turbid, blood-tinged fluid was removed. Fluid analysis was negative for malignancy but demonstrated 36,300; RBC’s and triglycerides concentration of
740 mg/dL. The patient was started on liposomal doxorubicin for his KS during his hospitalization and finally achieved adequate pain control and improvement of his ascites and scrotal edema after three rounds of treatment
as an outpatient.
DISCUSSION: Chylous ascites is an uncommon finding that is caused by
the presence of intestinal or thoracic lymph in the peritoneal cavity. The incidence is reportedly 1 per 20,464 admissions at Massachusetts General Hospital during a 20-year period. It is diagnosed by milky ascitic fluid with a
triglyceride content typically greater than 200 mg/dL. Causes of chylous ascites include: malignancy, trauma, chronic liver disease, inflammation, infection, postoperative. The underlying mechanism for the formation of chylous
ascites is due to the disruption of the lymphatic system from obstruction or
traumatic injury. While the exact cell of origin for KS remains unclear, the
current opinion is that KS tumor cells are derived from lymphatic endothelium. Hence, the development of chylous ascites may possibly be due to
in-situ KS in that region rather than metastasis to the thoracic duct as once
thought. Chemotherapy for KS can often improve symptoms (response rate
60-90%) as was seen in our patient.
210
A POPULATION-BASED INVESTIGATION OF OUTCOMES
OF CANDIDEMIA
Kabbani S1,2, Stein B1,2, Hollick R4, Harrison LH4, Farley M1,2,3. 1Emory
University School of Medicine, Atlanta, GA; 2GA Emerging Infections Program (EIP), Atlanta, GA; 3VA Medical Center, Atlanta, GA and 4Maryland
Active Bacterial Core surveillance (EIP), Baltimore, MD.
470
Purpose of Study: Describe risk factors associated with mortality from
candidemia.
Methods Used: Demographic and clinical data were collected prospectively
between 3/1/08 and 2/28/13 for candidemia cases identified through active,
population-based, laboratory surveillance in Georgia and Maryland EIP. Univariate analysis of risk factors associated with mortality at 30 days in children and adults was performed; p<0.05 was significant.
Summary of Results: Overall, 3,782 candidemia cases were identified with
a mean annual incidence of 14.5/100,000 population. While only 64.9% of
cases were hospital-onset (HO), 93.1% had at least one of the following risk
factors: use of central venous catheter (CVC) (84.6%), total parenteral nutrition (TPN) (34%), or antibiotics within 14 day (78.8%). Death occurred in
917 (26.4%) adults and 32 (13.8%) children. Fluconazole resistance was
found in 7.2% of cases and was not associated with mortality. Among adults,
mortality was significantly higher with age ≥45 (OR 2.28 CI 1.82-2.84), HO
(OR 1.86, CI 1.57-2.21), antibiotics(OR 1.6, CI 1.31-1.97), CVC (OR 1.46,
CI 1.17-1.83), ICU stay (OR 3.85, CI 3.18-4.65), renal disease (OR 1.72, CI
1.43-2.06), liver disease (OR 1.74, CI 1.42-2.13), malignancy (OR 1.43, CI
1.18-1.72), prior antifungal use (OR 1.37, CI 1.11-1.69), and infection with
Candida krusei (OR 2.21, CI 1.3-3.76) or C. tropicalis (OR 1.38, CI 1.08-1.75).
Black race (OR 0.8, CI 0.69-0.94), TPN administration (OR 0.84, CI 0.72-0.99),
CVC removal within 7days (OR 0.25, CI 0.21-0.3), antifungal treatment (OR
0.2 CI 0.16-0.25) and C. parapsilosis (OR 0.54, CI 0.43-0.69) were associated
with lower odds of death. In children, death was significantly associated with
male gender, CVC, antibiotics, ICU stay, and malignancy; antifungal treatment
and CVC removal were associated with lower odds of death.
Conclusions: Candidemia was strongly associated with use of CVCs, TPN,
and systemic antibiotics. Mortality from candidemia was higher in older adults,
those with underlying diseases or on systemic antibiotics, and infection with C.
krusei or C. tropicalis. Multivariable analysis will be conducted to determine independent risk factors for mortality associated with candidemia.
211
SIADH SYNDROME SECONDARY TO WEST NILE
MENINGOENCEPHALITIS
Karim A, Nakhla E, Akhter SR, Ahmed M, Islam A. Texas Tech Univ Health
Sciences Center, Amarillo, TX.
Case Report: Learning objective: Recognize the association of West Nile
Meningoencephalitis with SIADH syndrome.
Case Presentation: A 58 year old Caucasian male presented with encephalopathy worsening over 1 week. He had H/O Afib, HTN for which he was on
warfarin, valsartan and HCTZ. On admission he was drowsy but arousable,
oriented only to person, vitals were normal. Physical examination was significant for bilateral lower extremity weakness against resistance. Labs were
significant for sodium 119, potassium 2.5, cortisol, TSH, T4 levels were normal. Urine sodium 12, potassium 20, calculated serum osmolality 250, effective osmolality 248, normal lipids and total protein, urine osmolality 447.
Drug screening, alcohol level, RPR negative. With the initial 0.9% NS IV
100cc/hr kept Serum sodium around 125. For ongoing confusion 3% sodium
chloride was given following which sodium level came around 132. Corresponding urine sodium was 167. SIADH syndrome was highly suspected. He
had one time fever at home a week ago. Emperic antibiotic started. CT head
showed old right frontal stroke. CSF study was significant for aseptic meningitis. West Nile serology was positive.A diagnosis of SIADH syndrome secondary to West Nile Meningoencephalitis was made.
Discussion: WNV is an RNA arbovirus. The transmission of the disease in
humans occurs most commonly from a bite of an infected Culex mosquito.
Person-to-person transmission is not common but can result from blood
transfusion, organ transplantation, intrauterine or breastfeeding exposure.
The incubation period of WNV is 2-15 days. Approximately 20% will develop a self-limited flu-like illness called West Nile fever with no neurological manifestation. Less than 1% of infected persons develop neurological
disease. For patients with neuro invasive disease, 35-40% has meningitis,
55-60% has encephalitis, and only 5-10% have poliomyelitis. About 40%
of patients have an initial CSF that shows a pleocytosis with a neutrophil predominance that then becomes predominately lymphocytes. Hyponatremia is
seen in 1/3rd of patients. Our patient had hyponatremia with low effective osmolality, high urine osmolality, high urine sodium with clinical euvolemia
© 2014 The American Federation for Medical Research
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
with normal TSH and cortisol. A diagnosis of SIADH secondary to West
Nile Meningoencephalitis was made.
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BACTERICIDAL ACTIVITY OF AFFINITY PURIFIED HUMAN
ANTIBODIES AGAINST M-RELATED PROTEINS OF GROUP
A STREPTOCOCCI AND IMPLICATIONS FOR VACCINE
DEVELOPMENT
Murrell DS1,2, Agbaosi T1,2, Niedermeyer SE1,2, Penfound TA1,2, Hysmith ND1,2,3,
Dale JB1,2. 1University of Tennessee Health Science Center, Memphis, TN;
2
Veterans Affairs Medical Center, Memphis, TN and 3St. Jude Children’s Research
Hospital, Memphis, TN.
Purpose of Study: Group A streptococcal (GAS) infections cause significant morbidity and mortality throughout the world. Although multivalent
M protein-based vaccines evoke a broad range of potentially protective
antibodies, the epidemiology of GAS infections in developing countries
has prompted a search for additional vaccine components that may result
in broader protection. M-related proteins (Mrp) are expressed by 83% of
clinical isolates of GAS and they contain opsonic epitopes. In contrast to
M proteins, the structures of Mrp’s are highly conserved within three related
families represented by Mrp2, Mrp4, and Mrp49. In the current study we
evaluated the potential protective efficacy of human antibodies against Mrp.
Methods Used: Human sera were assayed for the presence of Mrp antibodies
by ELISA. Antibodies were affinity purified over columns containing immobilized recombinant Mrp. Indirect bactericidal tests were performed by the
Lancefield method.
Summary of Results: Sera from normal adults containing significant levels
of Mrp antibodies were chosen for affinity purification. Affinity purified
Mrp4 antibodies (AP α-Mrp4) reacted in high titer with recombinant Mrp4
but not with Mrp2 or Mrp49. AP α-Mrp4 were bactericidal against M28
GAS, which is a heterologous serotype expressing Mrp4. Similar results
were obtained with AP α-Mrp2, which demonstrated bactericidal activity
against M2 GAS. In related studies, 56% of serum samples from pediatric
subjects contained significant levels of antibodies against at least one of
the three recombinant Mrp’s.
Conclusions: Human serum contains antibodies that are bactericidal against
GAS and a significant number of children and adolescents develop Mrp
antibodies in response to infection. Taken together, our results indicate that
Mrp’s may represent additional vaccine antigens that could broaden the potential protective efficacy of GAS vaccines.
213
ALPS: THE ABYSS AND THE PEAK
Mushatt DM, Subedee A. Tulane University School of Medicine, New Orleans, LA.
Case Report: A 57-year-old male with history of chronic alcoholism presented to the ER with severe respiratory distress. He had noted myalgia, malaise and non-productive cough at the onset of his illness a week earlier.
Progressive dyspnea with worsening cough and scant whitish sputum developed in the following days. He reported drinking at least 1 bottle of wine and
smoking more than 1 pack daily for 3 decades.
At presentation, he was diaphoretic and unable to complete sentences. He
was afebrile and normotensive with tachycardia, tachypnea and oxygen saturation 71% on room air. Auscultation revealed rales in all lung fields. There
was no murmur or gallop, or peripheral edema. Initial tests showed severe
leukopenia (0.30 X 103/μL) with absolute neutrophil count 0/mL, platelets
150 X 103/μL and creatinine 2.3 mg/dL. Blasts or schistocytes were not seen.
HIV test was negative. Chest X-ray showed patchy opacities in all lung fields.
Treatment with ceftriaxone and azithromycin was started. WBC count increased to 10.4 X 103/μL by day 3, but respiratory status worsened and mechanical ventilation was begun. Blood cultures from admission grew Streptococcus
pneumoniae sensitive to penicillin and ceftriaxone. WBC peaked at 31.8 X 103/μL at
day 7. Fevers abated on day 9, and the WBC normalized. On day 13, fever and worsening WBC and oxygen requirements were again noted. Chest CT showed dense
confluent opacities in all pulmonary lobes. Antibiotics were changed to meropenem
and vancomycin. On day 17, he became severely hypoxemic and expired.
Discussion: The syndrome of alcoholism, leukopenia and pneumococcal
sepsis (ALPS) was first recognized when it was observed that in patients
Southern Regional Meeting Abstracts
with Streptococcus pneumoniae bacteremia, alcoholism increased risk of
leukopenia, and the latter was associated with higher mortality (80% vs.
20.5%). ALPS is fatal in 83% of cases. Our patient had "rebound" leukocytosis after initial leukopenia, which has been noted in ALPS. Dual antibiotic
therapy has shown better survival than single antibiotic therapy in cases with
severe bacteremic pneumococcal pneumonia. The poor outcome despite appropriate antibiotics in our patient probably was a result of severe initial pulmonary inflammatory burden. This case highlights the importance of suspecting
ALPS in alcoholic patients who present with sepsis and leukopenia.
214
PERSISTENT FEVER IN AN INFANT WITH MENINGITIS
Palm S1, Balan A1,2, Ramji F1,3. 1Oklahoma University Medical Center,
Oklahoma City, OK; 2Oklahoma University Medical Center, Oklahoma City,
OK and 3Oklahoma University Medical Center, Oklahoma City, OK.
Case Report: Tuberculous meningitis is associated with high morbidity and
mortality rates; most survivors of advanced stages have permanent
disabilities: blindness, deafness, paraplegia, and mental retardation. Early diagnosis and treatment can result in great outcomes. We present a 12-month
old infant with tuberculous (TB) meningitis diagnosed due to persistent fever. A 12-month healthy female presented with 2 weeks of fevers, cough
and emesis. In the emergency room she was alert, active, but wheezing,
tachypneic and hypoxic. She developed seizures, so CSF obtained consistent
with meningitis: WBC 397 cells/mm3, RBC 60 cells/mm3, protein 221 mg/dl
and glucose of 43 mg/dl. Ceftriaxone and vancomycin was started empirically.
PCR for influenza was positive and a CXR with left upper lobe infiltrate, so
oseltamivir was begun. On hospital day (HD) 4, MRI brain showed basilar meningitis and cerebritis involving inferior left frontal and temporal lobes. She
remained febrile without clinical improvement and negative CSF bacterial
cultures, so on HD 6, CSF was repeated showing worsening indices: WBC
251, RBC 43, protein 260 and glucose of 29. TB was suspected due to lower
CSF glucose and positive tuberculin skin test. Further epidemiological inquiry
revealed a history of contact with family members in Honduras treated for
TB. A final lumbar puncture was performed on HD 8 prior to starting antituberculous drugs showing WBC 223, RBC 15, protein 225 and glucose 22.
Gastric aspirates and CSF cultures returned positive for Mycobacterium tuberculosis complex. After initiation of antiTB therapy she became afebrile and
clinically improved except for development of right-sided hemiparesis. At discharge on HD 17, hemiparesis was improving. She completed an 18-month
therapy course. While her MRI still showed abnormalities, she was clinically
developing well without deficits. Persistent fever despite antibiotic therapy,
presence of basilar meningitis and worsening CSF indices, particularly low
CSF glucose, in a patient with family from an endemic country should raise suspicion for TB meningitis. As our patient made a full recovery, our report
illustrates the importance of early diagnosis and treatment of TB meningitis.
215
INTRAVENOUS IMMUNOGLOBULIN THERAPY FOR
SEVERE CLOSTRIDIUM DIFFICILE ASSOCIATED
DIARRHEA
Panchavati PK2, Hassoun A1. 1Alabama Infectious Diseases Center, Huntsville,
AL and 2Huntsville Hospital, Huntsville, AL.
Purpose of Study: Clostridium difficle Associated Diarrhea (CDAD) is the
most common cause of hospital acquired diarrhea especially in elderly
patients and those with impaired antitoxin response. Severe CDAD can be
associated with significant morbidity and mortality even with the use of
recommended therapy. We report our experience with use of Intravenous Immunoglobulin (IVIG) in severe CDAD.
Methods Used: Retrospective chart review
Summary of Results: Retrospective chart review of seven patients with severe CDAD treated with IVIG from October 2007 to February 2013, three
females and four males. The mean age was 78.16 (range, 71-89) years, with
mean hospital stay of 13.83 (range, 4-31) days. All patients were diagnosed
with CDAD by fecal Clostridium Difficile toxin by polymerase chain reaction (PCR) and severe cases was diagnosed with more than 15 episode of Diarrhea per day, organ dysfunction and significant leukocytosis. Most
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
common presentation was Diarrhea, and hypotension with 70% of patients
been on pressers. Most common co-morbidities were diabetes mellitus and
malignancy. Most common risk factors were antimicrobial therapy and immunosuppressive. Mean WBC was 32.58 (range 22.31-46.97), while renal
insufficiency was present in 66.6%, with mean serum Cr of 2.18 (range
1.6-4.3), none of them had megacolon. All patients received Metronidazole
(500 mg PO TID) for mean 7 (range 4-11) days before the IVIG infusion
as well as Vancomycin (125 mg PO daily) before and after the IVIG infusion
for mean 15 (range 2-20) days, however two of them received additional
treatment, one received Rifaximin (200 mg PO TID) and Fidoxamicin (200 mg
PO BID) for total 4 day and 8 days respectively. All patients received one dose
of IVIG of (400 mg/kg) and had significant resolution of symptoms including
diarrhea within 2-4 days. Leukocytosis resolved within 4 days and renal insufficiency return to baseline in all of them within 7 days. Only one patient (16.6%)
had recurrence of the CDAD after 6 month and none of them needed surgical
intervention. None of the patient had any associated toxicity with IVIG.
Conclusions: Intravenous immunoglobulin is a useful adjuvant therapy for
the treatment of severe Clostridium Difficile Infection, further studies needed
to confirm its benefit.
The patient was empirically started on Bactrim and steroids for presumed
Pneumocystis carnii pneumonia (PCP). The patient underwent bronchoscopy
with bronchoalveolar lavage that was positive for PCP. The patient experienced an acute shortness of breath approximately 12 hours following the procedure and imaging showed development of a large left tension pneumothorax
that resolved with chest tube placement. The patient then developed another
acute episode of shortness of breath, tachycardia and tachypnea 24 hours after
removal of the chest tube. Imaging showed a new left apical pneumothorax.
The second pneumothorax resolved with 100% non-rebreather mask. Data
supports that current or previous PCP increases the rate of pneumothoraces in
AIDS patients. Furthermore, mortality rate increases linearly with recurrent
pneumothoraces. The overall mortality rate of patients with pneumothorax in
AIDS is approximately 34% (range between 10% and 81%) [3,4,5]. Early recognition and treatment of the underlying PCP is the key in decreasing mortality.
216
INHIBITION OF TOXIN PRODUCTION IN
STAPHYLOCOCCUS AUREUS KERATITIS
Patel R2,1, Bierdeman M1, Arana A1, O’callaghan R1. 1University of Mississippi
Medical Center, Jackson, MS and 2University of Mississippi Medical Center,
Jackson, MS.
Purpose of Study: Staphylococcus aureus, the most common cause of eye
infections, presents challenging antibiotic resistance and secretes multiple
damaging toxins. Killing these bacteria does not arrest the activity of the previously secreted toxins and there is no drug that can stop these toxins from
damaging tissue. Mediating the greatest ocular damage is α-hemolysin, a secreted protein able to lyse cells and cause apoptosis. The present study
hypothesizes that a flavonoid, able to inhibit α-hemolysin production in vitro,
can be developed into a formulation able to arrest toxin production in the S.
aureus infected cornea.
Methods Used: S. aureus (strain 8325-4) was grown overnight in tryptic soy
broth with or without the flavonoid (500 mg/mL). Each culture supernatant
was quantitatively assayed for toxin-mediated hemolysis of rabbit erythrocytes. To test the inhibitor in vivo, S. aureus (100 CFU) was injected into
corneas (n = 4 per group) of anesthetized rabbits and the inhibitor or its vehicle was topically applied. Damage was quantified by grading seven ocular
parameters on a scale of 0 to 4 and adding the grades to obtain a slit lamp
examination score (SLE).
Summary of Results: The results of this study show that the inhibitor
reduces toxin production in vitro by >1,000 fold. However, the SLE scores
(P = 0.58) and epithelial erosion area (P = 0.10) from this study were not statistically different from the control group.
Conclusions: The in vitro data provides evidence that the flavonoid
decreases the production of the cytolytic toxin. The in vivo data demonstrates
that the decrease in α-toxin production did not significantly reduce S. aureus
keratitis pathology in the rabbit eyes. However, the corneas were injected
with 1,000 CFU rather than 100 CFU (10-fold increase). More studies are
needed to correctly assess the treatment’s affect on 100 CFU injections and
furthermore, to enhance the in vivo effectiveness of the toxin inhibitor
217
A CASE OF THE NO FUN FUNGI
Patel R2, Grover I1. 1University of Mississippi Medical Center, Jackson, MS
and 2University of Mississippi Medical Center, Jackson, MS.
Case Report: The incidence of spontaneous pneumothorax (SP) in Acquired
Immunodeficiency Syndrome (AIDS) patients is 2-7 per 1,000 person-years
[4,6]. The frequency of pneumothorax complicating Pneumocystis carnii is
approximately 5%-10%. We report a case of 43-year-old African-American
male with a history of Hepatitis C, HIV/AIDS and non-compliance with
HAART regimen who presented with productive cough and hemoptysis for
two weeks and associated fevers, chills, chest pain and shortness of breath.
472
Large left pneumothorax with mild left to right midline shift.
218
UNUSUAL INFECTION IN A REPAIRED HEART
Powner JT1,2, Gallaher T1,2, Powner JT2,1. 1Georgia Regents University,
Augusta, GA and 2Charlie Norwood VAMC, AUGUSTA, GA.
Case Report: A 57 year old male with congenital transposition of the great
vessels, mitral and tricuspid bioprosthetic valves, atrial fibrillation on
coumdain, congestive heart failure, two previous cerebrovascular accidents,
and dual chamber pacemaker presented to an outside hospital with bleeding
and a supra-therapeutic INR. He was admitted for anemia and hypotension
complicated by acute kidney injury due to hypoperfusion, recurrent fever
and leukocytosis. He was initially started on levofloxacin for a possible pneumonia. Two sets of blood cultures at the sending hospital were positive for
diphtheroids. After transfer, again two sets of blood cultures drawn from different sites again diphtheroids. A transesophageal echocardiogram revealed
vegetations on his prosthetic mitral valve. Listeria monocytogenes was later
identified from his blood. After discussion with the patient, he revealed that
he had been eating soft cheeses.
The patient received intravenous vancomycin until the identification of
Listeria. He was then switched to intravenous ampicillin and gentamicin
and treated for a total of six weeks. He declined all surgical intervention,
and is doing well as of 2 months of follow up.
Infective endocarditis is usually caused by Streptococcus, Enterococcus
or Staphylococcus. The microorganism Listeria monocytogenes is a rare
cause of infectious endocarditis. Listeria monocytogenes is an aerobic
gram-positive rod that causes self-limited febrile gastroenteritis in healthy
patients, but can cause more severe disease in pregnant, neonatal, immunocompromised or elderly populations. Listeriosis can be a highly fatal
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
infection due to the difficulty in the diagnosis and patient populations usually
susceptible to Listeria monocytogenes. Listeria endocarditis is a complication
of listeriosis and can also occur as a single infectious entity. Mortality has actually been decreasing, but historical mortality rates are 35%. There may even be
certain Listeria monocytogenes strains that have increased ability to infect cardiac tissue. Treatment of choice is ampicillin and gentamicin, but other case
studies report using linezolid or linezolid and trimethoprim/sulfamethoxazole
with success. Valve replacement is recommended in valve dehiscence, cardiac
failure, or cardiac abscess.
219
BIRDS OF A FEATHER: A CASE OF DISSEMINATED
YERSINIA ENTEROCOLITICA INFECTION IN A
PREVIOUSLY HEALTHY FEMALE
Pryor J, Macariola D. East Tennessee State University, Johnson City, TN.
Case Report: A previously healthy 7 year old female presented with cellulitis of the scalp. The patient noticed a small, “pimple-like” area on her scalp
associated with pain that was first observed 3 weeks prior to admission. On
further questioning, it is revealed that an unidentified flying animal defecated
on her head 2 days before the initial pain and lesion were noticed. 4 days
prior to admission a wound culture was obtained at her pediatrician’s office,
and she was empirically treated with clindamycin. Physical exam was notable for a 2 cm raised ulcer with an eschar and a small amount of purulent
drainage on the scalp with cellulitis, bilateral tender posterior auricular
lymphadenopathy, and fever. She was started on IV vancomycin and
ceftriaxone with oral TMP-SMX and topical gentamicin. On hospital day
2, her previous wound culture returned positive for Yersinia enterocolitica.
Vancomycin was discontinued. Despite appropriate therapy, she remained
quite ill with intermittent fevers and painful lymphadenitis, so Gentamicin
was added. Due to the development of an urticarial rash, TMP-SMX and
ceftriaxone were discontinued and IV ciprofloxacin was started. Symptoms
continued to improve, and cervical lymph nodes spontaneously drained.
Hospital blood cultures were repeatedly negative. The patient was discharged
on day 8 with IV gentamicin and ciprofloxacin.
Yersinia, a Gram-negative bacillus, can cause differing degrees of illness
that ranges from gastroenteritis to serious systemic infections. Septicemia
has most commonly been seen in children and infants with iron overload
and various hemoglobinopathies. Common risk factors for infection include
consumption of chitterlings (pork intestines) and contaminated food and water. Yersinia has been isolated in the feces of various bird species such as
pigeons. Signs and symptoms of infections may include diarrhea (possibly
bloody), leukocytosis, mesenteric adenitis, fever, liver/spleen abscesses,
and reactive arthritis. Infection is treatable with TMP-SMX, gentamicin,
and 3rd generation cephalosporins. Though other common organisms are often responsible for cellulitis, Yersinia should be considered, especially if contact with birds is noted, as disseminated infection may occur.
220
HIV-ASSOCIATED HODGKIN LYMPHOMA:BEATING
THE ODDS
Reske T, Loch M, Ruiz MA, Parsons C. LSU, New Orleans, LA.
Case Report: HIV-associated Hodgkin Lymphoma (Ha-HL) is a non-AIDS
defining malignancy. Compared to the general population HIV patients have
a 14 times higher incidence to develop HL. Ha-HL is found in patients with
moderate immunologic impairment.
We present a 49 year old female, who was diagnosed 11 years prior with
HIV. She was on and off ART, when she presented 9 years later with weight
loss and lymph adenopathy. A LN and BM biopsy confirmed classical HL
(CD4 752 cells/mm3/ viral load 190 copies/ml). A PET scan showed diffuse
LNs and BM uptake. She was diagnosed with stage IV HL (IPS 3). At the
time of diagnosis she started to be compliant with her ART and was started
on ABVD chemotherapy. After 2 cycles she developed SOB and was diagnosed with low DLCO. Following this treatment related complication she developed ongoing pulmonary mycobacterium infections that precluded her
from restart of therapy. A PET scan 2.5 years after diagnosis showed complete resolution of her disease.
Ha-HL presents commonly as advanced stage disease. Classical HL with
mixed cellularity or lymphocyte depletion is the most common variant in
Southern Regional Meeting Abstracts
Ha-HL. This differs from HL in the general population. Co-infection with
EBV is found in almost all HIV patients, whereas only in 1/3 of non-HIV
infected individuals. Chronic antigen stimulation provoked by the HIV
virus is felt to lead to clonal cell expansion and promote lymphoma emergence. Introduction of antiretroviral therapy (ART) has altered outcomes
in HIV-HL significantly. Before ART the median OS was 15 months. A recent prospective trial showed a 90.7% OS after 4 cycles of ABVD chemotherapy+/- radiation or 8 cycles of BEACOPP. Treatment is complicated
by drug to drug interactions and treatment related toxicities. Even though
literature reports improved outcomes in HIV HL patients, biology and response to therapy remain at question. Micro environmental changes provoked by the HIV and co-EBV infection continue to be part of ongoing
study. Our patient’s response to only 2 cycles of chemotherapy appears
to beat the odds of appropriate therapy for optimal survival based on recent prospective trials. Mechanism of disease and tailored treatments continue to be poorly understood and need continued investigation. The
question remains on how to appropriately characterize individuals that require less aggressive treatment.
221
HYPERBARIC OXYGEN THERAPY IN THE TREATMENT OF
REFRACTORY OSTEOMYELITIS IN THE PEDIATRIC
PATIENT
Sexton TD, Jubran I. University of South Alabama, Mobile, AL.
Purpose of Study: To present a case series of novel treatments of chronic osteomyelitis refractory to traditional antibiotic therapy in pediatric patients
and to discuss the therapeutic benefits of hyperbaric medicine in refractory
osteomyelitis.
Methods Used: A case series of four patients ranging 11 to 18 years old
were given hyperbaric oxygen therapy for refractory osteomyelitis. MRI
showed osteomyelitis in all of these cases. Intravenous antibiotics were initiated in these patients. Wound debridement/drainage was done when indicated. This management was continued for six weeks with worsening CRP
and ESR per protocol this led to hyperbaric therapy treatments. US Navy
Table 66 consisted of 90 minute treatments, 5x’s/week. Within weeks the
CRP and ESR dropped. ESR, CRP and MRI results were followed throughout treatments.
Summary of Results: All patients were on the USN Table 66 mentioned
protocol, All patients were started on culture directed antibiotic therapy that
included cephalosporin and aminoglycosides. Prior to these Hyperbaric
treatments every patient in this case series presented with CRP> 24.0 and
ESR > 120 and after these treatments were conducted, significant improvement in MRI images and CRP/ESR were seen. Averagge CRP/ESR on discharge was 1.1mg/dl and 35mm/hr. respectively. Every patient had complete
resolution of Osteomyelitis.
Conclusions: When children present with osteomyelitis conservative management of antibiotics and wound care should begin. With little improvement
after 6 weeks or the osteomyelitis is refractory, adjunctive HBOT should be
considered. Refractory osteomyelitis that persists or recurs presents a high
probability of morbidity or mortality. Using these increased pressures; Hyperbaric oxygen delivery can ameliorate both acute and chronic sources of
ischemia. The decreased oxygen tensions in bone and tissue typically associated with bony infections can be returned to normal or above normal levels
while undergoing HBOT. HBOT shows considerable synergistic effects
when partnered with aminoglycosides and cephalosporins
222
NEUROSYPHILLIS IN A PENICILLIN-ALLERGIC PATIENT
Smith M, Gendusa P, Coleman R, Engel LS, Thien P. LSU Health Sciences
Center, New Orleans, LA.
Case Report: Neurosyphilis, caused by infection fo the brain by Treponema
pallidum, can be classified as early or late forms. Early neurosyphilis is more
common and can be asymptomatic or cause meningitis, cerebral arteritis, or
ocular disease. Late neurosyphilis can manifest as general paresis or tabes
dorsalis. The diagnosis is made with CSF studies. Current preferred treatment is Penicillin G.
CASE: A 68 year old African American man presented with an extensive
history of progressive lower extremity weakness and dementia. Initially the
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
473
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
patient was able to ambulate without assistance, and then in sequential order
required crutches, cane, walker and finally he has been wheelchair bound for
the past three years. He required assistance with all activities of daily living
including transfers to and from his wheelchair. Previous medical records
revealed a serum RPR titer of 1:132. He underwent a lumbar puncture and
his CSF was positive for FTA/ABS. CSF protein was elevated at 63mg/dl,
and CSF VDRL was nonreactive. The patient had a history of a penicillin allergy so penicillin desensitization was undertaken in the intensive care unit.
He successfully received three days of intravenous penicillin in the hospital
and was discharged home completion of IV penicillin therapy.
DISCUSSION: Neurosyphillis should not be discounted in a patient with a
negative CSF VDRL; the false negative rate approaches 30%. Patients with a
penicillin allergy may ceftriaxone or high dose doxycycline may be used to
treat patients with penicillin allergy but data is limited and titers should be
closely monitored if using these therapies. Patients who do not respond to the
alternate therapies may be candidates for penicillin desensitization. Of note,
there are no current controlled trials on the efficacy of the penicillin therapy.
Recommendations are based on penetrance of antibiotics into the CSF.
223
IS AZITHROMYCIN MODULATING INFLAMMATION VIA
TOLL LIKE RECEPTOR (TLR) PATHWAYS IN GROUP B
STREPTOCOCCUS (GBS) SEPSIS?
Upadhyay K1, Meals E1, English B2, Talati AJ1. 1CFRI, UTHSC, Memphis,
TN and 2Michigan State University, Grand Rapids, MI.
Purpose of Study: Combination of ampicillin(AMP) and azithromycin
(AZM) improved clinical signs, mortality and cytokine levels in our mouse
model of GBS sepsis. We proposed to study if the effect of AZM in modulation
of inflammation is secondary to bacterial factors[using AZM resistant strain
(AZR) of GBS] or host factors[using MyD88-/-, TLR9-/-, TLR2-/-mice].
Methods Used: Swiss Webster mice (wt 18g, age 3 wks) were injected
intraperitoneally (IP) with AZR GBS Ia (log 7-8 cfu/ml) and MyD88-/-,
TLR9-/- mice (wt 15 gm, age 3 wks) were injected with GBS Ia (log6 cfu/
ml). Mice were divided in 4 groups(grp). Grp 1 -no antibiotics, grp 2- treated
with AMP(100mg/kg/d; MIC ≤0.25 mcg/ml), grp 3- treated with AZM (10
or 50mg/kg/d; MIC 16ug/ml for AZR GBS and MIC ≤0.125ug/ml for AZM
sensitive strain ) and grp 4- treated with AMP+AZM. Antibiotics were administered q24h IP. Mice were monitored using “clinical sepsis score (CSS)”. Mice
were sacrificed at 120h and blood was obtained for later cytokine assay.
Summary of Results: For AZR GBS experiment; case fatality rate was
found to be 100% in grp 1 and grp 3; 50% in grp 2 and 0% in grp 4. Mean
serum TNF-α was lower in grp IV(246±67 pg/ml) compared to grp 1 (6952
±701 pg/ml), grp 2 (7209±1826 pg/ml) and grp 3 (5980±626 pg/ml)
(p≤0.05) The CSS was significantly lower in grp 4 compared to other grps
(fig). Also the case fatality rate was 100% in grp 1; 50% in grp 2 and 0%
in grp 3 and 4 using MyD88-/- mice. However, no difference was seen in
mortality among TLR9-/- mice. Similarly, the CSS was significantly lower
in grp 3 and grp 4 compared to grp 1 and grp 2 in MyD88-/- mice but no
significant difference was seen in TLR9-/- mice.
Conclusions: The effect of AZM in modulation of inflammation may be related to TLR9 and is seen even in mice infected with AZR GBS.
Medical Education, Medical Ethics and Advocacy
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
224
EMBRACING THE GREY: ADVOCATING FOR PATIENTS
WHEN THE STORY AND THE SCIENCE DON’T ADD UP
Burge L, Shropshire DL. University of Oklahoma Health Sciences Center,
Oklahoma City, OK.
Case Report: Skeletal fractures in children less than 1 year of age are
concerning for child abuse. Fractures that are unaccompanied by a reasonable trauma history usually result in further medical evaluation and a referral
to Child Protective Services (CPS).
Our patient is a 2 month old breast-fed, African American female who
reported to the ED after being seen by her PCP for acholic stools and direct
474
hyperbilirubinemia. HIDA scan was consistent with biliary atresia, and the
patient was admitted for further testing and treatment. A chest xray demonstrated multiple healing rib fractures. A full skeletal survey revealed an acute
corner fracture involving the distal left ulna and a concern for a second fracture involving the distal right femur. There was no evidence of brain injury or
retinal hemorrhage. CPS was notified of the concern for child abuse given
the xray findings, and the patient and her siblings were placed in emergency
foster care. A social evaluation of the family did not reveal previous reports
of abuse, neglect, or other familial risk factors.
The patient underwent a Kasai procedure but was unexpectedly found to
be profoundly hypocalcemic postoperatively and required IV calcium supplementation. Further evaluation revealed low levels of Vit. D and elevated
PTH; therefore, the patient was started on Vit. D and calcium supplementation. This case spurred a spirited debate among physicians as to the cause of
the fractures. The radiology literature contains a few case reports of children
with Vit. D deficiency and fractures. but these are disputed by most child
abuse experts. However, this particular child is more medically complex than
a typical infant with Vit. D deficiency. Ultimately, due to the medical uncertainty and clean social history, the child was reunited with her parents.
The physical signs of child abuse can be found in certain uncommon
medical conditions. Usually the combination of medical and social facts
provides the diagnosis; however, at times the answer is unclear. It is in these
times we must be willing to embrace the uncertainty of medicine and advocate for our patients and their families.
Pediatric Clinical Case
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
225
DON’T JUST ASSUME INFECTION: A CASE OF NONINFECTIOUS CERVICAL LYMPHADENOPATHY
Adimora-Nweke D, Freeman B. Tulane University SOM, New Orleans, LA.
Case Report: A 9 year-old girl with history of right neck swelling previously
treated with antibiotics represented one week later with fever, new left-sided
neck pain and swelling. She denied sore throat, stridor, dysphagia, animal exposure, recent travel, weight loss and night sweats. Her exam showed an indurated and tender left neck mass.
CBC showed neutrophil-predominate leukocytosis. CRP was elevated.
Throat culture, monospot, and PPD were negative. CT showed left sided cervical lymphadenopathy. Surgical debridement was performed and she was
empirically re-treated with antibiotics. Lymph node biopsy returned with diagnosis of Sinus Histiocytosis with Massive Lymphadenopathy. She was
treated with steroids and her cervical adenopathy improved.
Cervical lymphadenopathy (LAD) is a common pediatric complaint and
can be categorized into infectious and noninfectious causes, with infectious
causes being more common. Infections include viral respiratory illness, oropharyngeal infections, bacterial adenitis, EBV, CMV, tuberculosis, and cat
scratch disease. Noninfectious causes include leukemia, lymphoma, connective tissue disease, autoimmune diseases, and histiocytosis.
Rosai Dorfman Syndrome (Rd), also known as Sinus Histiocytosis with
Massive Lymphadenopathy, is a noninfectious cause of cervical LAD. This
disorder may present with unilateral or bilateral tender cervical LAD and is
often misdiagnosed leading to mismanagement with antibiotics, excessive
imaging, and debridement.
Laboratory examination for Rd includes CBC, CMP, urinalysis to assess
vital organ involvement. ESR, ferritin, and serum gammaglobulins are usually elevated. Diagnosis is made via nodal fine needle aspiration or excisional
biopsy. Histopathology shows distinctively large histiocytes with abundant
pale cytoplasm and phagocytosed lymphocytes.
Rosai-Dorfman most commonly affects the cervical lymph nodes, however extra-nodal disease occurs in up to 43% of affected patients. Imaging
such as ultrasound, CT, and MRI are used to determine disease extent in
patients with systemic symptoms. Patients with vital organ compromise or
nodal disease with complications require therapy. Treatment options include
excisional biopsy, debulking procedures, radiotherapy, corticosteroids, and
chemotherapy agents.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
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227
1Q21.1 DELETION SYNDROME:FURTHER EVIDENCE OF
PHENOTYPIC VARIABILITY
TESTICULAR ADRENAL REST TUMORS IN TWO SIBLINGS
WITH CONGENITAL LIPOID ADRENAL HYPERPLASIA
Almannai M, Martinez J. University of South Alabama Children’s &
Women’s Hospital, Mobile, AL.
Case Report: Deletions of chromosome 1q21.1 have been associated with
intellectual disability, microcephaly,and varieties of congenital anomalies
and dysmorphic features. We report four children with 1q21.1 chromosomal
deletion who presented with diverse clinical manifestations suggesting that
this genomic imbalance does not offer a specific clinical phenotype.
Case#1: A male child presented at 19 months with FTT, dysmorphic
features and speech delay. He has history of hypospadias and hypoplastic
proximal radii bilaterally. He has a 1.06 MB deletion that overlaps with the
TAR region.
Case#2: A male ascerteined at 20 months of age because of developmental delay, abnormal behavior, and dysmorphic features. He has periorbital
swelling, thick lips, puffy cheeks, and joint laxity. He has Ebstein anomaly
and was initially suspected to have Williams Syndrome. Family History
was positive for intellectual disabilities. He was found to have a deletion of
1.1 Mb in size. No parental genetic testing was done.
Case#3: A 33 months old Boy with autisim. He presented with microcephaly and language delay. He had no associated anomalies or dysmorphic
features and the family history was negative. He has a deletion of 2.4 Mb in size.
Case#4: A 10 years old male presented with developmental delay and
learning difficulties. He is overweight and has dysmorphic features(See table).
Later he developed goiter. His father has intellectual disabilities, but no parental
testing was done. He was found to have deletion of 1.23 Mb in size.
Conclusion: 1q21.1 genomic region is extremely complex. Patients with
deletions are more common than those with a reciprocal duplication of this region and most affected patients have a deleted region that spans 1.35 Mb. Our
experience confirms that although 1q21.1 microdeletions are associated with
human disease, the broad range of clinical manifestations prevent its characterization as a syndromic entity. In addition, no genotype-phenotype correlation
could be established, further complicating genetic counselling in these families.
Alsaheel A1, Roth C2, Congeni J3, Vargas A1. 1LSUHSC, New Orleans, LA;
2
LSUHSC, New Orleans, LA and 3Children’s Hospital, New Orleans, LA.
Case Report: Introduction: Congenital lipoid adrenal hyperplasia(CLAH) is
a rare and the most severe form of adrenal hyperplasia, frequently caused by
mutations in the steroidogenic acute regulatory protein (StAR)gene. Patients
with CLAH typically present with adrenal crisis in early infancy and those
with a 46,XY karyotype have female genitalia due deficient fetal sex steroid
synthesis. Recently it has been recognized that the phenotype can be quite
variable due to partial activity of StAR mutations(non-classical). Adrenals
and testes share common embryological origin and the testes harbor nests
of adrenal cells susceptible to ACTH stimulus.
We report two French brothers whom had been diagnosed with CLAH
(mutations in p.Gly221Ser, p. Arg53Leu, p. Arg188Cys of StAR gene) with
normal male genitalia presented with testicular adrenal rest tumors (TARTs).
Case A: A 14 year old Caucasian French boy, diagnosed with CLAH at
age 2 years, presented with mild testicular discomfort and negative history
for trauma, fever or genitourinary symptoms. He had a similar episode six
months earlier. Testicular ultrasound (U/S) showed two lesions in each testicle, largest one in the left testis 2.6×3.6×1.7 cm. Hormonal studies showed
elevated ACTH and PRA; tumor markers were negative. Hydrocortisone
dose was adjusted up 18 mg/m2/day, encouraging adherence to medication.
Follow up U/S showed diminishing tumor size.
Case B: The 15.5 year old brother was first diagnosed with CLAH at age
1.5 y. Screening testicular U/S showed solid masses bilaterally; largest one
was in the right 1.0 × 0.8 ×1.1 cm. Hormonal studies showed elevated ACTH
and PRA. Hydrocortisone was adjusted up 16 mg/m2/day and like his
brother the TARTs showed improvement in follow up U/S.
Conclusion: To our knowledge this is the first report of two brothers being affected by non-classical CLAH and TARTs. It expands the current
knowledge of the disease and its variable presentations. It also emphasizes
the importance of testicular U/S screening for early diagnosis and management of TARTs.
ACTH:adrenocorticotrophic hormone; PRA:Plasma renin activity.
228
NEONATAL AORTIC THROMBOSIS
Astrug L. 1UT Southwestern Dallas - Childrens Medical Center Dallas, Dallas,
TX and 2UT Southwestern Dallas - Parkland Hospital NICU, Dallas, TX.
Case Report: Coagulation issues in neonates are rare. Sources of thrombosis
can be due to abnormal anatomical features of vasculature, central line
complications, or coagulation disorder. Much is unknown regarding etiology/
location of thrombosis given its low incidence rates and limited literature. There
have been reported cases of neonates with aortic arch thrombosis with unstable
clinical presentations like seizures or cardiac disease symptoms. Unlike previously reported cases, this neonate did not present with hemodynamic instability,
signs of cardiogenic shock or seizures. Born at 34 weeks EGA, the infant had
partial systemic color change and lacked palpable femoral pulses in the first
minute of life. The infant’s father and kin have a strong history of thrombotic
disease. An exact diagnosis and etiology remains unknown despite extensive
hematologic evaluation. On initial examination, the neonate had partial color
change with a clear demarcation line superior to the umbilicus that horizontally
transcribed across the lower abdomen; superiorly pink, inferiorly pale. The femoral and pedal pulses were not present on palpation. After ruling out coarctation
of the aorta with an emergent echocardiogram, an abdominal/pelvic sonogram
with Doppler studies revealed an aortic thrombus that spanned from the abdominal aorta into the common iliac arteries, only partially obstructing blood flow.
After full body surveillance sonography, no additional thrombi were found. Head
ultrasound was normal. The infant was treated with heparin and transitioned to
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
475
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
aspirin therapy. Prior to discharge, abdominal and pelvic MRA demonstrated
complete dissipation of the abdominal thrombus without any further clot formation. This case is significant due to minimal presenting clinical symptoms and
questionable timing of thrombosis occurrence. Presentation at birth with minor
perfusion difficulties suggests that the thrombus had formed in utero. There were
no anatomical abnormalities or complications consistent with prolonged decreased blood flow, suggesting that the thrombus likely formed later in fetal life.
Besides partial color change, the neonate did not present with any other
symptoms. Aortic thrombosis can be extremely detrimental, increasing morbidity
and mortality if not diagnosed immediately.
229
OXYGEN REQUIREMENT AS A SCREENING TOOL FOR THE
DETECTION OF LATE PULMONARY HYPERTENSION IN
EXTREMELY LOW BIRTH WEIGHT NEONATES
Aswani R1, Hayman L1, Nichols G1, Isshiki G1, Luciano A1, Amankwah E2,
Leshko J2, Dadlani GH2,1. 1University of South Florida, Tampa, FL and 2All
Children’s Hospital- Johns Hopkins Medicine, St. Petersburg, FL.
Purpose of Study: Many Extremely Low Birth Weight(ELBW) neonates develop Pulmonary Hypertension (PH) late in their clinical course and over
60% go undetected by early screening echocardiography.The signs of PH
are subtle and may be masked by underlying broncho-pulmonary dysplasia
(BPD). No standardized screening protocol exists for the detection of late
PH. The Purpose of this study is to assess the utility of oxygen supplementation as a predictor of late PH in ELBW neonates.
Methods Used: A retrospective single center review of 230 ELBW neonates
surviving more than 30 days between January 2008 to December 2011 was
performed.Logistic regression was used to estimate odds ratio(OR) and
95% confidence intervals(CI) for the association between oxygen supplementation and the diagnosis of PH.
Summary of Results: The incidence of late PH was 8.3%(19/230). Unadjusted
logistic regression analysis revealed that compared to neonates with oxygen
supplementation<30%, neonates requiring oxygen supplemental ≥30%had an increased risk of developing PH (OR=3.77, 95% CI=1.42-10.00, p=0.008). After
adjusting for birth weight, the elevated risk attenuated and was of borderline statistical significance (OR=2.47, 95% CI=0.89-6.84, p =0.08). Receiver operating
characteristic curve analysis showed an area under the curve of 0.69.
Conclusions: The need for oxygen supplementation ≥30% at day of life
30 may be a good screening tool for detecting late PH in ELBW neonates.
Clinical Characteristics of the Study Participants
none associated with HLH. This is the first reported case of fatal HLH
presenting as severe VOD in a non-BMT acute lymphoblastic leukemia
(ALL) patient.
Case: 18 year old female with existing autoimmune disease was diagnosed
with ALL. During chemotherapy she was admitted for zoster encephalitis
and developed platelet refractoriness, encephalopathy, and sudden clinical
deterioration. Laboratory/radiographic evaluation identified macrophage activation syndrome and VOD. Bowel sloughing and abdominal distension
prompted an exploratory laparotomy and liver biopsy which confirmed histologic HLH. The patient eventually succumbed to fulminant HLH despite receiving optimal therapy.
Discussion: HLH is a rare histiocytic reactive process due to mutations in the
perforin, MUNC13-4, or syntaxin 11 genes, or secondary to malignancy, infection, or autoimmune disease. The predominant symptoms are fever and
organomegaly. Lab testing reveals abnormalities in blood counts, liver function, fibrinogen, lipids, albumin, and electrolytes. HLH is most commonly a
secondary process rather than a primary disease. There are two reported
cases of familial HLH progressing to acute leukemia, one heterozygous for
MUNC13-4 and the other with reduced natural killer (NK) cell function
and perforin expression. This patient had potential HLH triggers that were
both infectious and autoimmune without any genetic predisposition to HLH.
Conclusion: Severe VOD can be a presenting sign of HLH in a non-BMT
oncology patient. Early initiation of HLH-specific therapy may decrease
morbidity and mortality risk for these patients.
231
SACROCOCCYGEAL TERATOMA WITH MULTIPLE
RECURRENCES BY INTRADURAL EXTENSION IN THE
NEONATE
Bechtel AS1, Gauger CA2. 1University of Florida Jacksonville, Jacksonville,
FL and 2Nemours, Jacksonville, FL.
Case Report: Sacrococcygeal teratomas are one of the most common
tumors of the newborn, and the most common site of germ cell tumors.
These can be classified by the Altman classification which breaks them in
to four types (Table 1). These can be complicated by relapse, as well as, on
rare occasions, growing teratoma syndrome (GTS). GTS is defined as an enlarging mass during or after chemotherapy with normal serum markers. Recurrence, does occur, but to our knowledge multiple recurrences are seemingly
rare, and even more so, via intradural extension. While this case did not fit criteria for GTS, these multiple recurrences were also unique as they were distant
from the original site. This case will discuss the presentation of a preterm infant,
prenatally diagnosed with a sacrococcygeal teratoma. Her initial presentation
and course, from newborn to two years of age. This included multiple
recurrences in varying sites, multiple histological types and both medical management via chemotherapy and surgery. Review of this case gives insight into
the differing presentations of recurrence and a more complex course, as well
as the importance of strict, regular and prolonged follow-up.
Altman Classification
232
230
FIRST REPORTED CASE OF HEMOPHAGOCYTIC
LYMPHOHISTIOCYTOSIS PRESENTING AS
VENO-OCCLUSIVE DISEASE IN A LEUKEMIA PATIENT
Barros T, Gratias E. UT Chattanooga, Chattanooga, TN.
Case Report: Introduction: Hemophagocytic lymphohistiocytosis (HLH)
is a life-threatening inflammatory disorder. There are many reported cases
of HLH in bone marrow transplant (BMT) leukemia patients, and a known
association between HLH and hepatic veno-occlusive disease (VOD). There
are only scattered cases of severe VOD in non-BMT oncology patients, and
476
PEDIATRIC EDUCATION FOR PATIENTS WITH SICKLE
CELL DISEASE
Bluett-Mills GM1, Garry C2, Chiu M2, Chavan R1. 1Tulane University, New
Orleans, LA and 2Tulane University, New Orleans, LA.
Case Report: The following was presented in longer form at an educational
day aimed at patients with Sickle Cell Disease (SCD) and their family
members. The case is an amalgamation of multiple patients.
S is the first baby born to his parents. When he is a day old, a sample of
blood is taken for a newborn screen and sent to a state lab, where he is diagnosed with sickle cell disease (SCD).
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
S and his parents are sent to a hematologist, where they learn that both
his parents are carriers with one normal hemoglobin gene and one sickle
gene. If they have another child together, there is a 25% chance that their
next child will have SCD. At this first visit S is started on PCN prophylaxis.
He has more blood work drawn during the first year of life that shows he has
HbSS, the most common and severe form of SCD.
At six months old, S has lower levels of hemoglobin that other children
his age.
At nine months old, S’s parents notice pain and swelling of the fingers of
his right hand. He is treated at home for dactylitis.
At twelve months old, S has a fever. He is taken to the emergency room,
where blood cultures are drawn and he is given antibiotics.
At three years old, S is more tired than usual and pale. He is complaining
of belly pain. His mother is able to palpate his spleen several centimeters below his rib. He is admitted to the hospital for splenic sequestration.
At 5 years old S has a fever. A few days later he is tired and pale. He is
much more anemic than usual and not making any new red blood cells. He is
diagnosed with an infection called parvovirus B19.
At 7 years old, S has his first pain crisis in his right leg. Over the year, he
learns ways to try to prevent crisis, including drinking plenty of water,
avoiding extreme temperatures, regular visits to his hematologist and pediatrician, and staying up to date on shots. In addition to standard shots, he
makes sure to get a yearly flu shot and gets pneumovax to prevent against
bacteria normally killed by the spleen.
As a child and teenager, S also gets screening and prevention for longer
term effects of SCD, including yearly eye exams starting at age ten,
transcranial doppler to measure of risk of stroke in children, and pulmonary
function tests.
Southern Regional Meeting Abstracts
to bronchodilators. Symptoms during the prior episode were similar, but
had resolved weeks before the onset of his current symptoms. There was
no associated fever, apnea, cyanosis, vomiting, diarrhea or rash. On exam,
he had diffusely course breath sounds, without nasal flaring, wheeze or supplemental oxygen requirement. Labs noted leukocytosis and chest radiograph revealed a cavitary lesion in the right upper lung field and round
opacities in the right middle and lower lung fields. Chest CT revealed a
thick-walled cystic lesion in the right upper lobe without air fluid level, an
area of consolidation immediately inferior to this, and a second area of consolidation in the right middle lobe.
Given these findings and history of pneumonia, there was concern for anatomic abnormality such as congenital pulmonary airway malformation
(CPAM) versus a complication of pneumonia. After a 14-day course of
antibiotics, repeat chest radiograph revealed interval decrease in size of the
right middle and lower lung consolidations, with continued demonstration
of the right upper lobe cystic lesion. Chest radiograph one month later
showed interval resolution of the right upper lobe cystic lesion and right middle and lower lung consolidations.
CPAMs are hamartomatous lesions which originate from tracheal, bronchial, bronchiolar, or alveolar tissue. Patients may be diagnosed prenatally,
in the newborn period, or remain asymptomatic until later in life. Severity
of symptoms reflects the extent of pulmonary involvement. In neonates,
findings include tachypnea, cyanosis and respiratory distress. Cough, dyspnea and recurrent pneumonia may be seen later in childhood. Imaging
findings may include cysts with or without fluid or air, and typically involve
only one lung. Up to 59 percent of cases regress prenatally. Post natal resolution is much rarer, making a pneumatocele more likely in our case. These
are thin walled cysts which occur in association with bacterial pneumonia,
particularly Staphylococcus aureus. Often, pneumatoceles involute spontaneously without long term sequelae.
233
PERSISTENT HYPOXEMIA IN A NEWBORN: AN EARLY
PRESENTATION OF NEHI SYNDROME
235
Bojanowski CM1,2, Messer A2, Levine S3, Beatty K2. 1LSUHSC-NO, New
Orleans, LA; 2LSUHSC, New Orleans, LA and 3LSUHSC, New Orleans, LA.
Case Report: Neuroendocrine cell hyperplasia of infancy (NEHI) is a recently described children’s interstitial lung disease (chILD) of unknown etiology. This is a rare disorder defined by clinical features, radiographic
findings, and lung tissue bombesin immunostaining. Typical presentation
includes tachypnea, hypoxia, and crackles in infants aged 1-24 months. High
resolution CT (HRCT) of the chest typically reveals a characteristic pattern
of ground glass opacities, most commonly in the right middle lobe and
lingula as well as mosaic air trapping in the lower lung fields. However,
the diagnostic gold standard is lung biopsy demonstrating increased numbers of bombesin-immunopositive pulmonary neuroendocrine cells within
bronchioles and alveolar ducts without evidence of other pulmonary abnormalities. The term "NEHI syndrome" is used to indicate a diagnosis of NEHI without lung biopsy based on a consistent clinical presentation and radiographic
findings. Here we describe the presentation and diagnosis of NEHI syndrome
in an 11 do ex 39 WGA female born via NSVD with no significant interval history and an unremarkable neonatal course. She presented with tachypnea,
subcostal retraction and hypoxia (O2 saturation of 71% on room air). Septic
work up remained negative. Echocardiogram was positive only for a small patent foramen ovale. HRCT showed a fine reticulonodular pattern. She was eventually weaned to room air at two months of age. Treatment of this disease is
supportive with oxygen supplementation and optimization of nutrition as the
mainstays of care. Given the general good outcomes of this disease, with median duration of symptoms approximately 18 months, correct diagnosis
provides ensuring prognostic information for family members.
COLCHICINE TOXICITY
234
NOW YOU C-PAM, NOW YOU DON’T
Bowles E DeBord J. Tulane University, New Orleans, LA.
Case Report: A three-month-old boy with a history of community-acquired
pneumonia diagnosed clinically one month prior was admitted for recurrence
of cough, congestion, and abnormal findings on chest radiograph. His
parents described a dry cough, worse at night, which responded minimally
Brennard MG, Monroe K. University of Alabama at Birmingham,
Birmingham, AL.
Case Report: An 18yo female presented with acute onset of nausea and nonbloody, non-bilious vomiting thought to be due to food poisoning by the
patient’s mother, but the patient quickly admitted to ingestion of her mother’s
colchicine in a suicide attempt. The ingestion happened 6 hours prior to presentation. On review of systems, she also complained of blurry vision,
frontal headache, sore throat, shortness of breath with intermittent sharp
chest pain and slight cough. She also felt light-headed and had body aches
“all over”. Vital signs were as follows: T 100.5F, HR 95, BP 113/76, RR
21, O2 sat 98% on room air. On exam, she appeared moderately ill with normal breath sounds, tachycardia and regular rhythm with no murmur. Her abdomen had hypoactive bowel sounds and tenderness at suprapubic area and
right upper quadrant. She was neurologically intact but had poor effort in
strength exam due to muscle aches. She was oriented with flat affect and depressed mood, but easily irritated. Initial labs revealed negative urine pregnancy test and drug screen, normal electrolytes and blood counts, normal
acetaminophen and aspirin levels. Abnormal labs had elevation of
transaminases, amylase and lipase, and creatine kinase. The effects of colchicine toxicity have been documented based on amount ingested and timing
post-ingestion. There is a direct relationship between the amount ingested
and severity of symptoms. During the first 24 hours, GI symptoms predominate with abdominal pain, vomiting, diarrhea, and nausea. This loss of fluid
can lead to dehydration, electrolyte imbalance, and even hypovolemic shock.
Hematologic factors may also be evident in the first stage, such as leukocytosis and coagulation factor consumption by the liver. Stage 2 is the most
dangerous and lasts from about day 2 until one week after ingestion. Critical
changes to organ function can lead to hepatic failure, renal failure,
arrythmias, cardiogenic shock, and even acute respiratory distress syndrome
(ARDS). In addition, bone marrow hypoplasia causes pancytopenia with a
high risk of sepsis. If the patient survives this devastation, the third stage
of toxicity brings resolution of organ failure, rebound leukocytosis, and alopecia. Our patient developed pancytopenia and required 2 weeks for full recovery of organ function, resolution of pain, and ability to tolerate nutrition
by mouth.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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236
EMERGENCY DEPARTMENT DIAGNOSIS OF SEVERE
ORBITAL PROPTOSIS AND FUNGAL RHINOSINUSITIS
REQUIRING EMERGENT ETHMOIDECTOMY
Burhop J, Clingenpeel J, Poirier MP. Eastern Virginia Medical Center,
Children’s Hospital of the King’s Daughters, Norfolk, VA.
Case Report: 15 year-old male, worsening right periorbital swelling, headache associated nocturnal awakening and diplopia. Prompted to visit emergency department due to 5 days of bifrontal headache and emesis. Child
admits to history of asthma treated with albuterol and inhaled steroids along
with chronic nasal congestion. Child denies travel or trauma history.
A CT of the orbits and sinuses obtained along with an MRI directly from the
emergency department revealing the diagnosis. Emergent ethmoidectomy, maxillary antrostomy and sphenoidotomy performed in OR. Surgical pathology and
culture consistent with fungal hyphae and eosinophilic infiltration without neoplastic changes. The right eye was saved with complete restoration of vision.
With aggressive eosinophilic and IgE mediated inflammatory response,
patients with history chronic sinusitis are a setup for invasive allergic fungal
rhinosinusitis. Most patients are immunocompetent, with rare findings of
diplopia, in additional to proptosis and headache, as was the case with our
patient. The involvement of ophthalmic signs is of particular concern. Early
identification in the emergency department, or acute care setting, along with
aggressive surgical intervention and debulking procedures, aids in resolution
of diplopia and irreversible ophthalmic damage.
the right side. Chest x-ray and CT thorax revealed significant amount of
complex fluid filling the right hemithorax.
Chest tube placement yielded 2.5L of cloudy exudative fluid. Output had
not subsided and the fluid color became milky white. Fluid triglyceride and
cholesterol levels were low. On day 16, lymphoscintigraphy (Fig. 1) identified a right apical leakage point. A medium-chain triglyceride (MCT) diet
was started but output persisted. On day 36, he was placed exclusively on total parenteral nutrition (TPN) and started on octreotide. On day 41, he underwent mechanical pleurodesis and ligation of his thoracic duct. Chest tube
output gradually decreased and he was discharged on day 60.
The likely etiology of the effusion is trauma. Presentation is consistent
with a gradual accumulation of chyle. Though his fluid analysis was equivocal
for a chylous effusion, lymphoscintigraphy ultimately confirmed the diagnosis.
Management relies on a MCT diet. Persistent output may warrant exclusive TPN. Octreotide is reported as an effective adjunct. Those who fail conservative therapy need pleurodesis and/or thoracic duct ligation.
Clinicians need to recognize and initiate therapy early to reduce longterm morbidities including malnutrition, immunodeficiency, and eventual
respiratory compromise.
FIG. 1
238
METASTATIC PEDIATRIC PARATHYROID CARCINOMA: A
THERAPEUTIC ROLE FOR CINACALCET
MRI with right side peri-orbital and sinus enhancement consistent
with diagnosis.
237
CHYLOUS EFFUSION IN A NINE YEAR OLD
Chan J1, Gillispie M2, Demissie S3. 1University of South Florida, Tampa,
FL; 2University of South Florida, Tampa, FL and 3All Children’s Hospital,
St Petersburg, FL.
Case Report: A 9-year-old boy presented with chest pain while playing football that resolved with rest. On exam, he was comfortable without dyspnea or
distress. There were decreased breath sounds and dullness to percussion on
478
Davidson J1, Lam C2, Bahrami A2, Diaz-Thomas A1. 1University of
Tennessee Health Sciences Center, Memphis, TN and 2St. Jude Children’s
Research Hospital, Memphis, TN.
Case Report: Parathyroid carcinoma is an exceedingly rare form of primary
hyperparathyroidism with an estimated prevalence of 0.005% of the US
adult population. It is even more unusual in children as only ten cases have
been reported. An 8-year-old African-American girl with neck pain, hypercalcemia (12.5 mg/dL), and elevated parathyroid hormone (453 pg/mL)
was originally diagnosed with parathyroid adenoma. Parathyroidectomy
was performed, but after multiple recurrences and escalating hypercalcemia over a period of five years, the patient’s disease was reclassified as malignant. Germline mutation analysis of the MEN1 and CDC73 genes
revealed no previously described mutations. The disease eventually metastasized to the lungs, and the patient underwent palliative bilateral lung
metastasectomies followed by post-operative initiation of cinacalcet. She
has maintained near-normal serum calcium levels and has been symptom
free for a period of 12 months. We demonstrate that cinacalcet is a safe
and efficacious component of the chronic management of hypercalcemia
in this setting.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
Physicians should consider MAS in the differential diagnosis of hypertension
in children. In the absence of renal dysfunction or target organ damage, medical
management of MAS is feasible if BP is well controlled on two antihypertensive
medications.
241
DUPLICATION 11Q22.3: CASE REPORT AND DELINEATION
OF A DISTINCTIVE ENTITY
Serum calcium (solid line) and PTH levels (dashed line)
stabilized on increasing cinacalcet maintenance therapy
(column graph, range 60-270 mg/day). Two 20 mg
pamidronate infusions and repeat unilateral pulmonary
metastasectomy (arrow) and were eventually employed
months later for increasing calcium and PTH levels. Ref. range
for serum calcium noted [8.5-10.5mg/dL].
Gallois JB1, Lacassie Y2. 1LSU Health Sciences Center, New Orleans, LA
and 2Children’s Hospital, New Orleans, LA.
Purpose of Study: With the advance of new molecular tests, new syndromes
are being quickly delineated. However, new information is sometimes difficult to interpret and proves challenging to determine if it is pathogenic or
represents normal variation. We report a 3-years-7-months-old girl, who
presents a quite evident abnormal phenotype including developmental delay/
intellectual disability, some autistic features, many dysmorphic features and
dermatoglyphic findings highly suggestive of a chromosomal abnormality in
which the aCGH showed a duplication 11q22.3 of 2.9 Mb involving at least
14 genes. However, the report was unclear due to the lack of published data implicating this duplication in human disease. The recent observation (personal
communication and one published reference) of at least 2 other patients with developmental delay/autistic and dysmorphic features and similar duplication allow us to suggest that this duplication is pathogenic and would represent a
new distinctive disorder. After this report, we believe that additional similar
patients will be identified and the existence of this new entity confirmed and
recognized.
Methods Used: Clinical evaluation and microarray were used.
Summary of Results: Duplication 11q22.3
Conclusions: This duplication seems to be pathogenic and represent a new
distinctive disorder.
242
A CASE OF BACK PAIN AND LOWER EXTREMITY
WEAKNESS PRESENTING TO THE PEDIATRIC
EMERGENCY DEPARTMENT. PALLAVI GHUGE MD, MPH
UNIVERSITY OF ALABAMA AT BIRMINGHAM. KATHY
MONROE MD UNIVERSITY OF ALABAMA AT BIRMINGHAM
240
MIDAORTIC SYNDROME IN PEDIATRIC PATIENT WITH
KNOWN RENOVASCULAR HYPERTENSION
Doose M. Tulane Hospital, New Orleans, LA.
Case Report: A 5 year-old African American female presented with decreased appetite and urine output, fatigue, abdominal pain, emesis and headache. She was born full term with no complications. Family history revealed
presence of hypertension of unknown etiology in both parents. Examination
revealed blood pressure (BP) of 170-210/120-160 mmHg (95% of BP 110/
70 mmHg), serum creatinine of 0.4 mg/dL, potassium of 2.7, bicarbonate
of 24 mmol/L and trace protein in the urine. Intravenous infusion of sodium
nitroprusside was initiated to manage hypertensive emergency. Additional
data showed elevated plasma renin activity (PRA) of 300 ng/ml/h, consistent
with renovascular hypertension. CT of the abdomen demonstrated that the
left kidney was 30% smaller than the right. MAG3 nuclear scan showed decreased function of the left kidney at 15%. Angiogram demonstrated 99%
stenosis of the left renal artery and slightly diminished caliber of infrarenal
abdominal aorta. Following left nephrectomy, patient did not require any
anti-hypertensive medications for a number of years. At follow-up clinic visit
at age of 15 years, BP was measured at 155/87 in left arm and 149/70 in
right arm. CT angiogram revealed a normal appearing right renal artery
and a 12 cm-long narrowing of the infrarenal aorta with maximum diameter
of 6-7 mm compared to proximal and distal segments, both with diameter of
11 mm. Given normal GFR, absence of symptoms or signs of inflammation,
and ability to control BP with two medications, no surgical intervention was required to treat midaortic syndrome (MAS). This case illustrates association of
unilateral renal artery stenosis presenting as hypertensive emergency early in
life with later development of progressive symptomatic idiopathic MAS.
Ghuge P, Monroe K. University of Alabama at Birmingham, Birmingham, AL.
Case Report: Our patient is an 11 year old previously healthy Caucasian female who presented with inability to move her legs for the last 4-5 hours. She
had one week history of bilateral leg and lower back pain that was associated
with her legs falling asleep which would resolve in a few hours. She denied
any trauma, URI symptoms, vomiting, bowel or bladder incontinence, upper
extremity weakness, difficulty with speech, vision or swallowing. Review of
systems was otherwise negative. Physical exam revealed an alert but anxious
girl with stable vital signs. She had intact cranial nerves and a normal neuro
exam in upper extremities. Strength was 1+ at her hips, knee and ankle, sensory exam demonstrated absent sensations from mid thigh downwards with
no 2 point differentiation and absent pain sensation. Plantars were upgoing
on the left but no clonus. Reflexes were intact. She was tender to palpation
over her lower thoracic and upper lumbar vertebrae. Differential included
mass occupying lesion, secondary metastasis, transverse myelitis, or possibly
early Guillian Barre syndrome. Labs showed normal CBC, urinalysis, CMP,
CRP and ESR. MRI of thoracic and lumbar spine was normal. Neurology
was consulted, CSF studies were obtained which were normal. Mom mentioned that she saw a flicker of movement in the patients legs while she
was in the MRI and further stated that patient had a hard time trying to adjust
since the arrival of her sibling 2 yrs ago. On repeat assessment, her sensory
level was not consistent with a dermatomal distribution. Gradually over the
course of time in the emergency department her exam improved. She initially
walked to the bathroom with assistance and later walked out of the ED on
discharge with plans to follow up with neuro psychologist and a counselor.
Conclusion: In summary her findings were consistent with Conversion disorder as her neurological exam gradually returned to normal. Patients like
this one can pose a medical dilemma and there is a danger of misdiagnosis
without a thorough work up to look for medical and organic etiologies of
the presenting symptom.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
479
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
243
BRONCHOCENTRIC GRANULOMATOSIS IN A TWO YEAR
OLD WITH TUBERCULOSIS
Gipson K1, Harrison E1, Price E1, Stark M2, Seybolt L1, Pepiak D1.
1
LSUHSC School of Medicine, New Orleans, LA and 2LSUHSC School of
Medicine, New Orleans, LA.
Case Report: Bronchocentric granulomatosis (BCG) is a rare histologic pattern of palisading histiocytes forming necrotizing granulomas of the distal
airways centered on bronchi/bronchioles. The most common etiology is a hypersensitivity reaction to Aspergillus, with majority of cases culture positive.
This pattern may be observed in other fungal, viral and mycobacterial
infections. CXR of BCG may manifest as single/multiple nodules or as extensive areas of consolidation.
We present a two-year-old male with exposure to active Mycobacterium tuberculosis (TB), positive interferon-gamma release assay and positive PPD with
persistent right perihilar and basilar opacification on serial CXR (Figure 1).
Transbronchial biopsy of the right lower lobe showed predominately uninucleate cells with reniform nuclei forming loose palisading necrotizing granulomas,
a histologic pattern consistent with BCG, and was negative for TB by cultures
and special stains.
This case illustrates the histologic pattern of BCG as a complication of
pulmonary TB in a very young patient. Interestingly, this child initially had
difficulty taking anti-TB medications, requiring a gastrostomy tube to facilitate treatment. We propose that the child’s initial poor medication adherence
may have contributed to the development of this rare complication. Considering this patient presentation, BCG should be added to the clinician’s differential when evaluating a ‘persistent opacification’ on CXR.
resuscitated, and started on ammonia removal. Family history included consanguineous parents and SIDS. Newborn screening and acylcarnitine (AC)
profile showed elevated long-chains and low free carnitine. Gene sequencing
showed a homozygous deletion for carnitine-AC translocase (CACT) deficiency. A low fat formula with intermittent feeds, medium chain triglyceride
oil and carnitine were started. Immediately, long-chains and long-chain to medium-chain ratio (LC:MC) decreased. Her echocardiogram showed normal
heart function at 1 month-old.
AC profiles were alike at 4 and 9 months-old with low free carnitine and
improving LC:MC. Though she was asymptomatic, her follow-up echocardiogram at 9 months-old showed dilated left ventricle (LV) with moderate
to severely decreased LV systolic function (LVSF). She was diagnosed with
dilated cardiomyopathy (DCM), and furosemide and captopril were begun.
Continuous feeds were begun to support euglycemia and to avoid long-chain
fat recruitment. After 3 weeks of treatment, echocardiogram revealed lownormal LVSF. At 1 year-old, AC profile showed improved LC:MC and low
free carnitine, however echocardiogram revealed LV dilation and worsened
LVSF. 1 week after optimizing feeds, LVSF improved substantially.
After birth, the infant experiences periods of fasting and must mobilize
free fatty acids. CACT transports long-chain fatty acids (acylcarnitine) into
the mitochondrial matrix, so they can be converted for use in the beta-oxidation cycle; generating acetyl-CoA for ketogenesis and gluconeogenesis.
Patients with CACT deficiency often present as neonates with cardiac arrest,
hypoketotic hypoglycemia, hyperammonemia and lactic acidemia. Lethal
cardiac arrhythmias are due to an accumulation of long-chain AC, which
causes an increase in intracellular Ca2+. Treatment goals are to: avoid hypoglycemia, prevent long-chain AC production and supply ample carnitine.
We postulate the improvement in heart function was due to nutrition optimization, although she was treated with usual medications for DCM. Due
to the high risk of mortality in CACT deficiency and the rapid weight gain
of infants, nutrition is vital.
245
POINT OF CARE ULTRASOUND IDENTIFICATION OF AN
INTRACRANIAL FOREIGN BODY
244
HEART FAILURE IN A PATIENT WITH
CARNITINE-ACYLCARNITINE TRANSLOCASE DEFICIENCY
Gradidge E, Asbeck E, Yang S, Andersson H, Morava-Kozicz E. Tulane University School of Medicine, New Orleans, LA.
Case Report: An hours-old Caucasian female presented with hypothermia,
hypoglycemia and hyperammonemia. At 4 days, she became pulseless, was
480
Hanna PJ, Baker MD. UAB, Birmingham, AL.
Purpose of Study: To describe how point of care ultrasound was used to describe skull fracture and intracranial foreign body
Methods Used: Case Report
Summary of Results: A 7 year old male presented to the emergency department 1 day following a gunshot wound to the forehead. Patient was reportedly shot by an air rifle (760 Pump Master ®) at close range by a 4 year
old relative. There was no alteration of mental status or loss of consciousness. Vital signs, Glasgow Coma Scale (15), and neurological exam were
all normal. A 1 mm puncture wound was noted in the right frontal scalp without palpable foreign body, soft tissue swelling, or bony step off. Plain radiograph revealed a metallic foreign body without evidence for skull fracture.
Point of care ultrasound demonstrated a right frontal skull cortical discontinuity with hyperechoic foreign body with ring artifact. Computed tomography revealed small open calvarial frontal skull fracture with frontal cerebral
hemorrhage. The patient was admitted to the Pediatric Neurosurgery service
and underwent removal of an intracranial, extradural foreign body. The patient had an uneventful recovery and was discharged the following day.
Conclusions: Utility of Point of care Ultrasound has been well described for
soft tissue foreign bodies and fractures. Ultrasound is not generally useful for
identifying foreign bodies embedded in bone. Bone acts as a strong reflector
of sound waves making foreign body identification with ultrasound difficult.
We described a case in which a metallic foreign boy was localized immediately beneath a skull fracture.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Skull Fracture with Step Off.
246
ELEVEN-MONTH-OLD WITH ALTERED MENTAL STATUS
Hines K, Dietiker K, Monroe K. University of Alabama at Birmingham,
Birmingham, AL.
Case Report: An eleven-month-old male was transferred from an outside
hospital emergency department with coffee-ground emesis and altered mental status. He had no accompanying family to provide further history. He had
presented earlier in the day with viral symptoms and had been discharged
home. Vital signs on arrival were notable for bradycardia and hypothermia.
He was awake, but had no spontaneous movements, and was only intermittently withdrawing to pain. Initial glucose was < 20 mg/dL. Blood gas,
electrolytes, and blood counts were normal. Liver enzymes and coagulation
studies were significantly elevated. Serum iron and acetaminophen levels
were mildly elevated. Urine drug screen was negative. Abdominal films,
chest films, and head CT were normal.
The clinical presentation following ingestion is extremely broad. Occult
ingestion should be considered in any child who presents with unexplained
laboratory derangements and multi-system organ dysfunction. This patient’s
constellation of symptoms could not be explained by a single poison, and
presentation was thought to be the result of polypharmacy. Iron ingestion
could account for coffee-ground emesis, elevated serum iron level, and
transaminitis, though these signs/symptoms are typically seen in different
phases following ingestion. Acetaminophen ingestion could also account for
vomiting and transaminitis, though again, these are often seen at different time
points following ingestion. Multiple medications, including sulfonylureas and
beta-blockers, can cause hypoglycemia, which can in turn cause altered mental
status and hypothermia. Bradycardia can also be caused by numerous agents,
including beta-blockers.
The patient’s family arrived three hours after the patient arrived in our
emergency department. The mother was a poor historian, though denied having any medications, including over-the-counter medications, in the home.
He was treated with deferoxamine and N-acetylcysteine for presumed iron
and acetaminophen ingestions, and required a significant amount of dextrose-containing intravenous fluids to maintain euglycemia. He was admitted
to the intensive care unit for monitoring. Mental status, transaminitis,
coagulopathy, and hypoglycemia all improved within 24-48 hours.
247
CONGENITAL ABDOMINAL MASS IN A NEONATE
Huff LL, Kiger J, Caplan MJ. Medical University of South Carolina,
Charleston, SC.
Case Report: A female neonate was delivered at 35 weeks GA to a Hispanic
25 year old G1 mother via SVD. Maternal history was significant for HSV
with no lesions noted at the time of delivery. Maternal GBS status was
Southern Regional Meeting Abstracts
unknown. Maternal labs were otherwise negative. At 20 minutes of life,
the infant was noted to have respiratory distress. Further examination
revealed an asymmetric abdomen with a right-sided large, smooth, solid abdominal mass. The mass was palpable from the liver edge to pelvic rim and it
nearly crossed the midline.
The infant’s respiratory distress resolved by 24 hours of life. An abdominal X-ray revealed significant radiopacity of the right abdomen with displacement of bowel towards the midline. Abdominal ultrasound and CT
were also performed which revealed a heterogeneous mass arising from the
lower pole of the right kidney with associated grade 3 hydronephrosis of
the upper pole collecting system.
Radical right nephrectomy with partial ureterectomy was performed. On
gross pathologic examination of the right kidney, it weighed 117.8g, and was
6.5 x 5.8 x 5.5 cm. It had a whorled “leiomyomatoid” pattern on gross examination and well-defined fascicles of cytologically bland myofibroblastic cells
on microscopic examination.
The clinical presentation and pathological examination were consistent
with a congenital mesoblastic nephroma (CMN), classical type. Originally
thought to be a congenital form of Wilms tumor, congenital mesoblastic
nephroma is defined as a separate entity and is rare, albeit the most common
renal tumor in infants under the age of 3 months.
Surgical nephrectomy with wide margins is considered the treatment
of choice; however, chemotherapy should be considered in stage III tumors,
if resection is incomplete, or if the tumor ruptures during surgery. The tumor
may recur locally or metastasize 5-10% of the time, which usually occurs
within 12 months of the diagnosis. Prognosis is excellent with a 5 year survival around 96%.
248
FOAMY URINE AND SICKLED CELLS
Huntwork MP1, Howell MP2, May J2, Yospiv I2, Singleton T2. 1Tulane University School of Medicine, New Orleans, LA and 2Tulane University School
of Medicine, New Orleans, LA.
Case Report: A 19 year-old girl with known sickle cell disease presented
with a one day history of lower back and thigh pain. The patient recently
had two ER visits and one hospital admission for vaso-occlusive crisis and
pneumonia.
Symptoms were typical of her usual pain crisis. On review of systems,
the patient reported “foamy” urine and a swollen face. She felt dizzy when
standing up rapidly. Chart review revealed that she received ten doses of
ketorolac over the past month, at various ER visits, clinic visits, and from
her hospital admission.
Physical exam was notable for scleral icterus, tachycardia without murmur, and orthostatic hypotension. Laboratory studies were significant for
BUN of 43 mg/dL, creatinine of 3.6 mg/dL (baseline is 0.5 mg/dL), bicarbonate of 17 mmol/L, albumin of 1.3 g/dL, and total bilirubin of 3 mg/dL.
Hemoglobin was 5.7 mg/dL, and reticulocyte count was 10%. Urinalysis
revealed protein greater than 500 mg/dL. The urine protein to creatinine ratio
was 27.
A renal biopsy was performed to determine the cause of acute kidney injury (AKI) and acute-onset nephrotic syndrome. Light microscopy demonstrated focal segmental glomerulosclerosis (FSGS) and the absence of
eosinophils. Electron microscopy revealed diffuse podocyte effacement and
the absence of dense deposits. A diagnosis of nephrotic syndrome due to
FSGS and minimal change disease was established.
Sickle cell disease can cause a nephrotic picture entirely on its own. Increased renal blood flow results in hyperfiltration, increased GFR and lower
serum creatinine. Chronic glomerular hyperperfusion can lead to glomerular
injury or FSGS, and proteinuria. Sickle cell crisis is associated with a decrease in creatinine clearance with a return to baseline around four weeks after the episode, suggesting a transient glomerular dysfunction during acute
episodes.
Our patient also had recent documented NSAID use. In children,
NSAID-induced AKI often occurs in conjunction with other comorbid
conditions, like sickle cell disease. NSAIDs inhibit cyclooxygenase, which
impedes prostaglandin-mediated vasodilation of the afferent arteriole, causing decreased renal blood flow, decreased GFR, ischemic injury and acute tubular necrosis.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
481
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
249
AMBIGUOUS GENITALIA AND LARGE ABDOMINAL MASS
IN A NEONATE: A CASE REPORT
Jones M1, Voelker C3, Lutfallah C2. 1Our Lady of the Lake Regional Medical
Center, Baton Rouge, LA; 2Our Lady of the Lake Regional Medical Center,
Baton Rouge, LA and 3Woman’s Hospital, Baton Rouge, LA.
Case Report: Introduction: Ambiguous genitalia is an example of a disorder of sexual development, in which the external genitalia do not have the
typical appearance of either sex. The birth of a child with ambiguous genitalia is a challenging and distressing event for the family and physician and one
with life-long consequences. The most common cause of ambiguous genitalia is congenital adrenal hyperplasia. True hermaphrodites are extremely rare.
Case Presentation: A 22 year old G3P2002 with no significant past medical
history was referred to Maternal Fetal Medicine specialists for a suspicious
finding on prenatal ultrasound. A prenatal MRI was performed and showed
a large fetal abdominal mass and indeterminate genitalia. The infant was
born via spontaneous vaginal delivery at 39 weeks and 1 day with APGAR
scores of 8 and 8. The physical exam showed no abnormalities with the
exception of a firm, distended abdomen and ambiguous genitalia. Within
2 weeks of birth, the patient developed urosepsis and required placement
of a vaginostomy tube transabdominally and antibiotics.
Discussion: Extensive work up revealed a true female hermaphrodite with
XX chromosomes and both ovarian and testicular tissue. The patient’s large
abdominal mass was discovered to be a hydrocolpos.
Conclusion: Due to the unique formation of the patient’s anatomy regarding
the genital tract, a situation was created in which the patient had reflux of
bladder contents into a hydrocolpos. This process began in utero enlarging
the vaginal area which folded over itself preventing emptying of the hydrocolpos. This process lead to stagnant urine and an environment primed for
urosepsis.
Next Steps: The family has decided to raise the infant as a female. She will
require extensive corrective surgery to normalize the function and appearance of her genitalia. The male testicular tissue will be removed. The patient
will also be followed long term by endocrinology to manage hormone
regulation.
250
PROPTOSIS: AN EYE POPPING DIAGNOSIS
Kant S. University of Alabama Birmingham, Birmingham, AL.
Case Report: Case Report: A 12 yo M presented to our Emergency Department (ED) with chief complaint of right eye swelling and pain. His symptoms
started 10 days prior to presentation with right eye discharge, pain, diplopia
and fever to 101. The fevers lasted for 2-3 days then self-resolved. In addition
to eye swelling and pain he developed light sensitivity and pain with eye movement. Treatment prior to ED presentation included acyclovir, erythromycin
ophthalmic ointment and tobramycin/dexamethasone eye drops. On the day
of ED presentation he had worsening pain and his eye seemed to be bulging.
Physical exam was notable for an alert patient who appeared to be in pain. He
had erythema and swelling of the right upper eyelid, conjunctival injection
along with mild proptosis of the eye. Eye movement was restricted in all
directions. Pupil was round and reactive to light. Examination of the left eye
was normal. Visual acuity: right eye 20/40, left eye 20/20. Our initial concern
was for orbital cellulitis. Patient was given a dose of clindamycin while we
obtained a CT scan with contrast of the orbits. The CT scan showed thickening
of the right extra-ocular muscles, stranding within the post-septal and intracoronal fat, right scleral thickening and proptosis. The prime consideration
was pseudotumor of the right eye vs. orbital involvement by thyroid disease.
Ophthalmology was consulted who agreed with the diagnosis. Thyroid function studies including free T4, TSH and thyroglobulin antibody were found to
be normal. Patient was admitted to the general pediatric service and given IV
methyl-prednisone. After 24 hours of IV steroids, patient had improvement in
symptoms. He was discharged home on oral steroids for 1 month.
Discussion: Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome (IOIS) is a rare unusual inflammatory condition. IOIS is
likely an autoimmune-mediated entity. Factors implicated in causation include viral, genetics and environmental. Although more common in the fifth
482
decade of life, it can occur at any age and has been described in patients as
young as 2 years. Differential diagnosis includes orbital cellulitis, thyroid
ophthalmopathy, sarcoid, lymphoid tumor, lymphangioma, metastatic carcinoma and rheumatologic disorders. Diagnosis is made with the help of imaging (CT or MRI). Prompt response to steroids is a key feature of IOIS.
251
EXCRUCIATING PAINFUL GOITER SECONDARY
TO HASHIMOTOS THYROIDITIS REQUIRING
THYROIDECTOMY FOR PAIN CONTROL
Kashyap L1, Chalew S1, Simon L2, Gomez R1. 1LSUHSC, New Orleans, LA
and 2LSUHSC, New Orleans, LA.
Case Report: Hashimotos thyroiditis usually presents as painless thyroid
swelling. Painful Pediatric Hashimotos thyroiditis is a rare condition with
limited literature on pain management. We report a 15 yr old female who
presented with a month history of fatigue, malaise and progressive painful
midline thyroid swelling, no difficulty in swallowing, no fever or recent
URI symptoms. Exam was remarkable for diffusely visibly enlarged, very
tender, and non-nodular thyroid. Thyroid function tests, CRP, complete
blood count were normal. Ultrasound revealed diffusely enlarged nonnodular, non-cystic gland with mild increased vascularity. Diagnosis of
Hashimoto’s thyroiditis was confirmed by biopsy and thyroid antibodies.
Over 6 weeks period pain management with Ibuprofen, Levothyroxyne,
corticosteroid, Gabapentin and Amitryptilline was not successful. Finally
total thyroidectomy resulted in complete resolution of thyroid pain.
Conclusion: Thyroidectomy could be considered for the rare case of painful
Hashimotos thyroditis in children.
252
A CASE OF UNDIAGNOSED CYSTINOSIS AND
WAARDENBURG SYNDROME PRESENTING IN A CHILD
WITH CONGENITAL HEARING LOSS, VOMITING,
CONSTIPATION, AND FAILURE TO THRIVE
Langford S, Workman L, Multerer S, Kenagy D. University of Louisville,
Louisville, KY.
Case Report: We are reporting a 20 month old F with history of congenital
deafness who presented with complaint of several episodes of non-bloody,
non-bilious vomiting and severe constipation over the last several months.
She was noted to be below the 3rd percentile for weight and exhibited motor
delays. Serum electrolytes, a head CT and skeletal survey were performed
and the patient was admitted to the hospital for further work up of failure
to thrive.
On exam the child was small for age, unable to stand, social and smiling
yet nonverbal. She had light blonde hair with a central forelock of white hair,
pale skin, wide set eyes and wide nasal bridge, but an otherwise unremarkable physical exam. Family medical history included congenital deafness in
mother, maternal grandmother, and brother. Given these exam findings and
family history, the patient and her family were diagnosed clinically with
Waardenburg syndrome after genetics consultation.
Her initial labs were notable for hypernatremia, hyperchloremia,
hypophosphatemia, and an anion gap metabolic acidosis along with glycosuria and a pH of 7 on urinalysis. Repeat serum chemistry after fluid resuscitation revealed worsening metabolic acidosis—a finding not explained by
Waardenburg syndrome. Head CTwas normal and skeletal survey had findings
suggestive of rickets. Slit lamp exam showed crystalline deposition in the eye
and later leukocyte cystine assay was elevated to 3.92 (normal range 0.12 +/−
0.06). This confirmed the cause of the patient’s metabolic acidosis—Fanconi
syndrome due to nephropathic cystinosis.
This is an unusual case pairing two rare and unrelated genetic diagnoses,
cystinosis and Waardenburg syndrome. Cystinosis is a lysosomal storage disease that is the leading cause of Fanconi syndrome in children and Waardenburg
is an autosomal dominant disorder that accounts for 2 to 35% of cases of congenital hearing loss. Waardenburg syndrome and nephropathic cystinosis have
not previously been associated in the literature and each can present at different
times with chronic constipation, fair pigmentation, and hearing loss.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
253
A FATAL CASE OF ROCKY MOUNTAIN SPOTTED FEVER
LeJeune G, Vanchiere J, Bocchini J, Scott L. LSU Health Shreveport,
Shreveport, LA.
Case Report: A 5-year old female visiting east Texas from North Carolina
was transferred to the PICU at LSU Health Shreveport with suspected
RMSF. She had multiorgan failure and was transferred for evaluation of
ECMO. Upon arrival, patient GCS was 3 and she required multiple
vasopressors to maintain blood pressure. After aggressive resuscitation, the
patient was stabilized and determined not a candidate for ECMO. CRRT
and plasma exchange therapy were initiated. 2 weeks prior, a tick was found
on her shoulder. 5 days later she developed fever, sore throat and a
maculopapular rash. She was seen by her doctor a week following the tick
bite and started on cephalexin for Strep throat. A petechial rash developed
10 days after the tick bite and the her condition deteriorated during the drive
to Texas. She became lethargic and not taking liquids or food. At a local ER,
her platelets were 20,000 and a full sepsis workup was done. She was begun
on vancomycin, ceftriaxone and doxycycline and admitted to a community
hospital PICU. She continued to deteriorate and had cardiac arrest following
her admission. She was transferred to our facility due to worsening clinical
status. In addition to CRRT and plasma exchange, patient was continued
on broad spectrum antibiotics including doxycycline for coverage for
suspected RMSF. She had minimal response with no improvement in neurological status. A nuclear medicine study noted no effective cerebral perfusion. Brain death was confirmed and she was removed from mechanical
ventilation. She expired 11 days following onset of symptoms. Subsequently,
the PCR sent to CDC confirmed the diagnosis of RMSF. RMSF, caused by
Rickettsia rickettsii, a gram-negative, obligate intracellular bacterium, is
the most common rickettsial infection in the US. It can be characterized by
a wide spectrum of symptoms and typically is curable, but potentially can
be lethal. According to the CDC, case fatality rates have dropped from 2030% to 1.1-4.9% after antibiotic therapy was implemented as standard of
care. Early recognition and initiation of therapy are important factors for reducing risk of mortality. This case demonstrates the importance for Primary
Care doctors to recognize the signs and symptoms of RMSF and implement
prompt empiric therapy that could save the life of their patient.
255
NEURONAL CEROID-LIPOFUSCINOSIS PRESENTING
WITH REGRESSION OF MILESTONES, SEIZURES
AND ASPIRATION
254
LINEAR AND WHORLED HYPERMELANOSIS: AN
INTERESTING NEWBORN FINDING
Leysath A, Burns JJ, Lee A. Florida State University College of Medicine,
Pensacola, FL.
Case Report: A full term newborn was evaluated by the physician on the
first day of life. Physical exam noted a linear, horizontal, hyperpigmented
skin pattern on the newborn’s back. Per nursing staff, the pattern was present
and birth and had not changed. Exam was unremarkable otherwise. Family
history was negative for a similar dermatologic problem. Due to the cutaneous findings, a cranial ultrasound and an echocardiogram were obtained;
both were normal. A diagnosis was subsequently made and the mother
counseled accordingly. The infant was discharged on day of life 3 with routine follow up.
Linear and whorled nevoid hypermelanosis (LWNH) is a rare cutaneous
disorder characterized by hyperpigmented, streaky and whorled macules in
Blaschko’s lines. Blaschko’s skin line patterns may take the shape of a
“V”, “S” or perpendicular lines for regions involving the trunk, arms and
legs. Diagnosis is based on classically described “zebra striped” hyperpigmentation on physical exam. Differential diagnosis includes incontinentia
pigmenti, linear epidermal nevus and nevoid hypermelanosis. It can be distinguished on physical exam by absence of pustules or vesicles in the hyperpigmented areas. LWNH is usually an isolated finding, but can be associated
with intracranial and cardiac abnormalities. There is no specific treatment.
Dermatology referral may be made if the diagnosis is uncertain or for cosmetic concerns. Otherwise, individuals with isolated cutaneous findings experience no adverse affects.
Lowe GS, Dunlap M. University of Oklahoma Health Sciences Center,
Oklahoma City, OK.
Case Report: Neuronal Ceroid-Lipofuscinoses (NCLs) are a devastating
type of inherited neurodegenerative disorders primarily presenting with loss
of milestones, seizures & progressive visual, cognitive and motor deterioration resulting in premature death. We present a case with these symptoms
in addition to the presenting symptom of aspiration. Diagnosis was made
by Electron Microscopy of a conjunctival biopsy and aided by the discovery
of a biological sibling with similar neurodegenerative symptoms admitted to
our hospital at the same time.
A 5 year old female with a history of complex partial seizures and mild to
moderate global developmental delay presented to our hospital with
symptoms of aspiration, difficulty breathing and fever. She was slowly
achieving milestones until she began having seizures at age two. Her seizures
became increasingly difficult to control but by age 3 she was able to walk and
speak a few words. She had a complex social situation and was placed in foster care at age 3 due to medical neglect with an unknown family history. In
the three months preceding her admission, she had rapid regression with increased seizure activity causing falls with ambulation, speaking fewer words,
and incontinence. She became nonverbal and non-ambulatory one month
prior to admission and began having difficulty feeding. She was admitted
for aspiration pneumonia after she failed outpatient treatment and received
a gastrostomy tube and fundoplication to address her feeding issues; intractable seizures continued. Due to vision loss ophthalmologic evaluation was
performed which suggested Batten disease, conjunctival biopsy was recommended. The EM evaluation of this biopsy yielded large lysosomal inclusions within vascular pericytes containing curvilinear membranous profiles
characteristic of NCLs Classic Late Infantile variant.
NCLs are described in the genetic literature. This case report aims to
raise awareness in General Pediatrics of NCLs as important in the differential
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
483
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
diagnosis in a patient with regression of milestones, seizures and aspiration.
It also demonstrates the importance of family history in diagnosis and need
for genetic counseling for affected families.
256
FOUR MONTH OLD WITH VOMITING POST HEAD TRAUMA
Maldonado L, Takagishi J. University of South Florida, Tampa, FL.
Case Report: A four month old male fell off his grandmother’s bed, approximately three feet high, onto a tile floor. He cried immediately and there was
no loss of consciousness. He was evaluated in the Emergency Department,
cleared, and sent home to follow up in his pediatrician’s office the next day.
In the office, parents report a significant change from baseline since leaving the ED. He has had emesis with all feeds, decreased appetite, fussiness,
and decreased interaction with the family. Parents deny fever, diarrhea, shortness of breath or rash. The emesis was non-bloody and yellow to green in
color. Mother noted that she felt ill and that grandmother had been diagnosed
with “the flu.”
His physical exam revealed a temperature of 98 degrees Fahrenheit, pulse
140, respiratory rate 40 and a weight of 7.74 Kg, 250 grams less than the day
prior. He appeared sleepy and uncomfortable, fussy but consolable by father.
His skin was warm with no rash or lesions. His head was normocephalic and
atraumatic. The anterior fontanel was open and soft. Pupils were equal and
reactive to light. His lungs were clear to auscultation bilaterally with no increased work of breathing. His heart rate was regular and without murmurs.
His distal pulses were brisk. Abdomen was soft, mildly distended, not tender,
without masses, and with positive bowel sounds. His neurologic exam
showed decreased activity from baseline, fussy but intermittently consolable.
He was referred to the Emergency Department for further workup. A
head CT without contrast was within normal limits. During the collection
of blood, urine and CSF, the patient produced a small bloody stool. An abdominal ultrasound showed multiple distended loops of bowel, suspicious
for obstruction, and a mass in the right upper quadrant with concentric rings,
consistent with an intussusception. An air contrast enema was performed and
did not reduce the intussusception. He was then taken to the operating room
for an exploratory laparotomy for intussusception reduction and appendectomy. He was admitted to the PICU post operatively and after two days he
was discharged home with stooling, tolerating feeds, and an improved activity level. Follow up visits showed adequate intake, output, weight gain, and
neurologic examination.
257
THE MEDICAL MANAGEMENT OF CERVICAL
LYMPHADENITIS CAUSED BY ATYPICAL
MYCOBACTERIUM
Mathias MD, Shamoun MP, Durham M. Childrens Medical Center, Dallas, TX.
Case Report: PURPOSE: To discuss the medical versus surgical management of atypical mycobacterium cervical lymphadenitis
CASE REPORT: A two year old previously healthy boy presented with
seven weeks of right neck mass swelling. The mass was initially the size of
an egg and had continued to grow. The mass was mobile, nontender, and
nonerythematous. He had received five days of clindamycin, three days of
azithromycin, and one dose of amoxicillin-clavulanate. During this time,
the node had mildly decreased and then increased in size. Review of systems
revealed one day of fever at presentation and non-drenching night sweats. He
did have cat, dog, and cow exposure. He had no travel exposure nor incarcerated contacts. An ultrasound had shown a 4.5cm right submandibular lymph
node consistent with lymphadenitis. A MRI showed a large inflammatory
conglomeration of lymph nodes, some with necrotic areas, as well as, a large
abscess. He went for Incision and Drainage (I&D) of the abscess and was
started on trimethoprim-sulfamethoxazole for coverage of oxacillin sensitive
Staphylococcus aureus, methicillin resistant Staphylococcus aureus, and
Bartonella. Acid fast bacilli stains from I&D came back positive. Tuberculin
sensitivity test and Quantiferon gold were negative. Cultures returned positive for Mycobacterium avium-intracellulare complex. Due to the location
484
and size of the node and possible deformity or injury to Cranial Nerve XI,
Otolaryngology elected not to excise the node. The patient was started on
long-term therapy with rifampin and azithromycin
DISCUSSION: While excision of node is the classic treatment of atypical
mycobacterium, there are cases where the anatomical challenges of excision
make medical therapy a reasonable alternative. (1) However, as morbidity
(repeat operations and sinus tract formation) is shown to increase without
complete excision, close follow up is warranted. (2)
Hazra R, et al. Lymphadenitis due to nontuberculous mycobacteria in
children: presentation and response to therapy. Clin Infection Dis. 1999
Jan;28(1): 123–9.
Scott CA, et al. Management of lymphadenitis due to non-tuberculosis
mycobacterial infection in children. Pediatric Surg Int. 2012 May;28(5):
461–466.
258
NEONATAL SCABIES WITH THROMBOCYTOPENIA
MASQUERADING AS MUCOCUTANEOUS HERPES
SIMPLEX INFECTION
Melson S, DeLeon S, Yates AM. University of Oklahoma Health Sciences
Center, Oklahoma City, OK.
Case Report: Rashes are a common complaint among neonates and the differential is broad. Moreover, many of the skin lesions that are common
among neonates can appear as vesiculopustular lesions and the differential
diagnosis for these lesions alone varies from benign conditions without
long-term sequelae to infections with high rates of morbidity and mortality.
Our patient is a previously healthy 25 day-old male who presented to a
tertiary children’s hospital with a diffuse rash characterized by vesicles over
an erythematous base. His physical exam was strongly suspicious for herpes
simplex virus (HSV), a diagnosis supported by multiple dendritic lesions on
ophthalmologic exam. Laboratory studies including spinal fluid HSV PCR
and skin scrapings were sent and the infant was admitted on IV acyclovir,
along with erythromycin ophthalmologic ointment and polymixin/TMPSMX eye drops. After 48 hours, HSV culture/PCR were reported as negative
and at 72 hours, the rash had evolved from vesicular lesions into crusted
lesions with minimal erythema. At the same time he also developed marked
thrombocytopenia, raising concern for other congenital TORCH infections.
An Infectious Disease consultant had suspicion for neonatal scabies. Subsequent evaluation by dermatology with skin scrapings confirmed a diagnosis
of neonatal scabies. He was treated with permethrin cream and showed rapid
improvement in both his rash and thrombocytopenia. Repeat ophthalmologic
evaluation resulted in a revised diagnosis of corneal abrasion, likely secondary to pruritis. He was discharged home in good condition.
Neonatal scabies is a rare diagnosis that can be treated with relative ease.
The common lesions are eczematous, crusted vesicles, however, our patient
illustrates a case of scabies with grouped vesicles over an erythematous base,
the lesion classically associated with HSV. Additionally, he also suffered
from thrombocytopenia, which has previously not been reported in the setting of scabies. This case exemplifies the diagnostic dilemma faced by pediatric providers when faced with neonatal rashes and underscores the
importance of maintaining a broad differential, particularly when confronted
by vesiculopustular lesions.
259
AN INFANT WITH DEVELOPMENTAL DELAY AND
SIGNIFICANT HEART MURMUR
Mike S. LSU Health Shreveport, Shreveport, LA.
Case Report: Children with developmental delay are frequently seen in general pediatric practice. Early identification of delay is essential, especially if
delay is the result of an organic cause that affects multiple organ systems.
The diagnosis becomes particularly challenging when the child does not exhibit the classically described characteristics of a known syndrome. A high
index of suspicion becomes paramount to identify the correct diagnosis
and initiate the appropriate health maintenance and surveillance protocols.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
The patient is a two year old African American male who presented at six
months of age for evaluation of a significant cardiac murmur. Echocardiogram revealed moderate pulmonic valve stenosis with otherwise normal cardiac function. At nine months of age the patient had not begun babbling,
sitting or crawling. At fifteen months of age the patient was not crawling,
pulling up, or walking.
On physical exam the patient had a somewhat dysmorphic appearing face,
however there were no characteristic syndromic findings to suggest the cause
for his developmental delay. There was no family history of mental retardation
or congenital syndromes. A chromosomal microarray showed a microdeletion
on chromosome 7 and the diagnosis of Williams syndrome was made.
Williams syndrome affects 1: 7500-10,000 births. Children with Williams
syndrome often have cardiovascular anomalies, short stature, characteristic facies,
and developmental delay or mental retardation. Because multiple organ systems
are affected by the disorder, children with Williams syndrome require specially
tailored health supervision. This case explores the identification of an important
genetic syndrome in a child who did not fit the classically accepted phenotype.
260
HYPOPLASTIC LEFT HEART SYNDROME IN A PATIENT
WITH FETAL HYDANTOIN SYNDROME
Mumphrey CG, Barkemeyer B, Zambrano RM. LSU Health Sciences Center,
New Orleans, LA.
Case Report: The association between antiepileptic drugs and congenital
malformations in the offspring of women with epilepsy was first discussed
in the late 1960s. Women taking antiepileptic medications carry a two- to
sevenfold higher risk of congenital malformations than the general population. This risk is proportional to the increase in number of pharmaceutical
agents used. In 1975, Hanson and Smith proposed the name “fetal hydantoin
syndrome” after identifying a special clusters of anomalies which included
craniofacial features, microcephaly, intellectual disability and hypoplasia of
the distal phalanges in the offspring of the women taking phenytoin. We
present a newborn male with maternal exposure to both phenytoin and
lamotrigine during the whole pregnancy, and born with several classic
features of fetal hydantoin syndrome and also with hypoplastic left heart.
Congenital heart disease is seen less frequently with fetal hydantoin syndrome. The most common defects include: pulmonary or aortic valvular stenosis, coarctation of the aorta, patent ductus arteriosus and septal defects;
most commonly ventricular septal defects. To our knowledge this would be
the first report of a patient with hypoplastic left heart. Unfortunately after
post operative complications support was withdrawn at DOL#32 and the patient died. Interestingly, this infant had a twin sister who also exhibited the
classic fetal hydantoin phenotype but without congenital heart disease,
suggesting that individual genetic variation plays a role in the variability of
the phenotype. Even though there has been a growing body of evidence
supporting a genetic etiology for left-sided heart defects, including HLHS;
not all genes involved have been identified. In addition, the teratogenic
mechanisms involved in phenytoin exposure also remain unclear, and are
likely to be complex. The association between fetal hydantoin syndrome
and hypoplastic left heart in our patient may offer insight to their etiologies
and any possible gene(s) that may be associated. Even though lamotrigine
is considered one of the safest antiepileptic drugs that can be given during
pregnancy, we cannot rule out that polytherapy may have played a role in
our reported novel association of hypoplastic left heart syndrome with fetal
hydantoin syndrome.
Southern Regional Meeting Abstracts
only of a rice-based, non-dairy beverage, which had been his sole source
of nutrition since three months of age. Physical exam revealed weight,
height, and head circumference of <3%, <3%, and 10-25% respectively.
Other pertinent exam findings include a pale, irritable, afebrile infant with
thin, lightly colored hair. Pitting edema was evident in all extremities with a diffuse sandpaper rash. An erythematous, maculopapular rash was present periorally
with some honey-colored crusting. A brightly erythematous, sharply-demarcated
diaper rash was present with a single vesicle noted on the right proximal thigh. No
involvement of mucous membranes noted. His physical exam paired with his dietary history raised concern for nutritional deficiency. Laboratory results were significant for an albumin of 1.6, prealbumin of 5.5 (range: 11-23), zinc level of
22 (range: 60-120), and a 25-OH vitamin D level of 26. After admission to the
hospital, the patient was started on zinc supplements and was transitioned to an
appropriate formula. He tolerated the formula well, and prior to discharge the rash
and edema had mostly resolved.
A rice-based, non-dairy beverage is an inadequate sole source of nutrition
for a growing infant. After consumption of the rice-based, non-dairy beverage for over seven months, our patient presented with several findings consistent with zinc deficiency, including dermatitis and failure to thrive. In
addition to zinc deficiency, this inappropriate nutrition source led to a protein
deficiency and a Kwashiorkor-like presentation. This case reinforces the importance of obtaining a thorough dietary history in infants, as well as the importance of parent counseling.
262
A LACTING ADOLESCENT ON ATYPICAL
ANTIPSYCHOTICS: A CASE REPORT OF AN
EMERGING CHALLENGE
Myers WT. University of Louisville, Louisville, KY.
Case Report: The use of atypical antipsychotics for behavioral control in the
pediatric population has been increasing rapidly. The advantages of these
medications are thought to outweigh the risks due to a marked decrease in
the extrapyrimidal effects seen with use of typical antipsychotics. With this
increased pediatric patient exposure, increased scrutiny of the incidence
and severity of adverse reactions in children must also occur. We describe
a 17 year old female who presented to the Emergency Department with the
complaint of a 3-4 day history of milky discharge from both breasts associated with some vaginal spotting and a reported increase in breast size over
the course of two months. The patient had a complex history; including,
PTSD, Mood Disorder NOS, and eosinophilic esophagitis. She had been taking ziprasidone until shortly before admission due to experiencing unacceptable weight gain when placed on olanzipine. Her other medications included
sertraline, lamotrigine, and lansoprazole. Physical exam demonstrated an
overweight adolescent with a BMI of 28.56 and milky discharge consistent
with lactation. Otherwise, her complete physical exam, including visual
fields and neurologic exam, was within normal limits. Laboratory analysis
showed an elevated prolactin level of 30.2 ng/mL (normal 3.0-18.6 ng/mL)
and negative urine and serum pregnancy tests. She was admitted, her
ziprasidone was held, and over the course of two days had a head MRI w/
contrast performed, coupled with serum cortisol and thyroid studies; all were
within normal limits. Her lactation ceased by day 2 of hospitalization and at
discharge her prolactin levels had decreased to 13.4 ng/mL. This case clearly
demonstrates a classic case of drug-induced hyperprolactinemia. The patient
was at risk for this condition primarily due to her exposure to atypical
antipsychotics, but also due to the co-administration of an SSRI. A review of
the literature associated with drug induced hyperprolactinemia demonstrates
the short term and long term effects of this condition, along with potentially resource-saving information on clinical management.
261
ZINC DEFICIENCY IN AN INFANT DRINKING RICE MILK
Murphy M, Carter-King L, Eggie D, Beatty K. LSUHSC, New Orleans, LA.
Case Report: An eleven-month old male presented with a progressively
worsening rash over the past three months as well as a six week-history of
diffuse, worsening edema. The patient also had a two-day history of fever,
decreased activity level, and cold symptoms. Detailed history revealed numerous food allergies including milk, eggs, and soy as well as multiple formula changes since birth secondary to diarrhea and vomiting. Diet consists
263
A PATIENT WITH MICROCEPHALY AND SIMPLIFIED GYRI
PATTERN- A PHENOTYPICAL VARIANT OF THE 15q24
MICRODUPLICATION SYNDROME
Patel B. University of South Alabama, Mobile, AL.
Case Report: Abstract: Duplications in the 15q24 region have been
reported to be associated with neurodevelopmental disorders, however, to
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
485
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
date, no reports of microcephaly with simplified gyri or brain malformations
have been reported. Reports of 15q24 microduplication as a result of recombinant homolog of the 15q24 recurrent microdeletion syndrome have been
characterized as developmental delay, microcephaly, loose connective tissues,
downslanting palpebral fissures, hypertelorism, broad nasal base, broad medial
eyebrows, long smooth philtrum and full lower lip. We encountered a case of a
young girl with dysmorphic features including narrow bifrontal diameter and
sloping forehead who presented with developmental delay and postnatal overgrowth and microcephaly. A high resolution comparative genomic hybridization (figure3) revealed a heterozygous duplication of chromosome 15 q24 of
1.26Mb. Patient had a brain MRI done which showed Group1 microcephaly
with simplified appearance of the gyri and shallow sulci with too few gyri.
Our case is a novelty presentation of microcephaly and simplified gyri pattern
linked to microduplication of chromosome 15q 24.
264
RECURRENT PANCREATITIS WITH CONSERVATIVE
MANAGEMENT OF A LARGE PSEUDOCYST
Patel B, Ponnambalam A. University of South Alabama, Mobile, AL.
Case Report: Drainage of a pseudocyst is indicated if symptomatic and for
progressive enlargement. The multitude of treatments and therapies available
make it a challenging choice for many physicians.
Here we discuss the case of a 9-year old female who presented with severe epigastric pain and vomiting. Review of laboratory values showed elevated amylase and lipase and a mutation in SPHINK-1-gene, rest of the tests
were normal. She was managed conservately and discharged home. Patient
was non-compliant with discharge recommendations and had multiple outpatient visits for continued epigastric pain. She had two more hospitalizations.
On the first of the two subsequent hospitalizations, abdominal imaging showed
a pseudocyst at the head of the pancreas, which initially was 4cm in size. This was
treated conservatively with nasojejunal feeds, low fat diet with pancrealipase and
esomeprazole. Monthly ultrasounds were done on an outpatient basis to evaluate
progression of pseudocyst and for any other complications. Over next three
months, though the symptoms improved, the pseudocyst increased to 6.3 cm
(See image). The decision was made to continue with conservative management
of this large pseudocyst. She responded well to this management and her
symptoms resolved. Amylase and lipase returned to normal limits. The
pseudocyst resolved with pancreas returning to its normal size.
This case illustrates that conservative medical management can be appropriate in a large pancreatic pseudocyst. Recognition of this benefit is important as it may in the future help with clinical decision making to avoid
invasive procedures with complications and risks of their own.
486
265
INFANT WITH MRSA NECROTIZING FASCIITIS
Patel P, Yu J, Bhatia J. Children’s Hospital of Georgia, Augusta, GA.
Case Report: Case Presentation:
Infant is a 7 day old 30 week, twin B, transferred from another hospital for
worsening erythema and swelling of the right hand and arm for 3 days due to
phlebitis from a PICC line. Infant had RDS initially for which he required surfactant and subsequent CPAP. A PICC line was placed on day of life 3 in the right
arm. On DOL 6, Infant became febrile and swellig and erythma of the PICC insertion site noted which progressed upto hand in a day. PICC line was removed and a
new PICC line was inserted in the left arm. Blood culture was obtained and the
infant started on Van & Gent as Infant was febrile (CBC benign, CRP- 4.2). On
DOL 7, the right hand, arm became more erythematous and swollen which coincided with a positive blood culture for gram positive cocci, Clindamycin added. A
Doppler showed an abscess in the right mid-arm. A Orthopedist consulted and
abcess incised, drained. MRI right upper extremity concerning for necrotizing
fasciitis. Infant then transferred to our institution for further management. Physical examination at our institution was notable for tense swelling proceeding
from the right hand to the ipsilateral hemithorax with significant erythema
and discoloration of the right forearm. Perfusion of the right hand was
poor with a prolong capillary refill time and absent pulses. Diagnosis of
compartment syndrome made, Infant underwent fasciotomy. Noted to have
non-viable subcutaneous tissue, large amount of cloudy fluid and pale
muscles intra-operatively. Antibiotics were continued and serial blood cultures
obtained. MRSA(Methicillin Resistant Staphylococcus Aureus) was persistently isolated from blood cultures for 3 days necessisating the addition of
Rifampin. Infant required vigourous supportive measures. Received Vancomycin for total of 3 weeks from the first negative blood culture. At the end of
therapy, fasciotomy wounds healed well and infant moved fingers and arms
spontaneously. This case highlights the invasive nature of this condition, the
need for prompt recognition and intervention.
266
UNILATERAL TONSILLAR ENLARGEMENT: A CASE OF
SPORADIC BURKITT’S LYMPHOMA
Prudhomme A1, Raulji C2, Morrison C2, Stark M3. 1LSU-HSC Children’s
Hospital, New Orleans, LA; 2LSU-HSC Children’s Hospital, New Orleans,
LA and 3LSU-HSC Children’s Hospital, New Orleans, LA.
Case Report: Burkitt’s Lymphoma (BL) is a rare form of high-grade nonHodgkin’s lymphoma of B-cell origin. There are three clinical subtypes: Endemic, Sporadic and Immunodeficiency-related.
The endemic subtype is seen in equatorial Africa, is associated with
Epstein-Barr Virus (EBV) and involves facial bones. Immunodeficiencyassociated BL occurs in immunocompromised patients such as those with
HIV, congenital immunodeficiency or allograft recipients.
Sporadic BL occurs worldwide, accounting for up to 40% of lymphoma
in children in the U.S. and Western Europe. It usually involves the GI tract,
but may also involve the head and neck, ovary, breast, bone marrow or CNS.
We describe a case of BL in a 6-year-old Caucasian male presenting with
unilateral tonsillar enlargement. He presented at his pediatrician’s office with
sore throat. The right tonsil was enlarged with a small exudative lesion. A
Rapid Strep test was positive. He was treated with antibiotics for 3-5 weeks
for presumed tonsillitis. The tonsil enlarged further and developed a large
white plaque. There was no fever, night sweats, or weight loss. He was referred for tonsillectomy. Morphologic review revealed reactive follicular hyperplasia and architectural effacement by a diffuse monomorphic infiltrate of
lymphoma cells with a starry sky appearance. The left tonsil revealed reactive follicular hyperplasia. A diagnosis of stage-2 Burkitt’s Lymphoma, CMYC positive, with an 8:14 chromosomal translocation was made. He was
referred to a Hematologist/Oncologist for staging and treatment.
Sporadic non-African BL accounts for less than 1% of head and neck
malignancies with an incidence of 2-3 cases per million per year in the U.S.
and Europe. BL is one of the most rapidly-growing tumors. BL of the tonsils,
although rare, presents with unilateral painless tonsillar enlargement and throat
discomfort. Because of the aggressive nature of BL and its significant response
to chemotherapy, timely diagnosis is crucial for appropriate treatment and better
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
outcomes. The possibility of malignancy should remain in the differential for
patients with these presenting signs and symptoms.
Southern Regional Meeting Abstracts
GNR was critical to this patient’s recovery. Furthermore, the rapid initiation
of ECMO was also key to this patient’s stabilization.
269
267
SUCCESSFUL CARDIOPULMONARY BYPASS WITHOUT
EXCHANGE TRANSFUSION IN SICKLE CELL DISEASE
Raney L1, Gardner R1,2. 1LSUHSC, New Orleans, LA and 2LSUHSC, New
Orleans, LA.
Case Report: Cardiopulmonary bypass presents risks for patients with sickle
cell anemia (SSD) since external oxygenation of blood and intraoperative hypothermia increase the incidence of sickling and associated vaso-occlusive crisis.
Currently, reduction of these risks involves exchange transfusion pre or
intraoperatively in an effort to reduce hemoglobin S, with a goal HbS ranging
from <5 to 30%. We present a case of successful cardiopulmonary bypass,
without complication, in a patient with SSD who did not undergo exchange
transfusion preoperatively. K.A. is a 14 yo F with SSD and a history of frequent
pain crises requiring hydroxyurea treatment. She was found to have an aortic
valve tumor on a routine echocardiogram. In preparation for surgery, a CBC,
and hemoglobin electrophoresis were drawn. Her Hb was 9.5, Hct 29, and her
HbS 17%. Of note, she recently had a simple transfusion during a pain crisis.
During surgery, she received standard general anesthesia, was placed on bypass,
and was cooled to 33°C. The tumor was excised, she was rewarmed, and then
weaned from bypass. Acid-base status, core body temperature, oxygenation, and
hydration status were all closely monitored. The operation was completed without
complication, and without exchange transfusion. She had an uncomplicated post
op course where she was extubated on POD #2, chest tubes removed on POD
#3, and pain was managed on her home regimen of oral MS contin and Tylenol.
The tumor was incidentally found to be an aortic valve papillary fibroelastoma.
This case demonstrates not only the risks but the precautions necessary for cardiothoracic surgery in patients with SSD. It also shows that once given proper supervision and assiduous intraoperative care, SSD patients can safely undergo
cardiopulmonary bypass and thermal cooling without vasoocclusion.
CONGENITAL CERVICAL PILOCYTIC ASTROCYTOMA
PRESENTING AS HYPOTONIA IN A NEONATE
Shakir AK, Holcomb JW. Georgia Regents University, Augusta, GA.
Case Report: Astrocytomas are the second most common CNS tumors in the
newborn, often presenting as hydrocephalus or macrocephaly. We are reporting
a case of cervical pilocytic astrocytoma that presented as hypotonia.
A 35-week gestation infant born by C-section, to a G2P0 mother, was hypotonic since birth, with presumed bilateral plexus palsy. On admission neurological exam, he had generalized hypotonia that was worse in upper
extremities (UE), spontaneous flexion of right UE and lower extremities,
but no spontaneous movements of left UE, spontaneous closing of fists,
but no palmar grasp, 3 beats of ankle clonus, DTRs could not be elicited.
Pupils were equal and reactive, root and suck reflexes intact and Moro weak
and asymmetric. Sepsis evaluation, liver and thyroid function, ammonia,
CPK were normal. MRI of the brain and cervical spine (DOL 3) was suspicious for a neoplastic cystic lesion of the cervical cord. MRI with contrast
showed a 5/3/16 mm intramedullary lesion at C3-C7 consistent with low
grade astrocytoma or ganglioglioma. The infant underwent excisional biopsy
and debulking on DOL 8. Pathology revealed pilocytic astrocytoma WHO
grade I. The infant recovered well with improvement in tone. He has limited
left wrist flexion on DOL 30.
We report an unusual type, location, and presentation of a congenital cervical pilocytic astrocytoma that presented with generalized hypotonia immediately after birth.
268
CHROMOBACTERIUM VIOLACEUM SEPSIS: COMPONENTS
IN TREATMENT
Richard KR1, Lovvorn J2. 1UAB, Birmingham, AL and 2UAB, Birmingham, AL.
Case Report: 11 yo male with progressive buttock abscesses. He was initially given PO Clindamycin for presumed Staphylococcal infection. I&D
later revealed gram-negative rods (GNRs) and the patient remained hospitalized and started on IV Ceftriaxone and Clindamycin. 24 hours after I&D, the
patient’s clinical status declined and he was transferred to our tertiary care
center. Upon arrival, the patient required intubation for impending respiratory failure and progressive shock. Vancomycin and Nafcillin were then
added. Rapidly, the patient declined, in spite of maximal conventional ventilator settings, aggressive fluid resuscitation, large-volume transfusions and
multiple vasoactive agents. Antibiotics were again expanded to include
Meropenem and Gentamicin. The patient continued to have progressive
multiorgan dysfunction, severe metabolic acidosis, ultimately requiring initiation of Veno-Venous (VV) ECMO, and later transitioned to Venoarterial
(VA) ECMO. The preliminary results of the patient’s I&D cultures grew
“brightly violet-colored gram-negative rods, consistent with Chromobacterium
violaceum.” Antibiotic coverage was broadened to include Ciprofloxacin and
Bactrim. The patient was without multi-agent GNR coverage for a minimum
of 96 hours from the time the initial buttock lesions were first evaluated. Until
C. violaceum was finalized, Vancomycin and Nafcillin were continued. Over
a five-week PICU stay, 14-day course of ECMO, and ongoing multi-antibiotic
therapy, the patient significantly improved. He was extubated on hospital day
19 and antimicrobials were further narrowed to Ciprofloxacin and Meropenem
on which the patient still remains.
Broad-spectrum coverage with Clindamycin, Vancomycin, and Nafcillin
in a patient with presumed gram-positive sepsis secondary to abscesses was
not adequate to provide clinical improvement in this patient. As the patient
worsened, antibiotics were than expanded to maximize gram-negative coverage. When preliminary cultures revealed chromobacterium violaceum, based
on case reports, Ciprofloxacin and later Bactrim, were added. Treatment of
MRI with contrast T1 sagital.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
487
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
270
272
PANCYTOPENIA AND GELATINOUS BONE
MARROW TRANSFORMATION IN ANOREXIA NERVOSA:
A CASE REPORT
273
Shankar A1, Benjamins L2, Beyda RM2. 1University of Texas Medical School
at Houston, Houston, TX and 2University of Texas Medical School at Houston,
Houston, TX.
Case Report: Anorexia nervosa is a psychiatric, eating disorder associated
with various hematologic changes such as anemia, leukopenia, and less commonly neutropenia and thrombocytopenia. Half the patients with anorexia
nervosa also have bone marrow atrophy. In rare and very severe cases, severe
neutropenia and gelatinous bone marrow transformation (GMT) can be seen.
In this report, we present a 17 year old Asian female with anorexia
nervosa who presented with fever, streptococcal pneumonia and respiratory
distress. She was found to have severe neutropenia and pancytopenia that
did not improve despite daily transfusions of red blood cells, cryoprecipitate,
fresh frozen plasma, and platelets. Peripheral blood smear showed normocytic,
hypochromic anemia with anisocytosis, few cells, and slight polychromasia. A
bone marrow biopsy was done and showed marked hypoplasia (cellularity
<5%) with diffuse gelatinous transformation. These findings were due to deficiency in carbohydrates, calories and depletion of the bone marrow fat stores.
Cases of anorexia nervosa with pancytopenia, severe neutropenia and
GMT are rare. These symptoms may be reversed with rapid adequate nutrition with close monitoring for refeeding syndrome. In addition, there are
reports that use of hematopoietic growth factors may also be used to accelerate bone marrow recovery. However once the patient is medically stable,
treatment of the underlying condition of anorexia nervosa is essential to prevent relapses.
271
RARE OVARIAN TUMOR MASQUERADING
AS HEMATOMETRA
Sharma A, Northrip K. University of Kentucky, Lexington, KY.
Case Report: An 11 years old African American female presented to the
afterhours clinic with the chief complaint of a vague deep intermittent lower
abdominal pain since 2-3 months without any radiation. It was associated
with worsening generalized abdominal distension, a 40lbs unintentional
weight gain and intermittent diarrhea alternating with constipation over the
previous 3 months. Her past medical history was noncontributory. She denied being sexually active or having reached menarche.
On examination, her abdomen was soft but distended without hepatosplenomegaly. A globular mass was felt rising from the pelvis. No focal tenderness or hernias were noted. Fluid thrill was appreciated. On digital rectal
examination, no hard stool, palpable masses or blood were appreciated. On genital examination, she had normal-appearing external genitalia except for a slight
bulge of the introitus. She was noted to have stage IV pubic hair and breast development. An abdominal radiograph revealed mild fecal burden without evidence of obstruction. The possibility of an occult malignancy was suspected
but in view of her young age and absence of any B symptoms, it was considered
prudent to avoid imaging radiation exposure prior to specialty consult. A presumptive diagnosis of hematometra was made based on the constellation of
signs and symptoms and she was referred to OBGYN for further management.
Later, a CT scan of abdomen and pelvis revealed a 14x11x10cm cystic
mass arising from the right ovary with internal septations, along with a massive ascites. She underwent laparotomy with right ovarian cystectomy, right
oophorectomy and partial omentectomy. Histopathology of the ovarian mass
revealed a juvenile granulosa cell tumor (JGCT) of the ovary (Stage IB).
Ovarian granulosa cell tumors (GCT) are extremely rare neoplasms arising
from the sex-cord stromal cells of the ovary, representing less than 5% of all
malignant ovarian tumors. JGCT represents only 5% of all GCTs and the
mean age at diagnosis is 13–17 years. Most GCTs produce estradiol leading
to isosexual precocious puberty. This might explain the advanced secondary sexual characters in our patient. Complete surgical resection, if achievable, is usually
curative. This case highlights the importance of considering how to structure a
plan so as not to miss a rare diagnosis while pursuing more common ones.
488
TREATMENT OF PEDIATRIC CHOLINERGIC REACTION
IN RURAL KENYA
Shirk AM. University of Alabama -Birmingham, Birmingham, AL.
Case Report: 15 month old male presented to rural hospital in Kijabe,
Kenya with fever, seizures, and agitation. Patient was full term infant, IUTD,
with motor development delay but no other significant PMH. Morning of
presentation mom found him on the floor under his crib, crying and combative. He had episode of “jerking” and one episode of NBNB emesis. ROS
positive for rhinorrhea, drooling, and decreased activity the previous day.
He had no known trauma or new food or herbal medicine exposure. In the
ER, patient had temp of 101.2, HR of 220, RR of 15 with subcostal
retractions and saturation of 74% on RA. Patient was agitated with intermittent 30 second seizures with left eye deviation. Pupils were 2mm, slowly reactive and patient was drooling. Auscultation revealed tachycardia with no
murmur, scattered crackles, and wheezing. Extremities were hypotonic, cool
and clammy with 1+ reflexes. Electrolytes showed Na 137, K 4.7, Ca of
9.8, and Cr of 1.3. CBC WNL with WBC 12,000. CSF had 2 WBC, 0
RBC and normal protein/glucose. Chest x-ray showed no infiltrate. Head
CT with mild brain atrophy but no traumatic findings. Patient was given
Ceftriaxone, 50/kg NS over 6 hours, and loaded with phenobarbital. Over the
next 72 hours patient developed diarrhea, bilateral infiltrates on chest x-ray, copious secretions, and required intubation. On day 4 of illness, despite no clear
history of cholinergic exposure, with seizures, vomiting, bronchorrhea, diarrhea, miosis, and diaphoresis patient was empirically given Atropine 0.04 mg/kg
with immediate improvement. Within 4 hours, PEEP and O2 were weaned,
and he was extubated within 24 hours. With q2 hour Atropine (drip was unavailable), fever, secretions, wheezing resolved, and reflexes improved. Patient was
slowly weaned to q6 hour atropine, and was discharged 2 weeks after presentation. Discussion: Cholinergic poisonings without a source are a rare, but important clinical dilemma. Cooking containers and specific endemic herbs must be
considered as possible sources when the toxidrome emerges. Mom used herbal
medicines with previous children, which is likely source despite multiple
denials in history. In resource limited settings, atropine can be used to effectively treat symptoms with careful observation and frequent dosing.
274
INTRACARDIAC MASSES AS INITIAL FINDING IN A CHILD
WITH ACUTE MYELOGENOUS LEUKEMIA
Singh M1, Cain J2, Ramji F3, Kern W4. 1University of Oklahoma, Oklahoma City,
OK; 2University of Oklahoma, Oklahoma City, OK; 3University of Oklahoma,
Oklahoma City, OK and 4University of Oklahoma, Oklahoma City, OK.
Case Report: We describe an 18 month old boy who presented with 4 day
history of increased work of breathing, decreased oral intake, and oliguria.
A chest radiograph showed cardiomegaly, which prompted an emergent
echocardiogram. The echocardiogram revealed a moderate pericardial effusion with infiltrative cardiomyopathy. Tumor masses were seen in all 4
chambers with consequent decrease in ventricular filling. Cells in the pericardial fluid were identified as myeloblasts by flow cytometry. A renal ultrasound showed bilateral hydronephrosis, caused by infiltrative masses in the
collecting systems of both kidneys. A bone marrow aspiration and lumbar
puncture were planned for the next morning but rapid decompensation, with
developing multi-organ failure, led to emergent use of extracorporeal membrane oxygenation (ECMO).
Literature review shows this to be an unusual site, especially in children.
While outcome in similar reports was poor, our patient remains a long-term
survivor after a challenging clinical course. Data on myeloid sarcoma was
reported during later incidental reviews and is incomplete. Although myeloid sarcoma was once thought to be rare, it appears that a significant minority of pediatric AML cases present with mass lesions. Depending on
anatomical site, such lesions may add to morbidity and early mortality.
Our patient’s presentation in cardiogenic shock, rapidly progressing to
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
multiorgan failure, use of pericardial fluid for diagnosis and the crucial role
played by imaging is unusual. Aggressive support was started with ECMO
and dialysis. Chemotherapy was also delivered but was attenuated due to
hepatic compromise. In spite of this inauspicious situation, this patient is
a long-term survivor-perhaps the only one reported in the pediatric literature with intra-cardiac myeloid sarcoma. The case is worth reviewing because of its unusual features. It also demonstrates the importance of
pediatric subspecialty team work for children with cancer presenting with
life threatening symptoms.
275
Southern Regional Meeting Abstracts
Radiographs showed generalized osteopenia with near-complete loss of carpal
bones and osteolysis of the distal ulna and left femoral head.
The severity of osteolysis in the absence of erosions or signs of inflammation and similar disease in a sibling suggested a genetic etiology. Additionally, the parents were reportedly second cousins. Based on the clinical
and radiographic findings, the patient was diagnosed with Winchester syndrome,
an autosomal recessive disorder characterized by multicentric osteolysis, leathery
skin, corneal opacities, coarse facies and renal anomalies. Our patient had coarse
facies and renal utrasonography was normal. The underlying genetic defect
has recently been identified in the matrix metalloproteinase-2 (MMP-2)
gene. Our patient highlights the importance of considering alternative diagnoses besides JIA in patients with an atypical presentation, poor treatment
response and familial disease.
MY BELLY HURTS…AGAIN
Smith D, Mitchell H, Monroe K. UAB, Birmingham, AL.
Case Report: Introduction: Abdominal pain is one of the most common
complaints in the pediatric ED. Many times this can be a result of a simple
viral infection, constipation or functional abdominal pain. We must be aware
though, that cases of abdominal pain that stand apart in their symptom quality or duration, require further workup and expansion of our differential. Hereditary pancreatitis is rare, but has significant sequelae, and should be
suspected in children with recurrent epigastric pain associated with nausea
and vomiting.
Case: The patient is a 7 year old male who presented to the ED with intermittent abdominal pain for 6 months. He has been evaluated by a pediatric
GI doctor, but no etiology had yet been discovered. At the time of presentation in our ED, the patient had 3 weeks of worsening epigastric pain, and one
day of rapid breathing. A chest x-ray in the ED showed a large right pleural
effusion. A CT of the chest, abdomen and pelvis subsequently revealed bilateral pleural effusions, pancreatic pseudocysts with calcifications and portalsplenic vein thrombosis with collaterals. Initial lab work revealed a WBC
count of 18 thous/uL and a lipase of 1553 U/L. The clinical picture was consistent with hereditary pancreatitis with acute flair and reactive pleural
effusions. The patient remained clinically stable but was admitted for fluid
and pain management along with therapeutic thoracocentesis.
Discussion: Hereditary pancreatitis is an autosomal dominant condition,
resulting in recurrent episodes of pancreatitis. Episodes begin in late childhood and are consistent with acute pancreatitis including sharp epigastric
pain, fever, nausea and vomiting. Episodes last 1-3 days and typically become more frequent as the patient ages. Recurrent pancreatitis will eventually lead to chronic pancreatitis, loss of pancreatic function, type 1 diabetes
mellitus and an increased risk of pancreatic cancer. Diagnosis is made
through clinical presentation, lab work consistent with pancreatitis and imaging revealing characteristic pancreatic changes.
Conclusion: Abdominal pain in children can be easily disregarded. It is
important to realize that hereditary pancreatitis is rare, but has life-long
implications and early diagnosis is important for proper management. A wide
differential in children with atypical presentations will protect against rare
diseases falling through the cracks.
276
ALL THAT HURTS IS NOT ARTHRITIS
Velayuthan S1, Gates P2, Chen H1,2, Raman V1,2. 1Louisiana State University Health Sciences Center, Shreveport, LA and 2Shriner’s Hospital for Children, Shreveport, LA.
Case Report: A 17- year-old Hispanic female was admitted for severe joint
contractures. Symptoms started at 2 years of age with fever, joint pain and
swelling. She was diagnosed with Juvenile Idiopathic Arthritis (JIA) and
treated with corticosteroids, methotrexate and anti-inflammatory medications.
Therapy was subsequently discontinued due to lack of response. Her symptoms
progressed to joint contractures and inability to walk. Her brother had similar
disease and died of respiratory complications at the age of 14 years. On examination, the patient was a small-built, wheelchair-bound teenager with severe
flexion contractures in the wrists, fingers, knees, ankles and toes without synovitis or joint effusions. Laboratory studies showed no signs of inflammation
and rheumatoid factor (RF) and anti-cyclic citrullinated peptide were negative.
Contractures in hands.
277
PLASTIC BRONCHITIS PRESENTING IN A 6 YEAR OLD
WITH PNEUMONIA
Walkup JD, Rincon-Subtirelu M, Johnson G. University of Tennessee at
Chattanooga College of Medicine, Chattanooga, TN.
Case Report: A 6 year old female with history of Fontan procedure presented to the emergency room with worsening cough of three days duration.
In addition to this, she had reports of wheezing and a prior diagnosis of
asthma. The initial evaluation, including chest x-ray, was significant for right
lower lobe pneumonia and elevated CRP.
Over the course of 3 days the patient had worsening hypoxemia and required increasing amounts of supplemental oxygen. Her respiratory exam
was quite variable. At times she appeared to be improving in the right lung
fields, but then developed diminished air movement on the left lung fields.
Because of left lung involvement, post-tussive emesis, and new onset of
wheezing, albuterol treatments were given which seemed to improve her
status.
On the fourth day of admission her case took an unexpected turn as she
began to complain of nasal congestion. During the night she began forcefully
blowing her nose trying to clear it and produced a small white tissue-like
substance. This “tissue” was roughly 1 cm in length and appeared to have
a branching pattern to it, resembling a lung cast. It is believed with the
patient’s frequent cough and post-tussive emesis that the lung cast became
lodged in the nasal passage.
Based on the patient’s Fontan physiology, changing lung exam, and
presence of a lung cast, a diagnosis of Plastic Bronchitis was made. Plastic
Bronchitis is the development of bronchial casts that cause airway obstruction, possibly due to endobronchial lymph leakage. This case is unique
because this process has been minimally described in palliated singleventricle physiology and the presence of asthma and pneumonia confounded the diagnosis.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
489
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
278
SEIZURES AND REGRESSION IN A PEDIATRIC PATIENT: A
RARE DISORDER IN A FAMILIAL COHORT
West C, Dunlap M. University of Oklahoma Health Sciences Center,
Oklahoma City, OK.
Case Report: Progressive loss of milestones, cognitive function, and new
onset seizure disorder is an ominous constellation of symptoms. The differential diagnosis includes neuronal ceroid lipofuscinoses (NCL), leukoencephalopathies, mitochondrial disorders, mucopolysaccharidoses, gangliosidoses,
and peroxisomal disorders, a daunting list at best. We present the case of
a 4 year old female who was found to have Neuronal Ceroid Lipofuscinoses,
late infantile type II (CLN2) after progressively worsening seizures and loss of
milestones. Diagnosis was made via lysosomal enzyme testing, with deficiency
of Tripeptidyl-peptidase 1 (TPP-1) in conjunction with concomitant evaluation
of a biological sibling.
Patient is a 4 year old female with complex medical history, clouded by
placement in foster care at 2 years of age. At that time she had a speech delay,
but normal growth and development in other domains. Her first seizure was
at 32 months. Initial genetics work up was negative. She was followed by her
primary care provider for progressively worsened seizures, loss of motor and
speech milestones, and dysphagia with aspiration events. At 4 years she was
admitted for placement of a gastrostomy tube secondary to dysphagia and aspiration. Hospital stay was extended by surgical complications. During her
admission it was noted that a biological sibling, who was being cared for
in a separate foster placement, was also admitted with very similar symptomatology and clinical course. Expanded genetics evaluation included lysosomal enzyme testing for our patient, and conjunctival biopsy with
electron microscopy for the sibling that showed lysosomal inclusions characteristic of Neuronal Ceroid Lipofuscinosis.
Neuronal Ceroid Lipofuscinoses are a group of rare disorders. This case
highlights the importance of continuity of care, particularly for children whose
biological family history is incomplete. As research into this inherited genetic
condition is ongoing, it is important to identify cohorts of affected families
whose disease course and response to treatment provide direction for future research. Lastly, this case draws attention to consideration of NCL in the differential diagnoses when seizures and developmental regression are present.
maintain her blood pressure. After 21 days of antibiotics and acid reduction
therapy, there were no signs of pneumatosis on x-ray, the baby was feeding well,
and she was discharged home.
Fewer than 40 cases of gastric pneumatosis have been reported, mostly in
association with necrotizing enterocolitis and proximal intestinal obstruction. With a presumed infectious etiology, treatment includes broad spectrum
antibiotic therapy and hemodynamic support. When a child presents with
vomiting, it is important to remember this complicated gastrointestinal
history.
280
EARLY INDOMETHACIN USE INCREASES NEC IN
PREMATURE INFANTS
Abu El Haija M1, Rouce R2, Rassin D1, Jain SK1. 1University of Texas Medical Branch, Galveston, TX and 2Texas Children’s Hospital, Houston, TX.
Purpose of Study: NEC is a disease of prematurity with significant morbidity & mortality. Indomethacin (INDO) is COX-2 inhibitor used for treatment of PDA & IVH prophylaxis. INDO ↓es intestinal blood supply which
may ↑risk of NEC. Premature infants (PI) often receive inotrops (DOPA)
&/or steroids (HCT) for hypotension. We explored if early use of INDO
alone or in combination with DOPA &/or HCT ↑es NEC.
Methods Used: In retrospective 1:1 matched case control study, medical
records of infants with NEC (January 2007- March 2010) were collected.
Gestational age matched controls born within 3 months of cases were included. Continuous & categorical variables were compared in NEC versus
controls by using t-test & Pearson chi-square. We used multivariate regression analysis to determine the significance of receiving INDO, DOPA or
HCT alone/combination in developing NEC.
Summary of Results: 64 infants with NEC were matched to 59 controls (table). 63% of the infants with NEC received early INDO (IVH prophylaxis;
p0.02 figure 3) & 6% infants in NEC group developed PVL versus none in
control (p 0.005). NEC group received longer duration of TPN (p 0.0001).
Conclusions: Early INDO use↑es NEC, PVL & duration of TPN use in PI.
279
GASTRIC PNEUMATOSIS
Wright L, Caudill J, Crissinger K, Gremse D. University of South Alabama,
Mobile, AL.
Case Report: Gastric pneumatosis is a very rare entity. This case report
highlights an unusual presentation of an infant with a past medical history
of gastroschisis repair S/P primary repair after birth who developed gastric
pneumatosis.
A 1 month old female presented with a 1 day history of nonbloody,
nonbilious emesis, increased abdominal distention, and excessive irritability,
but no history of fever, rhinorrhea, cough, or diarrhea. She had stable vital
signs and her cardiopulmonary exam was normal. Her abdomen was protuberant, but soft, and nontender with good bowel sounds.
A complete blood cell count, CRP, and electrolytes were normal, but her
liver tests showed total bilirubin 4.9, direct bilirubin 3.7, ALT 69, and AST
84. An acute abdominal series showed mild gaseous distension. An UGI/
SBFT showed reflux to the oral cavity. Her labwork worsened over 3 days
to CRP 6.6 mg/dL, total bilirubin 8.3, direct bilirubin 6.5, AST 172, ALT
144, and GGT 161 so that she underwent a percutaneous liver biopsy. On the
evening of the biopsy, she developed increasing abdominal distention, tachycardia, respiratory distress, and hypotension. An abdominal x-ray showed free
air. Due to concern of a liver biopsy complication, an emergent exploratory laparotomy was performed, but revealed no bleeding from the liver biopsy site or
leaks in the duodenum or several bowel loops in the RUQ. The stomach was,
however, noted to have pneumatosis involving the gastric wall. There appeared
to be several areas of bruising or blood in the stomach wall without obvious perforation. An EGD showed severe gastritis, but no ulcerations or active bleeding.
Blebs in the submucosa were observed during the EGD. Postoperatively, she
remained intubated for 7 days and required a milrinone drip for 6 days to
490
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Perinatal Medicine
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
281
TRANSCUTANEOUS BILIRUBINOMETRY DURING & AFTER
PHOTOTHERAPY IN PRETERM INFANTS
Ansari S, Fonseca RA, Malloy M, Jain SK. University of Texas Medical
Branch, Galveston, TX.
Purpose of Study: Jaundice (NJ) occurs in ≥2/3rd infants. Visual inspection
is not accurate to evaluate NJ specially during & after phototherapy (PHT).
Transcutaneous bilirubin (TcB) is accurate but PHT affects reliability. We
evaluated accuracy of TcB from covered (TcB_C) & PHT exposed skin
(TcB_UC) in preterm infants (PI) during & after discontinuing PHT.
Methods Used: In prospective observational study, we included PI (30-36 wks)
requiring PHT. Infant’s forehead was covered by BiliEclipse & TcB_C/TcB_UC
were measured on infants forhead. PHT was turned off 5 minutes before
measuring TcB. Capillary blood sample was collected (within 15 minutes of
TcB) for serum bilirubin (TSB). TcB_C, TcB_UC, TSB were measured before starting PHT (baseline), every 24 hours during PHT & 24 hours after
discontinuing PHT. ANOVA (analysis of variance) & Bonferroni t-test were
used to compare TcB_c & TcB_UC with TSB.
Summary of Results: We included 64 infants, mean gestation was 33.4 wks,
birth weight 2022 grams, 62% hispanic & 17% DAT positive infants (table).
Before starting PHT, TcB_C, TcB_UC & TSB were not significantly different. TcB_C correlated with TSB after 46, 65 & 89 hours of PHT exposure.
TcB_UC was significantly lower than TcB_C & TSB after 46, 65 & 89 hours
of PHT exposure. 24 hours after discontinuing PHT, TcB_C remained significantly higher than TSB & TcB_UC.
Conclusions: In PI, TcB_C is reliable in measuring bilirubin during PHT
treatment but not after 24 hours discontinuing PHT. It may take >24 hours
to equiliberate TcB_C after discontinuing PHT.
Southern Regional Meeting Abstracts
282
BENEFIT VERSUS RISK OF LUMBAR PUNCTURES IN
EXTREMELY LOW BIRTH WEIGHT INFANTS
Baalbaki S, Ambalavanan. N. University of Alabama in Birmingham,
Birmingham, AL.
Purpose of Study: Meningitis is diagnosed by evaluation of cerebrospinal
fluid (CSF) obtained by lumbar puncture (LP). Extremely Low Birth Weight
(ELBW) infants are sometimes evaluated by LP soon after birth if exposed to
preterm prolonged rupture of membranes (PPROM) or chorioamnionitis,
which are known risk factors for early onset neonatal sepsis. However, it is
unknown if the increased venous pressure associated with infant positioning
for LP increases the risk of severe intraventricular hemorrhage (IVH). We hypothesized that LPs on the first day of life (DOL) are associated with increased risk of severe IVH that may outweigh the benefits of confirming
meningitis in ELBW infants.
Methods Used: Retrospective data analysis of electronic medical records of
343 ELBW infants born at the University of Alabama in Birmingham Hospital in 2010-12 was performed to quantitate the identification of meningitis
and incidence of severe IVH in relation to other clinical variables. A
p<0.05 was considered significant.
Summary of Results: Ninety-two of 343 neonates underwent a LP on
DOL1. Infants receiving a LP were significantly heavier at birth and their
first CBC had higher white blood cell counts, percentage of neutrophils,
and platelet counts. Their mothers were more likely to be diagnosed with
clinical chorioamnionitis and to have had longer PPROM. Only 1 CSF culture
was positive, but the organism (coagulase negative staphylococcus) was considered a contaminant. Of those receiving a LP, 27.2% (25) developed severe
IVH compared to 13.5% (34 of 251) of those who did not. Patients receiving
a LP were 2.97 times more likely to develop severe IVH after adjustment
for other important clinical variables using logistic regression.
Conclusions: A LP on DOL1 is associated with a higher risk of severe IVH
in ELBW infants and a low yield of positive cultures. Clinical algorithms including LPs as part of the sepsis workup in preterm infants may need reevaluation if these findings are validated in larger or multicenter studies.
Demographic
283
TOPICAL NYSTATIN FOR PREVENTION OF CATHETER
ASSOCIATED CANDIDIASIS IN ELBW INFANTS
* % excludes 5 missing values
Bodin MB1,2, Godoy G2, Philips JB1. 1Univ of AL at Birmingham, Birmingham,
AL and 2DCH Regional Medical Center, Tuscaloosa, AL.
Purpose of Study: Systemic infections with Candida species continue to be
major causes of morbidity and mortality in NICUs. The incidence of such
infections in ELBW infants with central catheters has been reported to be between 4% and 15%. Multiple prophylactic protocols have been developed in
attempts to reduce these infections. No protocols published to date used topical therapy and instead relied on oral or systemic treatment. We initiated a
topical nystatin protocol in an attempt to reduce the frequency of systemic
Candidal infections in extremely low birthweight infants with central
catheters while minimizing the risks associated with oral or systemic
prophylaxis.
Methods Used: All infants with a central venous and/or arterial catheter had
a thin layer of nystatin cream (100,000 units/gm) applied to the axillae,
groin, neck, and behind the ears once daily until the access device(s) were
removed. We retrospectively reviewed the electronic records of all infants
<1 kg birthweight admitted to DCH Regional Medical Center between 1/
1/2000 and 12/31/2010 to identify those infected with Candida species. We
also reviewed the microbiology department blood and spinal fluid culture
results. This sudy was approved by the IRBs at the study site and at UAB.
Summary of Results: 464 ELBW infants were admitted during the study period. All had at least one central catheter placed and all received the nystatin
protocol. Of these, 3 (0.65%) developed Candida bloodstream infections.
Conclusions: Topical application of nystatin cream in ELBW infants with
central catheters appears to be an effective prophylactic regimen for prevention of central catheter associated systemic Candidiasis. A larger, randomized trial is needed to confirm this observation.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
491
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Regional Meeting Abstracts
analysis was limited due to small sample size; additional biochemical analyses will be performed after enrollment of an additional 50 infants.
284
TRANSFUSION ASSOCIATED NECROTIZING
ENTEROCOLITIS (TANEC) AT KOSAIR
CHILDREN’S HOSPITAL IN VERY LOW BIRTH
WEIGHT INFANTS (<1500GMS)
1
2
1
286
1 1
Crabtree CD , Pakvasa M , Radmacher P , Adamkin DH . University of
Louisville, Louisville, KY and 2Emory University, Atlanta, GA.
Purpose of Study: To identify recent cases of NEC and to determine if
PRBC transfusions were temporally associated.
Methods Used: A five-year retrospective chart review of all very low birth
weight (VLBW) infants with a diagnosis of NEC (Bell’s Stage ≥ II). Prenatal
data, postnatal clinical course and NEC outcomes were reviewed.
Summary of Results: Sixty cases of NEC from 888 admissions over 5 years
were identified (rate 6.8%, range 2.9% to 15.2%). TANEC occurred in 45%
of cases (27/60). Neonatal profiles were similar between TANEC and nonTANEC infants. Maternal pregnancy profiles were similar except for a significant occurrence of maternal diabetes in non-TANEC infants (15% vs. 0%,
p=0.035). Enteral feeding initiation was at ~4 days of life in both groups
and receipt of human milk (HM) as first feeding was similar. The feeding rate
at the diagnosis of NEC was significantly lower in TANEC infants (125 ±
30 mL/k/d) compared to non-TANEC infants (140 ± 24 mL/k/d, p=0.045).
The age at diagnosis was similar although weight at diagnosis of NEC was
lower in TANEC infants (1159 ± 284 g) compared to non-TANEC infants
(1325 ± 316, p=0.038). At diagnosis of NEC, 90% of non-TANEC infants
were receiving either preterm formula (PTF) or HM fortified with PTF compared to 69% of TANEC infants (p=0.044). Interestingly, 6% of non-TANEC
and 4% of TANEC infants were never fed. Late onset sepsis occurred in
12 infants in each group (37% and 44%, non-TANEC and TANEC infants, respectively) and each group had a similar exposure to antibiotics prior to diagnosis. There were similar organisms recovered from blood and urine. Medical
NEC occurred in 56% of TANEC infants and 61% of non-TANEC infants.
Both groups experienced 18% mortality.
Conclusions: The 5-year rate of NEC for our NICU was similar to nationally
reported rates. Mortality was somewhat lower than national figures. Variability
in rates of NEC from year to year was seen. TANEC occurred more often than
expected but at a rate similar to NEC without contemporary transfusion. The
rate of late onset sepsis was higher than expected and merits further scrutiny.
The role of maternal diabetes during pregnancy was an unexpected finding
which requires further investigation.
IMMUNIZATION STATUS OF INFANTS PRIOR
TO DISCHARGE FROM A LEVEL IV NICU
AT A CHILDREN'S HOSPITAL
Ferguson JM1,2, Harsono M1,2, Sahni J3, Dhanireddy R1,2, Pourcyrous M1,2.
1
University of Tennessee HSC, Memphis, TN; 2Le Bonheur Children’s Hospital, Memphis, TN and 3Le Bonheur Children’s Hospital, Memphis, TN.
Purpose of Study: AAP recommends all infant, regardless of their BW or
GA, be immunized at the recommended age. However the primary care
providers and parents of NICU patients are hesitant, specifically with infants
who have surgical complications and congenital anomalies, and therefore
immunizations are delayed. Since a majority of these infants are still in the
NICU at 2 months of age, it is important to initiate the primary immunization
in the NICU. A recent article reported that only 51% of infants prior to discharge from the NICU were “up-to-date” with primary immunization. The
objective of this study is to assess the immunization status of infants in the
NICU at a children’s hospital where infants may have complex clinical
conditions such as congenital heart disease, multiple congenital anomalies
and surgical interventions.
Methods Used: This is a retrospective chart review of primary immunization
status of 62 infants who were still in the level IV NICU at Le Bonheur
Children’s Hospital at two months of age during the year 2011.
Summary of Results: Birth weight of 37 infants was < 1500g (VLBW) and
of 25 infants was ≥ 1500g. Amongst VLBW infants, 54% needed surgical
GI-interventions. In the VLBW infants, 78% (29 of 37) received primary
immunization ≤ 90 d of age, while 22% (8 of 37) received immunization after
90 d of age. Amongst infants with BW ≥ 1500g, 52% needed surgical GIinterventions and 40% needed intervention for complex congenital cardiac anomalies. Surprisingly, 100% of these infants were immunized before 70 d of age.
Conclusions: Based on the CDC criteria, 2 month old immunizations received at < 91 d of age are considered “up-to-date”. We had 100% “updo-date” compliance in infants with BW ≥ 1500g and 78% compliance in
infants < 1500g. Immunizations that are initiated in the NICU may improve
compliance of subsequent immunizations in these vulnerable infants. Much
of the success in this NICU can be attributed to the multidisciplinary medical
team’s review of immunization needs on daily rounds.
285
287
EARLY VITAMIN D SUPPLEMENTATION FOR PREVENTION
OF RESPIRATORY MORBIDITY IN EXTREMELY PRETERM
INFANTS
BRONZE BABY SYNDROME IN A BLUE FUGATE
2
1
1 1
2
Craig C , Ambalavanan N , McNair T . UAB, Birmingham, AL and University of Alabama School of Medicine, Birmingham, AL.
Purpose of Study: To determine if vitamin D supplementation to extremely
preterm infants reduces respiratory complications, reduces infection, and
prevents bronchopulmonary dysplasia.
Methods Used: Extremely preterm infants born at 28 gestational weeks or
less were randomized to receive a placebo, high dose: 800 IU/day or low
dose: 200 IU/day oral vitamin D; each infant received the dose for 28 days.
This project was an interim analysis of the first 25 patients enrolled in this
study (planned total enrollment=100). An enzyme immunoassay (ELISA)
for 25(OH) vitamin D was done on blood samples from: cord blood (day
1), postnatal day 14, and postnatal day 28.
Summary of Results: Vitamin D concentrations were compared to the number
of days requiring respiratory support, defined as the need for supplemental oxygen or positive pressure ventilation to maintain oxygen saturation levels between 88-95%. The average vitamin D concentrations were 13.57ng/ml in
cord blood, 29.5ng/ml in day 14 blood, and 34.14ng/ml in day 28 blood (normal
range 30-50ng/ml). The rank correlation coefficient for the length of stay in the
hospital was R=-0.33 for cord blood, R=-0.15 for day 14 blood, and R=-0.08 for
day 28 blood, with significant p values for each day.
Conclusions: Nearly all extremely premature infants were found to be very
deficient in vitamin D at birth. However, no correlation was found between
serum vitamin D concentrations and respiratory morbidity. This preliminary
492
George A, Sharma A, Dang S. University of Kentucky, Lexington, KY.
Case Report: A term exclusively breast fed female infant born via C-section
to a 24 year old Caucasian mother with gestational diabetes developed hyperbilirubinemia on third day of life. Risk factors for hyperbilirubinemia included parietal cephalohematoma, ABO setup and maternal gestational
diabetes. Serum bilirubin level was 20.3mg/dL and phototherapy was started.
After 8 hours of phototherapy, the infant developed a bluish-gray discoloration of skin. Parents belong to the family of “Blue Fugates of Kentucky”
so the differential diagnosis included methemoglobinemia, breast feeding
jaundice, ABO incompatibility and cholestasis.
The Fugates, a family that lived in the Appalachian region of Kentucky,
are the most famous example of hereditary methemoglobinemia. This population is named after the family of Martin Fugate- a French native who immigrated to Kentucky in 1820. This family carries an autosomal recessive
disorder, lacking the enzyme cytochrome-b5 methemoglobin reductase
which reduces spontaneously formed methemoglobin in the body.
Our patient was supplemented with formula and phototherapy was continued. Initial laboratory studies ruled out methemoglobinemia as well as
ABO incompatibility. Bilirubin levels started trending down but the discoloration persisted. Interestingly, when the temperature probe was removed from
the trunk, the discoloration was not noticed on that area. At that point, a diagnosis of bronze baby syndrome (BBS) was entertained.
BBS as a complication of phototherapy was first described by Kopelman
et al in 1972. BBS is rare and is noted in infants with elevated levels bilirubin
in their blood, especially after starting phototherapy. The etiology of BBS is
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
unknown, but it is believed to be caused by an abnormal accumulation of unexcreted photoproducts of bilirubin or an unknown pigment that has the
characteristic absorbance on spectrophotometry. Liver function tests performed on the infant were normal and her discoloration resolved after discontinuation of phototherapy confirming the diagnosis.
288
DOES INTENSE LACTATION EDUCATION HELP INCREASE
THE PROVISION OF BREAST MILK AT 32 WEEKS OR LESS?
Kahn L, Eichenwald E. University of Texas, Health Science Center,
Houston, TX.
Purpose of Study: The purpose of this study was to determine whether
more intense lactation education and ongoing maternal intervention would
increase the initiation of pumping and the duration and amount of breast
milk supply to preterm infants compared with standard protocols in place
in the NICU.
Methods Used: After IRB approval as a quality improvement initiative, enrollment began in October, 2012. We planned to enroll 60 mothers in each group
or stop enrollment by October 31, 2013 or whichever came first.
Inclusion criteria included:
32 weeks or less, inborn only
Maternal stability after delivery
English speaking
No contraindication to breast feeding
Baby expected to go home with birth mother
Intervention mothers were enrolled in months the PI was off clinical service. Infants delivered during service months served as controls and received
standard lactation education. The intervention included a face to face interview within 48 hours of birth by the PI, a special pamphlet addressing the
provision of breast milk to a premature infant and additional telephone contact at one week post discharge.
The primary outcome was the amount of breast milk available for each
baby assessed at 48 hours, one, two and four weeks of life.
Summary of Results: Recruitment is on going. Complete analysis will be
available at the time of the meeting. We expect to find increased provision
of breast milk to our premature population born to mothers in the intervention group.
Conclusions: Maternal provision of breast milk to preterm babies is an important aspect of their medical care with obvious health benefits. Understanding
whether different models of lactation support affect maternal decisions to provide breast milk to their vulnerable infants is essential.
Southern Regional Meeting Abstracts
respiratory status and abdominal distention. Due to concern for pulmonary hypertension, an echocardiogram was done. There was no evidence of pulmonary
hypertension but there were bilateral pleural effusions and massive abdominal
ascites. An abdominal ultrasound showed normal liver architecture and blood
flow. Paracentesis revealed milky white fluid significant for triglycerides
>1000mg/dL, 99% lymphocytes and WBC>2000/mm3. He was treated with
bowel rest, TPN, octreotide, spironolactone, and multiple paracenteses for comfort and with resolution of his pleural effusion but persistence of his ascites.
Work up for chylous ascites was negative with no evidence of abdominal
masses, malrotation on Upper GI, or liver dysfunction. We suspect that his
chylothoax and ascites could be a result of the rapid changes in the intrathoracic pressure secondary to his severe CLD and assisted ventilation. The
patient continued to have ascites and worsening electrolyte abnormalities despite maximum medical therapy. Pediatric surgery was consulted for further
evaluation and management, however due to continued deterioration in his
clinical status, the parents decided to withdraw care.
Discussion: This case represents a case of chylothorax and persistent chylous ascites due to suspected rapid changes in the intra-thoracic pressure in
a neonate with severe pulmonary disease. Although not a common presentation, it should be considered as an etiology when evaluating chylothorax and
chylous ascites.
290
COMBINING TRANSCUTANEOUS CO2 MEASUREMENT
WITH SERUM BICARBONATE TO PREDICT CAPILLARY
BLOOD GASES
Lee M, Elabiad M. University of Tennessee Health Science Center, Memphis, TN.
Purpose of Study: Our objective was to estimate a capillary blood gas
(CBG) by combining the partial pressure of carbon dioxide (PaCO2)
obtained noninvasively by transcutaneous PaCO2 monitoring (TCOM) with
serum bicarbonate obtained on a basic metabolic panel (BMP). The estimated CBG would be compared to the measured CBGs obtained as part of
routine care.
Methods Used: A TCOM measurement was be obtained within 60 minutes
of a scheduled lab collection that included a CBG and a BMP. As precision
of TCOM decreases with high PaCO2 levels, levels above 55 mm Hg were
excluded from the final analysis. CBG was estimated using the PaCO2 measurement on TCOM, the bicarbonate level on a BMP, and a calculated pH using
the Henderson-Hasselbalch equation. The study outcome was the number of
calculated CBGs that were in agreement with measured CBGs. Statistically,
this was defined as a measurement within 1 standard deviation from the mean
based on a Bland-Altman analysis and clinically a pH within ±0.1 units of the
measured pH but not crossing a pH interval of 7.3-7.45. If calculated pH
crossed the pH interval, then the clinical agreement drops to within ±0.05 units
of measured CBG.
Summary of Results: Twenty seven simultaneous measurements of TCOM
and blood work were obtained. Fifteen TCOM measurements were <55 mmHg.
289
IATROGENIC CHYLOUS ASCITES IN A FORMER PREMATURE
INFANT WITH SEVERE CHRONIC LUNG DISEASE
Lee C, Makoni MM, Chaaban H. OUHSC, Oklahoma City, OK.
Case Report: Introduction: Chylous ascites in infants and neonates is rare.
There is limited data on pathogenesis, diagnosis, and treatment. We present
a case of iatrogenic chylothorax and ascites in a former premature infant with
severe chronic lung disease (CLD).
Case Description: The patient was a 5 month old male infant who presented
to our hospital for gastrostomy tube (GT) and tracheostomy tube placement.
He was a former 26 week premature infant with a history of pulmonary hypertension, stage 4 bronchopulmonary dysplasia (BPD) with significant
hyperexpansion and suspected blebs, TPN cholestasis, and poor growth.
On arrival, he had severe respiratory acidosis requiring increasing ventilator
settings resulting in worsened hyperexpansion. He improved gradually and underwent an uncomplicated tracheostomy. A few days later, he had worsening
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
493
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Fourteen of the 15 calculated CBG had the pH within 1 standard deviation from
the mean and satisfied the clinical definition of agreement (figure).
Conclusions: Combining transcutaneous PaCO2 with serum bicarbonate is a
safe and accurate method to estimate a CBG. Its use would save on blood
volume and time of the procedure as well as on cost of running the CBG.
291
FIVE YEAR EXPERIENCE (2007-2011) WITH NECROTIZING
ENTEROCOLITIS AT KOSAIR CHILDREN’S HOSPITAL IN
VERY LOW BIRTH WEIGHT INFANTS (<1500GMS)
Pakvasa M1, Crabtree CD2, Radmacher P2, Adamkin DH2. 1Emory University, Atlanta, GA and 2University of Louisville, Louisville, KY.
Purpose of Study: To review the 5-year experience with necrotizing enterocolitis and transfusions.
Methods Used: A five-year retrospective chart review of all very low
birthweight (VLBW) infants who developed NEC (Bell’s Stage > II). Sixty
cases (6.8%) from 888 admissions were identified. Prenatal data and postnatal clinical course were reviewed as to the antecedents associated with NEC.
Summary of Results: The 5-year NEC rate for our NICU was 6.8% with annual variation from 2.9% to 15.2%. Perinatal and neonatal profiles revealed
mean birthweight 983 grams and 27 weeks’ gestation. Sixty-three percent
of infants were delivered by c-section and 78% of mothers had received
antibiotics prior to delivery. Approximately 1 in 4 mothers admitted to smoking during the pregnancy and 15% admitted to illicit drug use. Respiratory
distress syndrome was diagnosed in 98% of infants and 77% received at least
1 dose of surfactant. Forty-seven percent received pharmacologic therapy for
PDA and 13% were ligated. One half of the cohort developed late onset sepsis. Eighteen percent had positive cultures at diagnosis of NEC. Enteral feeding initiation was at ~4 days of life with 62% receiving human milk as first
feed. NEC was diagnosed at a mean (± SD) postnatal age of 28 ± 15 days.
Forty percent of infants were receiving human milk (HM, plain or fortified)
at the time of diagnosis and the feeding rate was 129 + 37 mL/kg/day. Transfusion associated NEC (NEC diagnosed within 48 hours of transfusion,
TANEC) occurred in 27 infants (45%). Medical NEC occurred in 58%,
and 18.3% of cases resulted in death.
Conclusions: The overall rate of NEC for our NICU was similar to nationally reported rates, while mortality in our cohort was lower than national
figures. Year to year variability in rates of NEC were seen. Late onset sepsis
and TANEC were more common than expected. We speculate that strategies
to decrease both late-onset sepsis and transfusion exposure will improve
outcomes.
292
ADDITIONAL EFFECTS OF ABSENT END-DIASTOLIC
FLOW IN PREMATURE INFANTS?
Parker WJ, Ray CS, Bazacliu C, Waller J, Bhatia J. Medical College of
Georgia, Georgia Regents University, Augusta, GA.
Purpose of Study: Determine the effects of fetal absent or reversed enddiastolic flow on morbidity and mortality in premature infants.
Methods Used: A 5-year retrospective study identified 58 fetuses with absent or reversed end-diastolic flow prior to delivery. Infants were grouped
into those <30 [n=26] or 30 to 36 and 6/7[n=32] weeks gestation. Data collected included maternal age, gravida and parity, birth weight, 1 and 5 minute
Apgar Scores, intrauterine fetal demise, death in delivery room, neonatal
death, hepatic dysfunction [Alanine aminotransferase, aspartate aminotransferase, direct bilirubin], Necrotizing enterocoitis [NEC], Intraventricular
hemorhage [IVH], oxygen requirement at 36 weeks post menstrual age
[CLD], length of stay and malformations. Data analyzed by t-tests or
Wilcoxon Rank Sum test for continuous variables and chi-square or Fisher’s
Exact test for catgorical variables.
Summary of Results: Infants below 30 weeks gestation demonstrated lower
median Apgar scores at 1- and 5 minutes, lower mean platelet count, higher
median length of stay and higher ALT, higher incidence of NEC, Stage II or
greater, chronic lung disease, death in the delivery room, and death in the
neonatal period than infants greater than 30 week gestation [Chi square or
Fisher’s Exact tests]. Further, incidence of chromosomal abnormalities or
malformations was significantly greater in infants > 30 than < 30 weeks
[p=0.0063] gestation.
494
Conclusions: Infants born after absent or reversed end-diastolic fetal flow
born prior to 30 weeks gestation, have lower Apgar Scores, higher ALT,
higher incidence of NEC, CLD and death compared to a cohort greater than
30 weeks. Incidence of congenital anomalies was greater in the larger cohort
of infants. These data are similar to previous reports for major morbidity, but
suggest the need for further studies based on the incidence of malformations.
Limitations of our study: retrospective, small number of subjects, no comparable group of infants without aberration in umbilical flow.
293
IS RETINOPATHY OF PREMATURITY AN EMERGING
PROBLEM IN INDIA?
Patel R1, Starner C1, Shah MJ2, Talati AJ1. 1UTHSC, Memphis, TN and 2Polio Foundation, Ahmedabad, India.
Purpose of Study: Retinopathy of prematurity (ROP) is a developmental
disease of the retina in which deficient vascularization occurs. ROP is a leading cause of childhood blindness worldwide and the number one cause of
childhood blindness in the U.S. The risks for ROP are multifactorial and include low birth weight and prematurity. The current screening guidelines in
the United States entails that any infant, who weighs less than 1500g at birth
or is born at 30 weeks or less, be screened because they are at risk for developing ROP. Since ELBW infants’ survival is poor in India, physicians do not
think of ROP as a significant problem. We proposed to review the problem of
ROP in an urban setting in India, and emerging nationality.
Methods Used: A prospective chart review, during a one-month duration in
Ahmedabad, India. Polio Foundation’s ROP team was followed and observed
the screening eye exams performed by Retcam and later reviewed by an Ophthalmologist. Retcam was used by the Polio Foundation team in several
hospitals throughout the city of Ahmedabad. Clinical data was collected via examining patient charts. Preterm infants with BW ≤ 2000 gm or ≤ 32 weeks
were screened.
Summary of Results: 67 patients who had a mean BW of 1479(SD 471)
grams were screened. There were 7 (10.5%) who required treatment and this
group had a mean birth weight of 1175grams (SD 445). The mean gestational age of the total population was 31.5 wk (SD 2.9). The patients requiring treatment had a mean gestational age of 29 wk (SD 1.5). There was no
correlation found between gender and developing ROP. Twenty-one percent
of the twin patients required treatment suggesting the possibility of it being
a risk factor. Of the 7 patients requiring treatment, 6 of them had been on
supplemental oxygen and 2 of them had been put on ventilation, suggesting
the definite role of supplemental oxygen.
Conclusions: A large number of babies needed treatment for ROP in this cohort. The infants requiring treatment were heavier in this cohort compared to
infants in United States. The data from each country should be collected and
guidelines developed to screen these preterm infants as more premature
babies survive in this emerging and developing nationalities.
294
PARTIALLY THROMBOSED UMBILICAL VEIN VARIX WITH
ASSOCIATED TETRALOGY OF FALLOT
Patrick-Esteve J, Eggie D, Buis M. LSUHSC-New Orleans/Children’s Hospital, Gretna, LA.
Case Report: Thrombosis of an umbilical vein varix (UVV) is a rare finding
with unclear clinical consequences. Various outcomes depending on associated
anomalies have been reported. One recent case series, of infants with UVV,
found a higher incidence of associated fetal abnormalities including fetal anemia, chromosomal abnormalities and intrauterine death. We present a case of
a partially thrombosed UVV with associated Tetralogy of Fallot.
Our infant, twin A, was born at 33 WGA via cesarean section secondary
to oligohydramnios, non-reassuring fetal status, and breech positioning. He
was born to a 32 year old, G3 P1 mom who conceived dichorionic diamniotic
twin boys after a round of clomid injections. Maternal history was complicated by chronic hypertension, anti-phospholipid antibody syndrome, hypothyroidism, and a history of HSV. Maternal medications included
Procardia, Lovenox, Aspirin, progesterone injections and Armour thyroid.
Prenatal ultrasound showed concern for umbilical artery aneurysm. The infant was delivered at 1425 grams and immediately required resuscitation
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
and intubation. Apgar scores at 1 and 5 minutes were 3 and 7, respectively.
The infant was soon transferred to a tertiary care facility where an Echocardiogram confirmed the finding of TOF, and an abdominal ultrasound
revealed a partially thrombosed UVV, without involvement of the portal vein.
The UVV was initially monitored with weekly abdominal ultrasounds,
followed by less frequent monitoring as the thrombosed varix remained
stable.
There is still no overall consensus on management of prenatally diagnosed UVV, although, regular NST and ultrasound for monitoring has been
recommended. Our infant presents with several findings known to increase
the risk of morbidity and mortality associated with UVV, including abnormal
NST, IUGR, TOF and oligohydramnios. Furthermore, he presents with a partially thrombosed UVV, a condition which has been associated with intrauterine fetal demise. Emergent delivery of our infant due to non-reassuring NST
may have prevented a potential intra-uterine demise secondary to a partially
thrombosed UVV. With close monitoring of his UVV no intervention was
necessary, and the patient is doing well at home with outpatient management
of his congenital heart disease.
295
INTRAVENTRICULAR HEMORRHAGEA PREVENTABLE BAGGAGE
Ravisankar S1,2, Higginson J1,2, Stroud T2, Williford A2. 1East Carolina
University, Greenville, NC and 2Vidant Medical Center, Greenville, NC.
Purpose of Study: We initiated a quality improvement project to decrease
the incidence of Intraventricular hemorrhage (IVH) in our unit. IVH is identified
as one of the major problems impacting the survival, neurodevelopment and eventual quality of life of premature neonates. We reviewed the data of all our inborn
patients over the last two years to evaluate the incidence of severe IVH and
all IVH (< 1500 gram patients) and were surprised by our high numbers 12.6% for severe IVH (IQR 3.3-10.3) and 31.1% for all IVH (IQR 13.627.0) compared to centers within the Vermont Oxford Network. Recognizing such worrisome numbers, we scrutinized our practice to identify key
variables which may have contributed to such high incidences and noticed
that our admission temperatures were well below the accepted standards.
Vermont Oxford data showed us with a 51.3% (IQR 8-34) rate for admission temperatures less than 36C in infants <1500 grams birth weight. Our
aim in this quality initiative is to reduce our incidence rate of IVH by half
and improve admission temperatures.
Methods Used: Streamlined admission protocol for all babies < 29 weeks
primarily starting in the delivery room to maintain optimal admission
temperatures, followed by a Golden hour rule to meet specific deadlines at
the end of the first hour. In addition to ensuring that the overbed warmers
are preheated to 100% we have started using a chemical warming mattress
and placing all babies < 29 weeks in a polythene bag more consistently with
a designated personnel from the delivery team ensuring compliance. A protocol for intravenous indomethacin for IVH prophylaxis was drafted for use
in suitable patients. Other changes include maintaining neutral head position
with the head end elevated at 30 degrees for the first 72 hours, clustered medical and nursing care with minimized handling, judicious use of sedation,
avoiding routine suction and decreased noise and light stimulation.
Summary of Results: Preliminary data has shown improvement with our
current efforts coupled with education and awareness.
Conclusions: A formalized protocol combined with collective efforts of the
team can improve hypothermia and reduce IVH rates of premature neonates.
296
DOES TREATMENT WITH BEVACIZUMAB AFFECT
NEURODEVELOPMENT IN INFANTS WITH RETINOPATHY
OF PREMATURITY?
Shipp KE1, Huff LL1, Taylor SN1, Deacon B 2, Saunders R2, Ebeling M1,
Ruddy-Humphries A1, Shirer K2, Katikaneni L1. 1Medical University of
South Carolina, Charleston, SC and 2Medical University of South Carolina,
Charleston, SC.
Purpose of Study: Intravitreal bevacizumab (IVB) is rapidly replacing laser
therapy as the treatment of choice for infants with retinopathy of prematurity
Southern Regional Meeting Abstracts
(ROP), but its systemic effects on the developing child remain largely unknown.
The purpose of this study is to determine whether infants with ROP treated with
IVB have worse neurodevelopmental outcomes than infants treated with laser
therapy (LT).
Methods Used: We performed a retrospective chart review of infants seen in
neonatal follow-up clinic comparing infants who received IVB (with or without LT) with those treated with LT only for ROP. Infants were evaluated in
cognitive, fine motor, language, and gross motor development using the
Capute Scales (CAT/CLAMS) and modified Peabody Developmental Motor
Scale. Developmental quotients (DQ) were calculated for prematurity-adjusted
age. Parents completed Ages and Stages Questionnaires (ASQ). Data analysis
was performed using Student’s t test and Fisher’s exact test.
Summary of Results: 21 infants were included in the study, with 12 in the
IVB group (IVBG) and 9 in the laser group (LTG). The mean gestational
age and birth weight for both groups were 24.9 wks (23-26 3/7) and 636 g
(435-955) respectively, and were similar between the two groups. 86% of
the subjects were ≤26 wks. The assessments occurred between 6 and 39
months of age, with 83% of the IVBG and 78% of the LTG being >1 year
old at follow up. The groups were comparable in z-scores of weight, length,
and head circumference. No significant differences were noted between the
IVBG and LTG in global cognitive (DQ 82 and 72, respectively, p=0.47),
language (DQ 93 and 73, p=0.12), or gross motor development (DQ 78
and 66, p=0.37). The groups showed similar findings in muscle tone, with
33% normal tone in the IVBG and 22% normal in the LTG (p=0.66). No significant difference was found in ASQ pass rate between the two groups, with
50% passing in the IVBG and 33% passing in the LTG (p=0.66).
Conclusions: In our study of children with ROP followed up to 39 months of
age (most >1 year old), no significant difference in neurodevelopment was
detected between those treated with IVB and those who received only LT.
297
IDENTIFYING THE POSSIBLE ETIOLOGIES OF CEREBRAL
PALSY IN AHMEDABAD, INDIA
Starner C1, Patel R1, Shah MJ2, Talati AJ1. 1UTHSC, Memphis, TN and 2Polio
Foundation, Ahmedabad, India.
Purpose of Study: Cerebral palsy (CP) is a nonprogressive syndrome of
neurological deficits that results in dysfunction of muscle coordination,
movement, and posture. CP is caused by brain injury and can be congenital
or occur later in infancy. Thesymptoms of CP depend upon the severity of
brain injury. Therefore, the disease can be very mild or extremely severe.
CP is classified based on the type of motor abnormality present in the patient.
The three broad classifications are spastic, dyskinetic, and ataxic. Spastic CP
can further be categorized by the extent of muscle paralysis in the appendicular skeleton and can range from monoplegic to quadriplegic. Dyskinetic CP
can be subcategorized into chorea, dystonia, and athetosis. In the US, the
prevalence of CP is about 2 to 4 in every 1000 children. The exact prevalence
of CP in India is difficult to obtain. The purpose of this study was to identify
preventable causes of CP in a population of patients receiving treatment at a
charitable organization in Ahmedabad, India. Future guidelines can be developed to address the identifiable causes.
Methods Used: In this study we collected information on current CP
patients attending the non-profit, Polio Foundation Hospital, in Ahmedabad,
India. During a four week stay in Ahmedabad, we observed and interviewed
the charts and parents of the children with CP. We looked at delivery mode
delivery location, and closely examined the birth history to identify the cause
of CP. The majority of patients were born in urban private hospitals and all
but one patient were born in a hospital.
Summary of Results: A total of 43 patients were assessed with mean age of
6.9 years (SD 4.6). There were 28 males and 15 females in the cohort.
Assessing the etiologies of CP from the 43 patients interviewed, we found
that 25 had a birth related cause, 2 were genetic malformations, 3 were preterm, 4 were infectious, and 8 were “other”. All 43 patients in the cohort
were undergoing physical therapy as treatment and 12 of them had needed
at least one operation or surgery in addition to physical therapy.
Conclusions: This study showed that CP in this cohort in Ahmedabad was
largely birth related. Home-delivery did not seem to be a major contributory
factor. Improving care during delivery and the neonatal period can help decreasing the incidence of CP in this population.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
495
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
298
RETINAL LASER ABLATION THERAPY AND CORPUS
CALLOSUM GROWTH
Thekkiam, Muralidharan S, Zayek MM. USA Children and Women Hospital,
Mobile, AL.
Purpose of Study: In spite of absence of major brain imaging abnormalities,
extremely low birth weight infants (ELBWI) remain at risk for major disabilities at later age. Abnormal development of corpus callosum (CC) and
cerebellum has been associated with adverse neurodevelopmental outcome.
Our aim is to determine whether severe retinopathy requiring laser therapy
may affect the development of the CC and the cerebellar vermis (VS).
Methods Used: ELBWI with severe intra-ventricular hemorrhage (Grade 3
or 4), periventricular leukomalacia or cerebellar hemorrhages were excluded
from the study. We reviewed charts of 63 ELBWI with gestational age <25
weeks and divided them into 2 groups, “Laser” (n=21), and “control” (n=42).
Brain MRI was performed for all babies at Term equivalent age (TEA). We
measured mid-sagittal surface area of the Corpus callosum (CCSA) and
the VS by Ultrasound (US) at birth, 6 weeks and 16weeks post natal age
(PNA) and at TEA by MRI.
Summary of Results: There was no significant difference in head circumference, weight and VS size at birth and at 16 weeks between Laser and Control
groups. However, the CCSA was significantly smaller, in the Laser group, at
16wks by US (p=0.034) (figure) and at TEA by MRI. The median
(interquartile range) of CCSA by MRI was 1.02 (0.84-1.19) cm2 in the Control group at PNA 16 (15-18) weeks vs. 0.65 (0.54-0.71) cm2 in the Laser
group at PNA 20 (17-22) weeks, (p<0.001). After adjusting for gestation age or birth weight, the decrease in the CCSA remained significant in
the Laser group.
Conclusions: Severe retinopathy in ELBWI requiring laser therapy is associated with a reduction in CC size. Pathophysiology for these findings
requires further analysis.
On physical exam, weight was 2870 gm, length 47 cm, OFC 34 cm.
There was episodic tachypnea with respiratory rate > 100/minute, inspiratory stridor and episodes of apnea lasting more than 45 seconds with central
cyanosis and bradycardia. Additional findings included hypotonia, horizontal nystagmus, polydactyly and a soft globular compressible 2 cm swelling
in the occipital region consistent with a cranial defect. No other dysmorphic
features were noted. Patient had initial blood gas analysis indicating respiratory alkalosis, with pH-7.58, pCO2-13, pO2-160, HCO3-12.2. CBC, CRP,
chemistry panel, urinalysis, lactate, pyruvate and ammonia were within normal limits. Head ultrasound found a 13mm defect in occipital skull with herniating brain matter. MRI showed a small midbrain with thickened and
elongated superior cerebellar peduncle (“molar tooth sign”). The 4th ventricle
appeared triangular shaped at mid level and bat-wing shaped superiorly. Cerebellar hemispheres appeared dysgenetic and triangular in shape superiorly.
There was a tiny herniation of a CSF filled sac through a bony defect in the midline occipital region. Joubert syndrome was diagnosed based on collection of
physical and imaging findings. Cardiac echo, ophthalmologic evaluation, ultrasound of abdomen were normal.
Interventions included intubation because of apnea episodes, orogastric
tube feeding and multi-disciplinary team including neurology, neurosurgery,
physical therapy, and speech therapy.
Joubert syndrome should be considered in the differential diagnosis of
infants having irregular breathing, inspiratory stridor, nystagmus, occipital
cranial defects and polydactyly.
300
CLINICAL FEATURES OF NEWBORNS READMITTED TO
THE HOSPITAL FOR MANAGEMENT OF
HYPERBILIRUBINEMIA
299
A NEWBORN PRESENTING WITH A CONSTELLATION OF
SYMPTOMS DIAGNOSED WITH JOUBERT SYNDROME
Vala SK, Burns JJ. FSU College of Medicine, Pensacola, FL.
Case Report: A full term male neonate was admitted to NICU for evaluation
of irregular breathing, hypotonia and stridor in the first hour of life. Mother
was 31 year old AB +, serology/GBS negative, with no history of drugs, alcohol or tobacco use during pregnancy. No ultrasound was done due to inadequate prenatal care. Delivery was by repeat C-section with Apgar scores of
5/7/8; a weak cry was noted immediately after birth. Family history was significant for developmental delay of male sibling who died at 6 months of age.
496
Waite S, Leysath A, Burns JJ. FSU, Pensacola, FL.
Purpose of Study: The purpose of this study was to determine which clinical
features are present in newborns re-admitted to the hospital for management
of hyperbilirubinemia.
Methods Used: A retrospective chart review was performed of newborns
readmitted for management of hyperbilirubinemia over a 2-year period. Frequency data obtained included feeding method, gestational age, the category
of bilirubin level per Bhutani nomogram at nursery discharge, age and percent weight loss at nursery discharge, presence of hemolysis, and age and
percent weight loss at readmission.
Summary of Results: 42 infants were readmitted to the hospital for management of hyperbilirubinemia. Descriptive statistics: 81% of these infants were
breast-fed, 69% were <38 weeks gestational age and 64.3% were both
breastfed and <38 weeks gestational age. Hemolytic disease was present in
11.9% of infants; 16.7% had jaundice in the first 24 hours of life and 9.5%
required phototherapy in the nursery. Mean age at discharge was 2.3 days
(S.D. 0.46). Category of discharge bilirubin: 2.4% high risk (>95th percentile), 42.9% high-intermediate risk (75%ile-95%ile), 40.5% low-intermediate
risk (40%ile-75%ile) and, 7.1% low risk (<40%ile). Average weight loss at
discharge was 5.79%; 2.4% had weight loss >10%. At readmission, average
age was 5.3 days (S.D. 1.07); average weight loss was 7.88% (S.D. 3.60);
average bilirubin level was 18.45 mg/dL (S.D. 2.32). Infants discharged
<48 hours old had higher bilirubin levels at readmission when compared
to those discharged > 48 hours old with total serum bilirubin levels of
20.0 mg/dL vs 17.7 mg/dL respectively (t =3.54;p= 0.001). 31% of the
infants readmitted for hyperbilirubinemia had >10% weight loss, which
was statistically different from discharge. (McNemar p=0.001).
Conclusions: Babies who are readmitted for hyperbilirubinemia usually are
breast fed, premature and are 5 days of age and a significant number had
weight loss >10% from birth weight. Discharge from the nursery < 48 hours
of life is associated with higher bilirubin levels on readmission. Babies even in
the low intermediate category of bilirubin at time of discharge can comprise a
significant proportion of readmissions. This study suggests that ensuring effective breastfeeding and even later discharge practices especially in preterm
infants might reduce some of the readmissions for newborn jaundice.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Pulmonary and Critical Care Medicine
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
301
AN UNUSUAL PSYCHOSIS: ANTI-N-METHYL-D-ASPARTATE
RECEPTOR ENCEPHALITIS: A CASE REPORT
Carthan KA, Murphy DJ. Emory University School of Medicine, Atlanta, GA.
Case Report: 28 year old female with a history of anemia presented to the
emergency department from a psychiatric facility with altered mental status
and seizure activity. At the psychiatric facility, she was receiving thorazine,
haloperidol, and benztropine for a diagnosis of severe anxiety with psychotic
features. Initial physical exam revealed a fluctuating level of consciousness
(i.e. periods of orientation to time and place alternating with periods of hostility and inappropriate laughter) as well as fluid responsive hypotension and
tachycardia. She was admitted to the MICU for hemodynamic instability and
altered mental status. Initial differential diagnosis included neuroleptic malignant syndrome (NMS), infectious encephalitis, toxic ingestion, autoimmune disorders, and paraneoplastic syndromes. Empiric treatment with
antibiotics for infectious encephalitis and bromocriptine for NMS was initiated pending the results of CT head, lumbar puncture, toxicology screen,
and electroencephalogram (EEG). When initial studies were non-diagnostic,
antibiotics and bromocriptine were discontinued and her symptoms were
controlled with benzodiazepines and anti-epileptics. On hospital day 21,
CSF fluid analysis showed anti-NMDA receptor antibodies supporting a diagnosis of anti-NMDA receptor encephalitis. Subsequent transvaginal ultrasound revealed a 3.7 cm x 3.5 cm right ovarian cyst and she later underwent
salpingo-oopherectomy. Her mental status slowly improved and she was
discharged home on hospital day 46.
Conclusions: Anti-NMDA receptor encephalitis is a rare cause of altered mental status with variable clinical presentations and frequent overlap with other
diseases. This syndrome is frequently associated with ovarian teratomas and removal is the treatment of choice. A high index of suspicion and early lab evaluation is important for prompt diagnosis and management of this disorder.
References:
Gregory S. Day, Sasha M. High, Bianca Cot, David F. Tang-Wai. AntiNMDA-Receptor Encephalitis: Case Report and Literature Review of an
Under-Recognized Condition. JGIM 26:811-816.
Southern Regional Meeting Abstracts
later showed an RVSP 75.2mmHg and RHC two years following her diagnosis was similar to her initial RHC.
Conclusion: PH is an under recognized morbidity in individuals with
CLOVES syndrome. Pulmonary embolism and venous thromboembolism
has also been reported and if untreated can progress to chronic thromboembolic
pulmonary hypertension and possibly death. This report highlights that prompt
recognition of PH in patients with this rare disorder may reduce morbidity.
References
Ahmad I Alomari. Characterization of a distinct syndrome that associates
complex truncal overgrowth, vascular, and acral anomalies: a descriptive
study of 18 cases of CLOVES syndrome. Clinical Dysmorphology. 2009,
18:1-7.
303
LIFE THREATENING WEGENER’S GRANULOMATOSIS: AN
UNCOMMON DISEASE MASKED BY COMMON SYMPTOMS
Cohen C1, Prabhakaran P1,2, Diaz F1,2. 1Children’s of Alabama,
Birmingham, AL and 2Children’s of Alabama, Birmingham, AL.
Case Report: Wegener’s granulomatosis (WG) is an ANCA-associated vasculitis that is progressive in nature, classically causing upper and lower airway disease with renal involvement. We report a 16 year-old previously
healthy female who presented critically ill with shock and pulmonary hemorrhage. The month prior to presentation included multiple upper airway
infections and cough progressing to respiratory distress with hemoptysis.
Community acquired pneumonia and acute respiratory distress syndrome
were suspected. She was admitted to the intensive care unit after intubation
and the initiation of vasopressors. A pulmonary-renal syndrome was suspected when she was found to have oliguric renal insufficiency and hematuria. The diagnosis of WG was confirmed with the detection of an antibody to
proteinase 3. Clinical improvement was noted after initiating treatment for
WG including intravenous corticosteroids, plasmapheresis, rituximab, and
cyclophosphamide. This report emphasizes that early recognition of symptomatology, with high index of clinical suspicion, is necessary for prompt diagnosis of WG. Often children with undiagnosed WG will present with
constitutional symptoms including fever, arthralgias, recurrent sinusitis, otitis media, and stridor. The development of diffuse alveolar hemorrhage,
which ultimately led to her diagnosis of WG, is the presenting feature in
up to 44% of cases.
302
CONGENITAL LIPOMATOUS OVERGROWTH,
VASCULAR MALFORMATIONS, EPIDERMAL NEVUS,
AND SCOLIOSIS SYNDROME AND PULMONARY
HYPERTENSION: A CASE REPORT
Carthan KA1, Udoji TN2. 1Emory University School of Medicine, Atlanta,
GA and 2The Ohio State University, Columbus, OH.
Case Report: 41 year-old African-American female with a history of systemic
hypertension and CLOVES syndrome was evaluated for progressive dyspnea
on exertion, and syncope. She was WHO functional class III and physical examination revealed loud P2 component of S2, S3 gallop and other stigmata of
right heart disease. There were multiple truncal vascular malformations and upper extremity epidermal nevi consistent with a diagnosis of CLOVE syndrome.
Transthoracic echocardiogram (TTE) showed a dilated right atrium with a pressure of 15 mmHg and an estimated pulmonary artery systolic pressure (PASP)
of 39.2 mmHg. Ventilation Perfusion (V/Q) scans showed several matched
defects. Right heart catheterization (RHC) showed a mean right ventricular systolic pressure (RVSP) of 84/5mmHg, PASP of 79/37 mmHg (mean 54 mmHg)
and a Fick cardiac output of 4.20 L/min.
She was diagnosed with Group 1 PH and was started on treprostinil
which improved her functional class. She was weaned off supplemental oxygen and outpatient pulmonary function test showed moderate restrictive
physiology and mild reduction in diffusion capacity. She was later switched
to sildenafil and ambrisentan and continued to do well. Repeat TTE one year
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
304
306
INTRA-ABDOMINAL CHYLOTHORAX
ESOPHAGEAL PERFORATION AND BILATERAL EMPYEMA
FOLLOWING ENDOSCOPIC ESOPHYX
Dixon K, Wilhelm C, Wilhelm A. University of Mississippi Medical Center,
Jackson, MS.
Case Report: A chylothorax is defined as leakage of lymphatic fluid or chyle
into the pleural space, normally caused by traumatic or mechanical obstruction
of the thoracic duct. Malignancy is the most common cause of non-traumatic
chylothorax. Here we present an atypical presentation of chylothorax.
A 42 year old African American female with hypertension and obesity
was admitted to the Gynecologic Oncology service for evaluation of an
adnexal mass found on ultrasound. She complained of menorrhagia, increasing abdominal girth, twenty pound weight gain, and worsening shortness of breath with dyspnea on exertion for several months prior to
admission. Physical exam findings included decreased breath sounds in
the right hemithorax, and a distended, non-tender, protuberant abdomen.
Computed tomography (CT) revealed massive bilateral ovarian masses
(18 cm and 19 cm in diameter), abdominal ascites, and a large loculated
right pleural effusion, with leftward mediastinal shift and compressive atelectasis of the right middle and lower lobes. Pleural fluid analysis from
bedside thoracentesis showed an exudative effusion with chylothorax,
and triglycerides of 234 mg/dL. A paracentesis was performed, and 4.5 liters
of hazy, yellow fluid were drained, however fluid studies were not performed. Her dyspnea improved after a chest tube was placed, and a second
CT of her chest was done after the fluid was drained. No mediastinal
lymph nodes, masses, or other cause of thoracic duct disruption was identified. The patient subsequently underwent total abdominal hysterectomy
and bilateral salpigo-oopherectomy. Pathology returned as metastatic carcinoma, likely breast primary. The chylothorax resolved after surgery,
and the chest tube was removed prior to discharge.
Pleural fluid triglycerides > 120 mg/dL suggests lymphatic drainage out
of the lymphatic system into the surrounding potential space. Because there
was no intrathoracic abnormality causing thoracic duct obstruction, our differential diagnosis for the etiology of her chylothorax included chylous ascites translocating through a diaphragmatic defect, intra-abdominal lymphatic
duct obstruction from massive tumor burden, and Meig’s syndrome. When
confronted with a chylothorax of unknown etiology, intra-abdominal pathology should be considered as the cause.
Edriss H, El-Bakush A, Nugent K. Texas Tech University Health Science
Center, Lubbock, TX.
Case Report: We report a 48-year-old white man was diagnosed with GERD
and hiatal hernia 15 years ago. He underwent transoral incisionless
fundoplication (TIF) after failing conservative therapy. A few hours after surgery, the patient developed midsternal chest pain. Physical examination
revealed decreased breath sounds on the left side, O2 saturation was 88%
on a 40% Venti-mask. The next day, the patient’s respiratory status deteriorated and he was intubated for mechanical ventilation. Chest x-ray showed
a large left-sided pleural effusion and bilateral pulmonary infiltrates consistent with consolidation. Laboratory studies: white blood count 16.9 × 109/L,
hemoglobin 16 g/dl, Arterial blood gases revealed: pH-7.27, PaCO246.7mmHg, PaO2-53.2 mmHg, HCO3– 21. Computed tomography of the
chest showed moderate-to-large left-sided and moderate right-sided pleural
effusions with compressive atelectasis/consolidation of both lower lobes,
left sided pneumothorax, and pneumomediastinum, findings consistent
with distal esophageal perforation. The patient spiked a fever (101.2°F) on
post-operative day two. A left-sided chest tube was inserted, and purulent,
brown-greenish fluid was drained (1300 ml). Pleural fluid analysis showed
an exudative effusion consistent with esophageal perforation with empyema.
The patient had fever for five days and leukocytosis despite the antibiotic
coverage, chest tube placement. He also developed acute kidney injury secondary to sepsis which resolved over four days. Blood cultures were negative; pleural fluid culture grew coagulase negative staphylococcus and diphtheroides. On
postoperative day eight, a left side thoracotomy and decortication for non-resolving empyema was performed. The patient was able to be successfully
extubated four days later. He was discharged six days after extubation.
The transoral incisionless fundoplication using the EsophyX device is
relatively effective in treating GERD. However, the complications with this
procedure can be life threatening.These include bleeding and esophageal perforation complicated with pneumothorax, empyema, respiratory failure, and
mediastinitis. We need post marketing studies to assess the safety, efficacy,
and clinical outcomes using this novel procedure, and these patients need
close post procedural follow-up
306A
305
CITALOPRAM OVERDOSE CAN CAUSE NONCARDIAC
PULMONARY EDEMA
Edriss H, Pfarr M, Nugent K. Texas Tech University Health Science Center,
Lubbock, TX.
Case Report: We report a 53-year-old man who ingested 2400 mg of
citalopram and presented to emergency department three hours post-ingestion
with altered mental status, somnolence, and a blood pressure of 67/45 mmHg.
He failed to respond to three boluses of normal saline (1000 ml each) and required vasopressors. The patient developed serotonin syndrome with hyperreflexia, rigidity, and ankle myoclonus. He had a tonic-clonic seizure in the
ER requiring intravenous lorazepam and phenytoin. An ECG showed QT prolongation. Chest X-ray on presentation was normal. Within 32 hours the patient
developed acute respiratory distress, hypoxemia, a wide A-a gradient, PaO2/
FiO2 < 200, and chest X-ray changes compatible with acute respiratory distress
syndrome (ARDS). He had normal central venous pressure, normal cardiac
biomarkers, normal systolic and diastolic function on echocardiography, and
no acute ST/T wave changes. His ABG showed a metabolic acidosis and a respiratory acidosis. The patient required intubation and ventilation but recovered
within 24 hours.
Citalopram has been associated with seizures and ECG abnormalities after overdoses. The respiratory complications and metabolic acidosis have
been reported only a few times in the literature. We are reporting the second
case of ARDS; the respiratory failure developed during hospitalization as the
neurotoxicity resolved. This man is the fifth case of metabolic acidosis due to
a citalopram overdose; his metabolic acidemia may be explained by drug metabolism to propionic acid. The respiratory acidosis seen in this patient has
not been reported previously.
498
WBC AND TEMPERATURE EVENTS DURING MECHANICAL
VENTILATION
Edriss H, Whiting JW, Ngo N, Nugent K. Texas Tech University Health Science Center, Lubbock, TX.
Purpose of Study: The criteria for SIRS include changes in WBC, temperature, respiratory rate, and heart rate. Changes in WBC counts and/or temperature could have important implications in patients on ventilators, but
the frequency of these changes (events) is uncertain.
Methods Used: We reviewed the electronic medical records and x-rays from
281 ventilation episodes in our medical intensive care unit from 12/1/2012 to
4/30/2013 to determine the characteristics of patients requiring ventilation,
the indications for ventilation, the initial arterial blood gases, and the number
of patients meeting the criteria for a ventilator-associated event. We determined the number of days during each ventilation episode in which the temperature ( <96.8°F, >100.4°F) or WBC ( <4K/μL, >12K/μL) was out of the
normal range.
Summary of Results: This cohort included 257 patients who required 281
episodes of mechanical ventilation. 25.3% of patients were <50 years old,
46.3% were 50-70 yrs, and 28.4% were >70 yrs. 55.4% were men, and
66.2% were overweight or obese. The mean APACHE 2 score was 13.5±5.9.
The initial PaO2/FiO2 was 210±110, the PCO2 was 45.1±22.8, and the pH
was 7.3±0.1. The median number of ventilator days was 4 with an interquartile
range (25%-75%) of 3-9. The overall mortality was 29.5%. 196 out of 275 eligible ventilator episodes (71.3%) had 1 temperature event; 95 had 2 temperature
events and 42 had 3 temperature events. 194 out of 253 eligible ventilator
episodes (76.7%) had 1 WBC count out of range; 51 had 2 WBC events and
8 had 3 WBC events. Nineteen patients (6.7% of episodes) met CDC criteria
for a ventilator-associated event based on deterioration in gas exchange. Twelve
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
of these patients with ventilator-associated events had an increased WBC
count during the two days before through two days after the event. Eleven
patients had an increased temperature during this period.
Conclusions: Increases in both WBC counts and temperatures occur frequently in ICU patients on ventilators and do not reliably identify patients
with ventilator events defined by the new CDC criteria. These SIRS parameters need evaluation when they occur but cannot be used to identify
patients with ventilator-associated complications.
307
HOSPITAL READMISSIONS FOLLOWING
TRACHEOSTOMIES IN THE NEONATAL INTENSIVE
CARE UNIT: A RECENT EXPERIENCE FROM ONE
ACADEMIC CENTER
Hundalani S1, Arnold J1,2. 1Baylor College of Medicine/ Texas Childrens
Hospital, Houston, TX and 2Texas Children’s Hospital, Houston, TX.
Purpose of Study: To describe the indications, surgical timing, length of
stay and early readmission rates of infants discharged from the neonatal intensive care unit (NICU) with tracheostomies.
Methods Used: Retrospective chart review of infants undergoing tracheostomy placement over an 18-month period from June 2011-Dec 2012 and
discharged home from the NICU in a large urban academic pediatric hospital. Data is presented as median (IQR).
Summary of Results: Of 46 infants who underwent a tracheostomy, 31
(67%) met inclusion criteria. 45% were male, 35% inborn, 39% ≤1kg at
birth; Post menstrual age (PMA) at tracheostomy was 50 weeks (44-54)
and total hospital stay was 162 days (109-207). While PMA at tracheostomy
was similar in patients ≤1kg and >1kg, ≤1kg group had a longer post tracheostomy stay (p=0.02). 95% tracheostomies were elective, of which 80% were
for prolonged mechanical ventilation. In 75% patients ≤1kg, the primary indication was chronic lung disease. In >1kg, airway obstruction was the primary indication in 52 % and chronic lung disease in 16%. 22% patients
were on tracheostomy collar at discharge. 26%(8/31) infants were readmitted
within 1 week, 84%(26/31) readmitted within 6 months, with 16%(5/31) requiring four or more admissions. 3/8 readmissions within the first week were
related to airway emergencies (1 mucus plug, 2 codes requiring CPR) and 3/
8 were related to infections. The six-month mortality rate was 3%. One death
within the first year post discharge was due to cardiopulmonary arrest secondary to tracheostomy dislodgement.
Conclusions: 37% readmissions in the first week post discharge were related
to airway emergencies, some of which could be prevented with effective
management by caregivers. Our tracheostomy education is very comprehensive including CPR education and rooming in prior to discharge. In addition,
we have piloted a simulation based project to train parents in tracheostomy
related airway emergencies. While hospital readmissions are to be expected
in this complex population, we hope to help them be more prepared and
skillful with the initial management of airway emergencies at home.
308
STATUS ASTHMATICUS IN A PATIENT WITH
HYPERTROPHIC CARDIOMYOPATHY
Kallur SA1, Dahm P1, Banker A2, Yadav A3, Erikson C1. 1University of
Texas Health Science Center Medical School, Houston, TX; 2University of
Texas Health Science Center Medical School, Houston, TX and 3University
of Texas Health Science Center Medical School, Houston, TX.
Case Report:
Introduction: Asthma is a common childhood illness that presents with
wheezing and affects 1 in 11 children. It is a chronic inflammatory disorder
of the airways, characterized by an obstruction of the bronchial tree. Hypertrophic cardiomyopathy (HCM) is a genetic defect of the cardiac myocyte.
Hypertrophic obstructive cardiomyopathy (HOCM) is HCM with obstruction of the left ventricular outflow tract. In the pediatric population the incidence of HOCM is about 4.7 cases/million. HOCM presents in 10% of
children as congestive heart failure, with wheezing, cough and chest tightness. Our case describes HOCM presenting with asthma.
Case Report: This is a case of a 13 year old male with past medical history
of mild intermittent asthma. He presented in respiratory failure secondary to
Southern Regional Meeting Abstracts
status asthmaticus. Per family, the patient began to develop symptoms the
day of admission. His typical rescue medications did not improve his symptoms. At the outside hospital, he was unresponsive, emergently intubated, and
transferred for a higher level of care. On our initial exam, the patient was noted
to have a harsh 2/6 systolic murmur louder at rest. The patient’s asthma was
treated successfully and he was extubated the following day. Upon standing
up on the day of extubation, he had a syncopal event. Cardiac enzymes were elevated. The echocardiogram from the day of admission validated the clinical
suspicion of HOCM. The patient had pulmonary function tests done at the time
of hospitalization confirming obstructive airway disease. He was discharged on
albuterol,, fluticosone, metoprolol, and montelukast,with follow up in cardiology clinic.
Conclusion: Beta-blockers are the mainstay of treatment to relieve
symptoms in HOCM . The use of beta-blockers in the setting of asthma is
controversial. In our search of the literature, there were no cases of HOCM
and asthma reported. This case presents an interesting dilemma where
treating one disease worsens the second. Further research needs to be done
to provide the safest treatment for this population.
309
TRANSFORMING GROWTH FACTOR BETA 1-INDUCED
EPIGENETIC MODIFICATION OF THY-1 IN PRIMARY
LUNG FIBROBLASTS
Neveu W, Mills T, Sueblinvong V. Emory University School of Medicine,
Atlanta, GA.
Purpose of Study: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease and its incidence increases with age. Although the causative mechanisms remain poorly understood, there is compelling experimental
and clinical data implicating the pro-fibrotic cytokine transforming growth
factor β1 (TGFβ1) in its pathogenesis. We previously identified in senescent
mice a pro-fibrotic lung phenotype with an increase in the number of
fibroblasts negative for Thy-1, a cell surface molecule associated with fibrosis. As Thy-1 acts as a fibrotic suppressor and that loss of gene expression by
epigenetic modification leads to a fibrotic phenotype in lung fibroblasts, we
evaluated whether TGFβ1 epigenetically regulates Thy-1 differentially in
primary lung fibroblasts from young vs. old mice.
Methods Used: Primary lung fibroblasts harvested from young (3 month) and
old (24 month) mice were treated with TGFβ1 ± the DNA methyltransferase
(DNMT) inhibitor 5-Azacytidine (5-AZA) for 72 hrs and analyzed for Thy-1
gene expression by quantitative RT-PCR. DNMT gene expression (e.g. DNMT
1, DNMT3a, and DNMT3b) were analyzed by quantitative RT-PCR in cells at
baseline and following TGFβ1 treatment at 24 and 72 hrs.
Summary of Results: TGFβ1 treatment for 72 hrs decreased Thy-1 gene expression in fibroblasts from young mice. In contrast, TGFβ1 treatment in the
presence of increasing doses of 5-AZA had a minimal impact on Thy-1 expression. As lung fibroblasts from old mice express higher levels of TGFβ1
and lower levels of Thy-1 at baseline, we treated them with 5-AZA alone and
identified an increase in Thy-1 gene expression relative to untreated cells. Interestingly, no significant difference was observed in DNMT expression in
response to TGFβ1 treatment in fibroblasts from young or old mice, but this
is in fact consistent with previous studies showing dissociation between
DNMT mRNA levels and protein activity.
Conclusions: TGFβ1 appears to induce Thy-1 methylation in lung
fibroblasts, and the decreased expression of Thy-1 in fibroblasts from old
mice may be a consequence of elevated TGFβ1 and activation of the DNMT
pathway. Future studies to determine how Thy-1 is regulated as fibroblasts
age may identify therapeutic targets in IPF.
310
HIV-RELATED PROTEINS INHIBIT THE NRF2/ARE
SIGNALING PATHWAY IN ALVEOLAR MACROPHAGES
Singh M, Fan X, Guidot D. Emory University and the Atlanta VA, Atlanta, GA.
Purpose of Study: Infection with the human immunodeficiency virus (HIV)
causes oxidative stress within the airways, as best reflected by glutathione depletion within the alveolar space, and induces alveolar macrophage dysfunction; however, the mechanism(s) are poorly understood. Nuclear factor
(erythroid-derived 2)-like 2 (Nrf2) is a redox-sensitive master transcription
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
factor that regulates the activation of the anti-oxidant response element
(ARE), a group of anti-oxidant gene targets that have a significant role in cellular responses to oxidative stress. We hypothesized that HIV- related
proteins (Tat and gp120), independent of HIV infection, decrease Nrf2 expression in alveolar macrophages and thereby impair activation of the
ARE, ultimately leading to oxidative stress and macrophage dysfunction.
Methods Used: A rat alveolar macrophage cell line (NR8383 cells) and a
human alveolar macrophage cell line (THP-1 cells) were incubated with either recombinant gp120 (100ng/ml) or Tat (10ng/ml) for 24 hrs (at which
time gene expression was assessed) or 72 hrs (at which time protein expression was assessed). The relative gene and protein expression of Nrf2 and the
Nrf2/ARE-dependent genes glutamate-cysteine ligase catalytic subunit
(GCLC) and NAD(P)H dehydrogenase [quinone] 1 (NQO1) were quantified
by QRT PCR and western blot analyses, respectively.
Summary of Results: Treatment with either gp120 or Tat decreased Nrf2
and NQO1 gene and protein expression in both cell lines. In parallel, THP1 cells treated with either GP-120 or Tat had decreased gene expression of
GCLC, which is the rate-limiting step in glutathione synthesis.
Conclusions: Taken together, these findings suggest that HIV-1-related
proteins, which are expressed and are present in relatively high concentrations
in the alveolar lining fluid in chronic HIV infection, inhibit anti-oxidant
defenses in the alveolar macrophage. We hypothesize that activation and/or
over-expression of Nrf2 may be able to rescue the alveolar macrophage from
the toxic effects of these HIV-related proteins and thereby enhance lung immune health in these vulnerable individuals.
311
EXTRA INTESTINAL MANIFESTATION OF
CROHN’S DISEASE
Velayuthan S, Umer S. Louisiana State University Health Sciences Center,
Shreveport, LA.
Case Report: Extra intestinal manifestations of Crohn’s disease in children
are diverse and well documented. We report a case of Crohn’s disease with
persistent pulmonary nodules treated as recurrent pneumonias with poor response to antibiotic therapy and responded well to oral steroids.
A 16-year-old girl with medically controlled Crohn’s disease presented to
us with a foot ulcer and pleuritic chest pain. The foot ulcer was diagnosed as
pyoderma gangrenosum with biopsy. The chest radiograph (CXR) at presentation revealed left lower and right middle lung lobe opacities. The previous
CXR obtained a year ago showed similar opacities and further history revealed
poor response to antibiotic treatment for presumed recurrent pneumonias. Serology for histoplasma, coccidioides and mycoplasma were negative. Immunoglobulins, serum angiotensin levels, anti glomerular antibodies and c ANCA
were normal. Computed tomography of chest showed left lower lobe nodule
with scattered multiple small nodular opacities. Gram stain from broncho alveolar lavage showed few neutrophils and the cultures were negative. Biopsy from
the left lung nodule showed nodular foci with chronic non specific inflammation. Sputum and tissue cultures for mycobacterium and fungi were negative.
Due to multiple unsuccessful treatments with different antibiotics and
unrevealing laboratory evidence for infection, pulmonary manifestation of
Crohn’s disease was suspected. Treatment with oral steroids was initiated
and the lung opacities and pyoderma gangrenosum on foot slowly disappeared.
The recent CXR shows some scars and no nodules or opacities. This case
implies the importance of awareness among clinicians about the uncommon
manifestation of Crohn’s disease.
312
THE FREQUENCY OF VENTILATOR-ASSOCIATED EVENTS
IN A MEDICAL INTENSIVE CARE UNIT
Whiting JW, Edriss H, Ngo N, Nugent K. Texas Tech University Health
Sciences Center, Lubbock, TX.
Purpose of Study: The CDC has developed new criteria to identify ventilatorassociated complications, including pneumonia. These criteria require an increase in FiO2 by at least 20% or an increase in PEEP by 3 cm H2O for at least
two days after a period of improving or stable gas exchange. We wanted to determine how frequently this happened and what caused the deterioration in gas
exchange.
Methods Used: We reviewed the electronic medical records and x-rays from
281 ventilation episodes in our medical intensive care unit from 12/1/2012 to
4/30/2013 to determine the characteristics of patients requiring ventilation,
the indications for ventilation, the initial arterial blood gases, and the number
of patients meeting the criteria for a ventilator-associated event.
Summary of Results: This cohort included 257 patients who required 281
episodes of mechanical ventilation. 25.3% of patients were <50 years old,
46.3% were 50-70 yrs, and 28.4% were >70 yrs. 55.4% were men, and
66.2% were overweight or obese. The mean APACHE 2 score was 13.5±5.9.
The initial mean PaO2/FiO2 was 210±110, the PCO2 was 45.1±22.8, and the
pH was 7.3±0.1. The median number of ventilator days was 4 with an
interquartile range (25%-75%) of 3-9.The overall mortality was 29.5%. Nineteen patients (6.7% of all episodes) met CDC criteria for a ventilator-associated
event; 6 met FiO2 criteria (31.6%) and 13 met PEEP criteria (68.4%). Twelve of
these patients (63.2%) had an increased WBC count (> 12K/μL) during the two
days before through two days after the event. Eleven patients had an increase in
temperature (> 100.4°F) during this period. Based on review of the medical
records, cultures, changes in antibiotics, and changes in chest x-ray, we identified the following explanations for the change in gas exchange parameters. Four
had pneumonia, 4 had atelectasis, 5 had CHF, 2 had ARDS, and 3 had miscellaneous reasons.
Conclusions: Approximately 7% of the patients in this cohort had a ventilatorassociated event. Most of these events represented PEEP changes. The clinical
diagnoses varied and included pneumonia, ARDS, atelectasis, and CHF. Only
4 patients appeared to have a ventilator-associated pneumonia. These new
CDC criteria may not identify most ventilator-associated complications.
313
THE UTILITY OF TRACHEAL ASPIRATES IN
THE MANAGEMENT OF PATIENTS WITH
VENTILATOR-ASSOCIATED EVENTS
FIGURE 1. CT scan showing pulmonary nodules.
500
Whiting JW, Edriss H, Ngo N, Nugent K. Texas Tech University Health
Sciences Center, Lubbock, TX.
Purpose of Study: The Clinical Pulmonary Infection Score uses the presence
of purulent secretions to help make the diagnosis of ventilator-associated pneumonia. It is unclear whether information recorded in the medical record about
sputum collected by tracheal suctioning provides useful information.
Methods Used: We reviewed the electronic medical records and x-rays from
281 ventilation episodes in our medical intensive care unit from 12/1/2012 to
4/30/2013 to determine the characteristics of patients requiring ventilation
and the number of patients meeting the criteria for a ventilator-associated
event identified by new CDC criteria. We analyzed information in the records
about tracheal aspirates on the event day using a semi-quantitative scoring
method to characterize the sputum recovered.
Summary of Results: This cohort included 257 patients who required 281
episodes of mechanical ventilation. 74.7% were older than 50 yrs, 55.4%
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
were men, and 66.2% were overweight or obese. The mean APACHE 2 score
was 13.5±5.9. The initial PaO2/FiO2 was 210±110, and the PCO2 was 45.1
±22.8. The median number of ventilator days was 4 (interquartile range: 3-9).
The overall mortality was 29.5%. 19 patients (6.7% of episodes) met CDC
criteria for a ventilator-associated event. Patients with purulent, thick sputum
in larger quantities had the same temperature, WBC, and number of ventilator days as patients with less abnormal sputum (Table).
Conclusions: There is significant variability in the quantity and characteristics of the sputum aspirated from patients with ventilator-associated events.
The information collected during routine care does not help with the clinical
diagnosis in these patients.
TABLE: Sputum classification and clinical parameters
*Semi quantitative scores for sputum volume, consistency, purulence, and blood. Values
range from 0-7. F-focal infiltrate, B-bilateral infiltrates, N-none, Pn-pneumonia, Vent-vent management, Atel-atelectasis, Aspir-aspiration, Un-uncertain
314
WHAT PATIENTS WITH NEWLY DIAGNOSED PULMONARY
HYPERTENSION WANT TO HEAR FROM PHYSICIANS:
A DESIRE FOR HONEST, CLEAR AND HOPEFUL
COMMUNICATION WITH CONCERN FOR NEED
OF IMPROVED PHYSICIAN EDUCATION
Yandle G, Grewal H, Erbil J, Lauto P, Smart F, DeBoisblanc B, Lammi MR,
Saketkoo LA. LSU Health Sciences Center, New Orleans, LA.
Purpose of Study: To broadly understand the health-related communication
needs of patients with pulmonary hypertension (PH). To collect qualitative
data from PH patients that will guide investigations development of communication protocols and self-efficacy strategies.
Methods Used: Patients from a sequential convenience sample at a national
PH patient conference were asked the following question: ‘For a patient with
a new diagnosis of PH, is there anything a doctor could say that would be
helpful? For example, what would you have liked to know when you were
first diagnosed with PH?’. Written responses were coded by 4 independent
investigators utilizing inductive analysis. Fisher’s Exact test was used to compare differences between groups.
Summary of Results: 72 patients (mean age 54 yrs) participated (Table 1).
Patients responded that optimism was important and that available treatment
options justified physician communication of hopefulness. Patients conveyed
that receiving compassionate, clear and honest knowledge about PH was vital. Discussions of disease mechanisms and treatment were rated as important to patients at time of diagnosis. Guidance on external sources of
knowledge was desired. Patients often volunteered that primary care and pulmonary physicians needed heightened education on PH. When comparing
iPAH to non-iPAH, significantly more iPAH patients responded that “Expected
Disease Impact” (p=0.02,OR=8.8), “Web-Site Guidance” (p=0.04,OR=3.3),
and “Support Organizations” (p=0.01,OR=4.7) were needed at time of PH diagnosis; but otherwise there were no significant differences amongst groups.
Conclusions: This examination provides important insight into desired patientphysician communication at time of PH diagnosis. These results suggest that
the development of physician communication protocols that include information on PH resources may support a stronger sense of patient well-being and
promote better health outcomes. Importantly, the frequency of patient
expressions of medical ignorance of PH (information not asked for) warrants
efforts to educate community physicians.
Southern Regional Meeting Abstracts
315
FATIGUE MAY PARALLEL DSYPNEA IN IMPACT ON
FUNCTIONING AND QUALITY OF LIFE IN PATIENTS WITH
PULMONARY HYPERTENSION
Yandle G, Grewal H, Erbil J, Lammi MR, Lauto P, Smart F, DeBoisblanc B.
Saketkoo LA. LSU Health Sciences Center, New Orleans, LA.
Purpose of Study: To take inventory of the most concerning symptoms of
patients with PH.
Methods Used: A convenience sample of patients attending a national PH
patient conference, independently completed a questionnaire designed for
this investigation to capture patients’ concerns in an unbiased manner. The
patients registered their personal and disease demographics with the research
division of the conference. Questions were open-ended, non-leading, nonspecific and inquired about patient experiences with regard to disease impact,
disability, access to health care, health provider communication and coping.
Surveys were analyzed by inductive methodology whereby data were deconstructed to single concepts from which a coding system was allowed to
emerge. Fatigue was assigned to phraseology referencing energy, exhaustion,
tiredness, or fatigue; while dyspnea was assigned to phraseology describing
breathing, shortness, difficulty walking, or desaturation. Depression and anxiety
were identified with similar inscrutable terms. Statistical comparisons of dichotomous categorical variables utilized Fisher’s Exact test. Comparisons across
patient groupings of age and medication utilized Chi square analysis.
Non-parametric variables were compared using Mann Whitney U.
Summary of Results: 71 patients completed the survey. Median age was 54
years (range 15-77). NYHA Functional Class (FC) assignments were I (8%),
II (51%), III (31%) and IV (3%). 82% of respondents supplied fatigue as an
unsolicited symptom while 84% supplied dyspnea. Anxiety, depression,
memory loss and pain were each experienced by <20% of the group. There
were 161 occurrences of fatigue phraseology within the questionnaires compared to 122 occurrences of dyspnea (p=0.1). Dyspnea (p=0.004) but not fatigue was correlated with NYHA FC. Neither dyspnea nor fatigue correlated
with age or type of medication used.
Conclusions: An unbiased survey of PH patients revealed fatigue as a central experience with high impact on patients’ quality of life and function. Fatigue rivaled dyspnea across all PH groups. Further investigations to measure
fatigue as a potential metric of residual disease activity and to examine strategies that manage fatigue in PH patients are warranted.
Renal, Electrolyte and Hypertension
Joint Plenary Poster Session and Reception
5:00 PM
Thursday, February 20, 2014
316
SPINAL EPIDURAL ABSCESS IN HEMODIALYSIS PATIENTS
Afzal U1, White JM1, Jin C1, Baer S2, Huber L1,2, Colombo R1, Kintziger K1,
Kheda MF1, Nahman NS1. 1Georgia Regents University/Medical College of
Georgia., Augusta, GA and 2VAMC, Augusta, GA.
Purpose of Study: Spinal epidural abscess (SEA) may be a serious infectious comorbidity in hemodialysis (HD) patients. SEA may be the result of
bacteremia (BAC), but other factors are possible. We have previously shown
that BAC occurs in over 20% of incident HD patients, suggesting a significant risk for this condition (Chebrolu, IDSA, 2012). To more fully address
the role of BAC and other co-morbidities associated with SEA, we queried
the United States Renal Data System (USRDS).
Methods Used: All incident HD cases from the USRDS for calendar years
2005-2008 were queried for a diagnosis of SEA, BAC and several potential
clinical covariates using ICD-9 diagnosis codes submitted for Medicare billing.
Descriptive statistics and log-binomial regression analysis were performed.
Summary of Results: For the 4-year period of study, 355,084 patients were
available for analysis. The median age was 65 years. SEA was identified in
660 (0.19%) patients. Vascular access type at the initiation of HD included
AVF in 47,732 (13.4%), AVG in 14,179 (4.0%) and vascular catheter in
293,173 (82.6%) patients. Of the demographic variables, black race [relative
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
501
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
risk (RR) 1.218, 95% confidence interval (CI) 1.025- 1.447] and female sex
[RR 1.179, 95% CI 1.009-1.379] carried significant risks for SEA. Infectious
comorbidities, including diagnosis of BAC [RR 7.757, 95% CI 6.432-9.355],
MRSA infection [RR 2.684, 95% CI 2.192-3.286] and hepatitis C [RR
1.660, 95% CI 1.223-2.253] were associated with the highest risk. Of the
non-infectious factors, diabetes mellitus [RR 1.515, 95% CI 1.278-1.797]
and vascular catheters [RR 1.347, 95% CI 1.008-1.800] carried an increased
risk of SEA.
Conclusions: Spinal Epidural Abscess is an uncommon but serious complication of HD. The risk of spinal epidural abscess is highest in patients with
infectious co-morbidities, especially bactremia, but diabetes and the presence
of a central vascular catheter are also significant risk factors.
317
EMERGENCE OF ATYPICAL MYCOBACTERIAL EXIT SITE
INFECTION IN CURRENT ERA OF USE OF TOPICAL
GENTAMICIN FOR INFECTION PROPHYLAXIS IN
PERITONEAL DIALYSIS PATIENTS
Ahmad M1, Bansal S1, Paolo F2,1, Salazar M3. 1University of Texas Health
Sciences Center at San Antonio, San Antonio, TX; 2South Texas VA Medical
Center, San Antonio, TX and 3University Hospital, San Antonio, TX.
Case Report: Peritoneal catheter-related peritonitis and exit site infections
(ESIs) are associated with significant morbidity in peritoneal dialysis (PD)
patients. Topical Gentamicin has been widely used for prevention of such
infections. Recently, there have been reports of atypical mycobacterial ESIs
from outside US and Gentamicin cream is postulated to be one of the potential risks because of selective pressure on other micro-organisms. Here, we
report a series of 3 patients who had Mycobacterium Fortuitum ESIs on prophylactic exit-site Gentamicin cream from our center.
Three patients, all male, average age of 30 yrs, on PD for 1-8 months,
presented with exit site pain, redness and exudate. They were started on empirical Cephalexin at the time of presentation. Initial culture reported gram
positive diphtheroid-like organisms. However, in 5-7 days laboratory identified organisms as M fortuitum sensitive to Azithromycin, Ciprofloxacin and
Doxycycline in each patient. Patients were switched to combination of either
of two of these antibiotics. Since atypical mycobacteria are ubiquitous in the
environment, cultures were taken from water supply, PD fluids and contact
surfaces from patient’s home and dialysis center to find the source, however,
these remained negative. First patient required removal of PD catheter for
non-resolving infection. Second patient had resolution of ESI but required
catheter removal for ESI and concomitant peritonitis from coagulase negative staphylococcus after 4 months of initial presentation. Third patient recovered completely. All 3 patients were able to stay on chronic PD after
brief discontinuation in 2 patients.
There is emergence of atypical mycobacterial ESI in recent years internationally. The clinical presentation is similar to other bacterial ESIs, therefore,
diagnosis requires high vigilance since the treatment involves prolonged use
of dual antibiotics. Topical gentamicin use is a possible risk factor but needs
confirmation in large studies. For now, use of Mupirocin and Gentamicin on
alternative basis is an option to avoid these infections.
318
WHEN COKE IS NOT HYDRATING: COCAINE INDUCED
ACUTE INTERSTITIAL NEPHROSIS
Bahaa Aldeen M, Talibmamury NB, Ismaael T, Adenwala YE, Smalligan RD.
Texas Tech Univ Health Sciences Center, Amarillo, TX.
Case Report: A 47yo African American (AA) man was admitted with 4 days
of back pain, N/V and low urine output. There was no h/o fever, dysuria, frequency, hesitancy, viral symptoms, trauma, rash, or constipation. Despite his
PMH of HTN, DM, and hyperlipidemia he denied taking any meds for 18
months including NSAIDs, acetaminophen or antacids. He denied smoking
and alcohol but admitted to cocaine use. No significant FH. PE: BP 235/125,
HR 90, T98, O2 sats nl. Lungs and heart normal, abd soft but bilat CVAT. Neuro
exam normal. Labs: Cr 10.5 (1.2 in 2010), BUN 63, Gluc 151, Ca 9.4, PO4 6.1,
Hgb 15, WBC 9.1, platelets 167k, amylase/lipase nl, AST/ALT nl, bili 1.4, AP
39, CPK 127. Hep panel, C&P-ANCA, anti-GBM, anti-myeloperoxidase,
ANA and H. pylori were all negative. C3, C4 nl. UA: 2+ blood, no WBCs or
502
eos, no casts, no albumin, neg for nitrate/LE and bacteria. Imaging: CXR,
abd x-rays, CT abd, EKG and TTE all normal.
Course: Pts urine output declined from 700cc/d to 400cc/d and the third day
he required hemodialysis with Cr 14. Renal biopsy showed typical findings
of interstitial nephritis. The patient was dialyzed for 10 days and responded
to steroids and went home with an improving Cr of 3.5.
Discussion: Internists encounter patients with acute kidney injury (AKI) on
a daily basis, most of which can be explained by prerenal azotemia, ATN, obstruction, or rhabdo among other etiologies. Cocaine is only rarely implicated as an etiology of AKI and if it is, usually the injury is due to ATN or
pigment effects. Acute interstitial nephritis (AIN) caused by cocaine, on
the other hand, has only been described in a handful of cases. AIN is a renal
lesion that causes a decline in creatinine clearance and is characterized by an
inflammatory infiltrate in the kidney interstitium and is most often associated
with drug therapy. AIN can also be seen in autoimmune disorders like SLE,
Sjögren’s, or sarcoidosis; or with infections remote to the kidney like
Legionella, leptospirosis and streptococcal disease. Our case was very similar to the other reported cases of AIN due to cocaine in that all have occurred
in middle-aged AA males and all have responded to steroids. This case
reminds clinicians to consider AIN in patients with AKI and a history of cocaine abuse.
320
A HEAVY HEART ON DIALYSIS
Baudy AJ, Ibie NC, Simon EE. Tulane Medical Center, New Orleans, LA.
Case Report:
Introduction: Pericarditis is the inflammation of the pericardium and was
first described by Richard Bright in 1836 in the context of uremic pericarditis
with renal failure. Since then two main types of pericarditis have been described: uremic pericarditis, which develops prior to renal replacement therapy or within 8 weeks of initiation of therapy; and dialysis-associated
pericarditis, which develops after being stabilized on dialysis. Our case
describes a patient who presented with dialysis-associated pericarditis with
large pericardial effusion after being on dialysis for several years.
Case Description: A 62 year old female with a past medical history of end
stage renal disease on dialysis for several years secondary to diabetes, and recent osteomyelitis presented to the hospital with complaints of shortness of
breath and wheezing that woke her up from sleep the morning of presentation. She states that she had orthopnea and PND, but denied any chest pain,
palpitations, nausea, vomiting, fever chills, or recent weight loss. Echocardiogram revealed findings of a large pericardial effusion. Of note, records
from the patient’s dialysis unit showed that patient has been showing up in
her dialysis regularly, but signs off after about 2 hours of dialysis. Dialysisassociated pericarditis was diagnosed and dialysis was intensified. The patient was dialyzed daily for ten days with no improvement in clinical status
or pericardial effusion. After extensive discussions between specialties, an
anterior pericardial window was later done and the patient clinically improved. More importantly her pericardial effusion resolved and she was
discharged to long term care facility.
Discussion: Dialysis-associated pericarditis occurs in 2-21% of patients on
dialysis and management is different between this condition and uremic pericarditis. Both conditions can lead to increased morbidity and mortality in
end-stage renal disease patients.It is very important for physicians to recognize to two conditions in order to prevent complications that may arise from
delaying proper care. The current evidence supports earlier surgical intervention in patients with dialysis-associated pericarditis with large pericardial
effusions, whereas patients with uremic pericarditis tend to respond to intensified dialysis.
321
SYNTHETIC CANNABINOIDS AND ACUTE KIDNEY INJURY
Berry JD, Srisung W, Prabhakar S. Texas Tech University Health Sciences
Center, Lubbock, TX.
Purpose of Study: The use of cannabinoids is on the increase in the community especially in the face of changes in state regulations. The use of several
synthetic cannabinoids (SCs) has also been on the rise but the full spectrum
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
of their adverse effects is unknown. Most reports of adverse events related to
SCs have been neurologic, cardiovascular, or sympathomimetic. We describe
here a case of acute kidney injury as a result of “Spice”, a popular synthetic
cannabinoid.
Methods Used: Case report with review of literature.
Summary of Results: A 31-year-old Hispanic male with no history of previous medical illness except for hypertension for a few years, was admitted
from the emergency room two days after a physical assault due to worsening
renal function. No external or bony injuries were detected. The patient admitted to abusing IV methamphetamine and smoking “Spice”, synthetic marijuana, a SC daily. On admission, patient was oliguric with his serum
creatinine was 2.6 mg/dL, and his BUN was 26 mg/dL. Urine analysis was
unremarkable except for trace protein and was negative for blood or hyaline
casts. The following day, the patient’s serum creatinine rose to 3.0 mg/dL,
and his BUN was measured to be 24 mg/dL. The patient had no prior history
of kidney injury or disease. An ultrasound of abdomen showed slightly enlarged kidneys with increased echogenicity and no hydronephrosis or mass.
From day 3, the urine output began to increase with gradual decline in serum
creatinine levels. On day 6, the Scr was 1.4 mg/dL and BUN had dropped to
18 mg/dL. A week later patient was found be asymptomatic with with return
of normal renal function. The course and recovery were compatible with
acute tubular necrosis (ATN).
Conclusions: Although a definitive association between the use of synthetic
cannabinoids and acute kidney injury has not yet been established, a causal
link has been proposed previously by the Center for Disease Control
[http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6206a1.htm, Feb 2013]
and in a recently published study [Clin J Am Soc Nephrol 8, 523526,2013]. Renal biopsies done in such cases (3 out of 4) revealed ATN.
The clinical course of our patient with history of SCs abuse together with recently published reports warrants more awareness of this clinical syndrome
in the medical community.
322
SEVERE HYPOCALCEMIA DUE TO DENOSUMAB IN
METASTATIC PROSTATE CANCER
Bhutta U1,2, Iqbal T2, Haragsim L1. 1OUHSC, Oklahoma City, OK and
2
OUHSC, Oklahoma City, OK.
Case Report: Denosumab, a human monoclonal antibody that neutralizes
receptor activator of NF-kB ligand (RANKL) has been shown to decrease
the incidence of skeletal-related events in metastatic bone disease. We report
a case of severe hypocalcemia following a single dose of Denosumab.
We were asked to see a 45-year-old gentleman with a 3-year history of
metastatic (bone, liver & lymph nodes) prostate cancer for which he had
been treated with androgen deprivation therapy in the past, along with
zoledronic acid for bone metastases.
He had issues with the zoledronate, mainly pains & he was switched to
Denosumab. He was seen in the ER a week after his first Denosumab injection
for worsening leg swelling and hematuria. His calcium level was found to be
low at 0.65 mmol/L and he was admitted to the hospital for IV and PO calcium
supplementation. On physical exam, Chvostek and Trousseau’s signs were negative and the EKG was normal. His vitamin D levels were low and PTH was inappropriately normal. He had not been on vitamin D supplementation at the
time of admission. He was started on ergocalciferol, calcitriol and high doses
of IV and PO calcium for around 12-13 days but his calcium levels never came
up to more than 1 mmol/L. He also developed severe hydronephrosis due to his
underlying malignancy and his renal function kept worsening. It was decided to
start him on dialysis for his renal failure and persistent severe hypocalcemia. His
calcium levels finally normalized after a few sessions of dialysis and he was sent
home on outpatient dialysis since he did not want placement of nephrostomy
tubes for his hydronephrosis.
Denosumab use has been associated with mild hypocalcemia in clinical
trial but not to the extent that was seen in our patient. Our patient had been
treated with bisphosphonate as well as Denosumab, and had osteoblastic
mets, all of which are risk factors for hypocalcemia. To prevent the cardiac
and neurological complications associated with hypocalcemia it has been advised to check and supplement vitamin D levels, and periodically monitor
calcium levels prior to starting therapy.
Southern Regional Meeting Abstracts
323
RITUXIMAB FOR HEPATITIS C - ASSOCIATED
CRYOGLOBULINEMIA
Desai J1,2, Saith S1,2, Afzal U1,2, Diamond M1,2, Nahman NS1,2. 1Georgia
Regents University, Augusta, GA and 2Charlie Norwood Veterens Association Center, Augusta, GA.
Case Report: Case Presentation: A 56 year old white male presented with
worsening edema for 3 months and orthopnea and pigmented patches on
both lower extremities for 1 year. 12 years prior, he was diagnosed with hepatitis C (HCV), cryoglobulinemia, biopsy proven MPGN and treated with cyclophosphamide and prednisone. The creatinine over the next several years
was reportedly 1.1 - 1.4 mg/dl. 7 years prior to this evaluation he developed
purpura and skin ulcers. His HCV viral titers were increased and he was
treated with ribavirin and interferon for 6 weeks, followed by peginterferon
for 48 weeks. At the most recent visit, creatinine was 2.7 mg/dl, 6 gm proteinuria, C3 low at 81 mg/dl and C4 low at 1 mg/dl. Rheumatoid factor
was 20 times normal at 234, and cryoglobulins were present. Repeat renal biopsy reportedly showed type I MPGN with possible microtubular structures
consistent with cryoglobulins. He was treated with plasmapheresis (PP) and
prednisone over the next several months with recurrent symptoms whenever
PP was stopped. HCV treatment was restarted but was stopped due to renal
impairment. Rituximab 600 mg weekly was started when serum creatinine
was 2 and proteinuria of 7 gm/day. The course was complicated by transient
thrombocytopenia but 8 weeks following 4 doses of rituximab the creatinine
was 1.3 mg/dl, RF normal, cryogloblulins were not present.
Discussion: This patient had HCV-associated cryoglobulinemia with biopsy
proven type 1 MPGN. His disease was resistant to prednisone and PP.
Rituximab, a monoclonal antibody to CD 20 present on B cells, was started
and followed by recovery of renal function sufficient to allow continued therapy for HCV. His course was complicated by thrombocytopenia which may
have been due to his underlying HCV versus a side effect of rituximab. This
case was similar to a few recent reports on the use of rituximab in resistant
HCV- associated cryoglobulinemia. There have also been some small studies
suggesting rituximab may be more efficacious than conventional therapy for
cryoglobulinemia.
Conclusions: Rituximab is effective and safe in treating HCV-associated
cryogloblulinemia and should be considered in patients resistant to prednisone and PP.
324
SIADH SECONDARY TO INTRACRANIAL PATHOLOGY:
FLUID RESTRICTION MAY NOT CUT IT
DiSalvo N, Engel LS, Martinez J. LSU Health Sciences Center, New Orleans, LA.
Case Report: A 45 year old male with a history of lung adenocarcinoma
with metastases to the brain and L3-L5 and C5 vertebrae presented to emergency department with a two day history bizarre behavior and increased agitation. He had received whole brain radiation 5 months prior as well as
chemotherapy with carboplatin, pemetrexed, cisplatin, and temozolamide
started 3 months ago. On exam he was moving all extremities continuously
without purpose and mumbling incoherent sounds. He was found to have a
serum sodium level of 125mmol/l, which was 135mmol/l 3 weeks prior. After about 1 liter of fluids from antibiotics and various medications his sodium
decreased to 120mmol/l. Strict fluid restriction was initiated and his sodium
responded minimally to 122mmol/l. His neurological exam remained unchanged. After 100cc and two 50cc boluses of 3% normal saline his sodium
rose to 128mmol/l over the next 24 hours and his symptoms completely resolved. With continued fluid restriction his sodium and neurological status
normalized.
Discussion: The mainstay of treatment for most patients with SIADH is fluid
restriction. However, this treatment plan is frequently ineffective in raising
sodium levels when patients have severe symptoms and intracranial disease.
Lack of well controlled studies comparing therapies for symptomatic hypontremia make us rely on case series and expert opinion to determine therapeutic plan. Based on current recommendations, hypertonic saline should be
reserved for previously well individuals who are symptomatic with seizures,
coma, or new focal neurologic findings and whose serum sodium levels are
less than 120 mEq/L.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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325
EARLY LINEZOLID-INDUCED LACTIC ACIDOSIS IN END
STAGE RENAL DISEASE PATIENT ON STATIN THERAPY
Gharaibeh K, Brewer M, Sandhu N, Juncos L. University of Mississippi
Medical Center, Jackson, MS.
Case Report: Lactic acidosis (LA) may be caused by drug-induced impairment of cellular respiration. Linezolid is used for bacterial infections due to
gram-positive cocci, including strains resistant to methicillin and vancomycin.
Statins are commonly used lipid-lowering agents. We present a case of significant LA secondary to linezolid toxicity in a patient with end-stage renal
disease (ESRD) while taking a statin.
A 32-year-old male with ESRD, systemic lupus erythromatous, Evan’s
Syndrome (autoimmune hemolytic anemia and idiopathic thrombocytopenic
purpura), and history of thrombocytopenia-induced mucosal bleeding
presented to our hospital complaining of a one-day history of hematochezia.
He was discharged 3 days earlier on linezolid for urinary Enterococcus
faecium. The remaining review of systems was negative. Vital signs were:
BP 115/65, pulse 98, afebrile. Physical exam revealed clear lungs, normal
abdominal exam, and no signs of hypoperfusion. Laboratory findings included bicarbonate 17 mmol/L, blood pH 7.37, and lactate 6.5 mmol/L.
The serum bicarbonate continued to trend downward and lactate continued
to trend upward, peaking at 9.9 mmol/L despite no signs of hypoperfusion
or other obvious causes of LA and continued high-bicarbonate hemodialysis.
Upon review of medications, the patient was noted to have received linezolid
for nearly one week in addition to long-term simvastatin therapy, both of
which have been separately reported to induce mitochondrial dysfunction.
The decision was made to discontinue both medications in favor of alternative treatment as linezolid could induce asymptomatic LA. After 48 hours
following discontinuation, the patient’s lactate level started to trend downward and normalized after 5 days.
LA is a toxic effect reported with prolonged linezolid therapy and is purported to be caused by inhibition of mitochondrial protein synthesis. Our
patient presented with early linezolid-induced LA likely secondary to decreased renal clearance while also receiving simvastatin, which is reported
to inhibit mitochondrial function that could accentuate mitochondrial toxicity leading to significant LA. Concluding that administration of linezolid
and statin simultaneously could exaggerate mitochondrial dysfunction leading to rapid LA in the presence of risk factors.
326
TUNNELED DIALYSIS CATHETER REMOVALS BY
NON-INTERVENTIONAL NEPHROLOGISTS:
THE UNIVERSITY OF MISSISSIPPI EXPERIENCE
Gharaibeh K1, Csongrádi 1,2, Saleh N1, Dreisbach A1, Tapolyai M3, Fülöp T1.
1
University of Mississippi Medical Center, Jackson, MS; 2University of Debrecen,
Debrecen, Hungary and 3University of South Carolina, Columbia, SC.
Purpose of Study: Tunneled (or cuffed) hemodialysis catheters (TDC) have
been in use since 1987. The prevalence of TDC use in the United States is
24%, the vast majority (83%) of patients with end-stage renal disease
(ESRD) in the United States initiate hemodialysis with a TDC. Bedside removal of TDC by non-interventional Nephrologist is not commonly performed or studied.
Methods Used: We performed a retrospective cohort review of our mixed in
and outpatient experience at the University of Mississippi Medical Center
between January 2010 and June of 2013. We collected data on patients and
procedure-related variables, success and complications rates. The study
was reviewed and approved by the University of Mississippi Human Research Office and data analyzed with SPSS v.19.
Summary of Results: Of the 138 subjects, mean age was 50 (+/−15.9) years,
49.3% were female, 88.2% African-American and 41% diabetics. Site of removal were the right internal jugular (IJ) in 75.4%, left IJ 14.5% and femoral
vein 10.2%; 45.7% took place in an outpatient setting. Main indications at
the time of removal were proven bacteremia in 32.8%, clinical septic or
infected state in 14.6%, along with “TDC no longer necessary” in 55.8%
due to either recovery of renal function or maturation of permanent dialysis
access. Trainees under supervision removed 78 (56.5%) of the TDCs; of
these, 5 cases (6.4%) needed hands-on assistance from Attending. All
504
removal has been technically successful and well tolerated without TDC tear
or soft tissue injury (bleeding) but we observed Dacron “cuff ” separation
and subcutaneous retention in 6.5% cases. Most of the outpatient removals
took place due to access maturations (p<0.0001). There was a significant association between outpatient removal and cuff retention (p=0.007) but not
with level of training or site of removal.
Conclusions: In this relatively large cohort of mixed inpatient and outpatient
bedside TDC removals, the procedure was well-tolerated with minimal complication rate.
327
PARTIAL BOWEL OBSTRUCTION IN
PERITONEAL DIALYSIS: THINK ENCAPSULATING
PERITONEAL SCLEROSIS
Grewal RK, Krane K, Paramesh A. Tulane School of Medicine, New
Orleans, LA.
Purpose of Study: Introduction: Encapsulating Peritoneal Sclerosis (EPS) is
a rare yet serious cause of peritoneal dialysis (PD) failure. The diagnosis is
based on clinical suspicion of clinical findings and confirmed with radiological and/or pathological findings. We describe a case of EPS in a PD patient
without sign of inflammation or radiological findings, diagnosed at laparoscopy and biopsy findings.
Methods Used: Not applicable.
Summary of Results: Case: A 56 year old male with a history of end stage
renal disease (ESRD) due to diabetic nephropathy on peritoneal dialysis
presented to an outside hospital with generalized abdominal pain and nausea.
The patient denied fever, chills, vomiting, diarrhea or dizziness, but had developed abdominal discomfort and inability to drain his peritoneal fluid. He
was diagnosed with partial small bowel obstruction based on clinical exam
and a CT scan. His symptoms did not improve with nasogastric suction
and he was transferred to Tulane Medical Center. At this time, his physical
examination was significant only for generalized abdominal tenderness with
a clean PD catheter exit site. Though PD fluid cell count showed: WBC of
4177/mm3, RBC of 13713/mm3 and a culture was negative, the catheter
would not drain any fluid. The CT scan was reviewed which confirmed the
findings of partial small bowel obstruction without any peritoneal enhancement or calcifications. Because of the non-functioning catheter, the patient
underwent laparoscopy which showed significant adhesions and dense thickened peritoneal cavity consistent with EPS. The PD catheter was removed
and hemodialysis was begun.
Conclusions: Though EPS is not a common condition, it should always be
part of the differential diagnosis in patients on PD presenting with symptoms
or signs of small bowel obstruction. In particular, this case illustrates the importance of clinical symptoms in patients on PD in considering a diagnosis
of EPS.
328
SHE IS ALL “DRESSED” UP: A CASE OF ALLOPURINOL
DEADLY COMPLICATION
Ibie NC Alper AB. Tulane University School of Medicine, New Orleans, LA.
Purpose of Study: 1) To help with early diagnosis of Drug reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome as a complication of
Allopurinol
2) To increase awareness and prompt initiation of appropriate management
for DRESS syndrome
Methods Used: NA.
Summary of Results: A 45-year-old woman with history of chronic kidney
disease stage 4, most recent creatinine of 2.7 mg/dL, hypertension, and recent
diagnosis of gout presented with a diffuse rash for 4 days starting from her
low back and progressing to include her entire body. The patient had been
started on allopurinol 100 mg daily 2 months prior. The patient’s development
of erythroderma, fever, facial swelling, acute kidney injury (Cr. 8.9 mg/dL), elevation in liver enzymes (AST 183 units/L; ALT 143 units/L; Alkaline phosphatase 91 units/L), and marked peripheral eosinophilia (Eosinophils 15.4%)
were consistent with a diagnosis of DRESS syndrome. Steroids were started
for the treatment of DRESS and allopurinol was held. The punch biopsy was
done which was consistent with a diagnosis of DRESS syndrome. The patient
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
was initially treated with 40 mg intravenous methylprednisolone every 8 hours
for 2 days and later switched to prednisone by mouth with improving clinical
symptoms. Laboratory values prior to discharge were- Eosinophils 1%; Creatinine 6.0 mg/dL; AST 145 units/L; ALT 173 units/L, Alkaline phosphatase
153 units/L. The patient was discharged with 40 mg of prednisone daily for
7 days, then 30 mg by mouth daily thereafter until her follow-up with dermatology. She presented back to the hospital 3 days after discharge with difficulty
swallowing and shortness of breath requiring intubation. Laboratory values
were mostly improved (Eosinophils 0%, Creatinine 3.8 mg/dL; AST 79 units/L;
ALT 153 units/L; Alkaline phosphatase 266 units/L). CT of the brain revealed
large intraparenchymal hemorrhage with subfalcine and uncal herniation. Care
was withdrawn per the family’s request the following day and the patient subsequently died.
Conclusions: Up to two percent of patients taking Allopurinol develop
DRESS. Though rare, DRESS syndrome is a side effect of allopurinol that
physicians should be aware of and about which they need to educate their
patients, prior to prescribing Allopurinol therapy, due to the significant mortality (10%) rate of this complication.
329
HEAD-TO-HEAD COMPARISON OF SAFETY PROFILES
OF INTRAVENOUS IRON SUCROSE, FERRIC GLUCONATE
AND TOTAL DOSE IRON DEXTRAN IN CHRONIC KIDNEY
DISEASE PATIENTS
Khan S1,2, Dossabhoy N1,2, Brignac2, Kallam A1,2. 1LSU Health Sciences
Centre, LA and 2Overton Brooks VA Medical Centre, Shreveport, LA.
Purpose of Study: There are multiple intravenous (IV) iron formulations
available today, but very few studies have made a head-to-head comparison.
The purpose of this pilot study is to compare the safety profiles of various IV
iron formulations in patients with chronic kidney disease (CKD) and iron deficiency anemia (IDA).
Methods Used: This is a retrospective chart review of patients receiving various IV iron formulations at a single center, the VA Medical Center,
Shreveport, LA. We included patients with IDA and CKD stages 3 and
higher for our analysis. As per prevailing clinical practice, K-DOQI criteria
guided the need and usage of IV iron in these CKD patients. Data was collected regarding patient demographics and comorbities, baseline renal and
hematological parameters, and adverse events noted with drug administration. Iron sucrose (IS) and ferric gluconate (FG) were given in doses used
routinely in clinical practice; whereas iron dextran (ID) at our center was
given as a total dose infusion (TDI) of 1000 mg administered IV over 4–6
hours (if negative reaction to an initial test dose).
Summary of Results: This pilot study examined the records of patients administered 609 doses of IV iron - 28% were white, 67% AA, 98% male, 49%
had DM, 73% had HTN; age 61+13 years. CKD stage 3 = 29%, stage 4 = 10%,
stage 5 = 59%; 33% were on dialysis. Baseline lab values: Creat 4.7+3.2 mg/dL;
eGFR 25+19 mL/min; Hgb 9.6+1.7 g/dL; iron 36+21 mcg/dL; T-sat 14+7%.
Adverse events (AE) were noted in 8 out of 261 administered doses
(3.1%) with ID - commonest being itching, chills and back pain. No AE
was noted with either IS or FG. No anaphylactic reaction was noted with any
product.
Conclusions: Iron dextran given as total dose infusion has a higher rate of
AE’s than routine doses of IS or FG. This may be explained by the much
higher dose used for ID; or by the relatively small sample size. However,
all of the AE’s were minor, and none were life-threatening.
* P ∼ 0.05 by chi-square test
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330
NORMAL KIDNEY PARAMETERS IN PATIENT WITH
RENAL FAILURE
Klomjit S, Hosiriluck N, Sutamtewagul G, Rassameehiran S, Phisitkul S.
Texas Tech Health Science Center, Lubbock, TX.
Case Report: Serum creatinine and urine output are long known to be
parameters for assessment of kidney injury. They have not been established
as the best parameters for kidney function evaluation.
75-year-old female with past medical history of dementia, hypertension,
and right nephrolithiasis, was admitted to the hospital for shortness of breath
and hypoxemia. She was diagnosed with aspiration pneumonia. During hospitalization, she was found to have progressive bilateral pleural effusion from
serial chest radiographs. Her urine output was adequate, ranging from 0.6 to
2 liters per day. Her daily fluid balance was positive about one liter every day.
Vital signs were within normal limit. Her weight was 45.5 kg. Physical examination was significant for decreased breath sound both lower lungs and
peripheral edema. Echocardiogram showed normal systolic function with
ejection fraction of 60-64% and normal diastolic function. Pleural fluid analysis
showed transudative profile. Serum creatinine was stable at 0.7-0.9 mg/dL. Estimated
glomerular filtration rate (eGFR) by MDRD formula was 97 ml/min/1.73m2.
Complete metabolic panel was unremarkable. Urinalysis was unremarkable.
Fractional excretion of sodium was 1.22% and urea was 48.35%. 24-hour urine
protein was 182 mg. Creatinine clearance was 11 ml/min. Serum cystatin C was
2.73 mg/L (normal values 0.5-1 mg/L). She was diagnosed with end stage kidney disease. Conventional hemodialysis was started. Her pleural effusion and
fluid retention reduced and she was discharged home with outpatient
hemodialysis.
This case illustrates common pitfall when physicians only rely on serum
creatinine and eGFR from MDRD formula. Patient underwent several therapeutic thoracocentesis for idiopathic pleural effusion before end stage kidney
disease was diagnosed. MDRD formula has limitation in low-body-weight patient to correctly assess renal function. Clinical vigilance is very important to diagnose renal failure in this case. Serum cystatin C may help, as it is not affected
by gender, age, race, protein intake, and muscle mass, unlike serum creatinine.
Calculated eGFR from CKD-EPI Cystatin C Equation is 18 ml/min/1.73m2.
The sensitivity and specificity of serum cystatin C to predict acute kidney injury
are 0.86 and 0.82.
331
OVARIAN SEROUS CARCINOMA: A RARE CAUSE OF
SYNDROME OF INAPPROPRIATE ANTIDIURETIC
HORMONE SECRETION
Klomjit S, Mankongpaisarnrung C, Dumrongmongcolgul N, Srisung W,
Gadwala S, Nugent K. Texas Tech Health Sciences Center, Lubbock, TX.
Case Report:
Background: The syndrome of inappropriate antidiuretic hormone secretion
(SIADH) associated with malignancy is relatively common. More than 75%
of these patients have small cell lung cancer. Ovarian tumors associated with
SIADH are extremely rare.
Case description: A 57-year-old postmenopausal woman presented with confusion and new-onset seizures. She had a past history of hypertension, hypothyroidism, and unknown gynecologic malignancy treated with transvaginal
hysterectomy 40 years ago. She was on hydrochlorothiazide and levothyroxine.
Physical examination revealed clinical euvolemia and no detectable focal neurological deficits. Admission laboratory showed serum Na 110 mmol/L, glucose 84 mg/dL, serum osmolality 234 mOsm/kg, uric acid 5.6 mg/dl, urine
sodium 147 mmol/L, and urine osmolality 442 mOsm/kg. Fractional excretions
of sodium and urea were 0.7% and 56%. Her serum cortisol and TSH were
within normal limits. Computed tomography (CT) of the head and chest was
unremarkable. CT of abdomen and pelvic showed bilateral complex adnexal
masses; CA-125 was 241 units/ml. An ovarian tumor in conjunction with
SIADH was diagnosed.
On admission, she was given hypertonic saline, and hydrochlorothiazide
was discontinued. Her serum sodium increased gradually to 115 mmol/L.
Her mental status was improved, but her serum sodium values remained
low. She then was treated with fluid restriction (1000 ml/day) and demeclocycline. Her serum sodium increased slowly to 125 mmol/L. She underwent surgery with bilateral ovarian mass removal, peritoneal washing, and
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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intra-abdominal tissue sampling. The pathology report showed serous carcinoma of ovaries with mesenteric metastasis.
Conclusion: SIADH is a common cause of hyponatremia. Malignancy-associated
SIADH needs to be excluded when patients present with severe hyponatremia,
even when a possible culprit medication, such as hydrochlorothiazide, is present. Pulmonary tumors, gastrointestinal tumors, and lymphoma are common
causes of malignancy-associated SIADH. Rare intra-abdominal tumors, particularly ovarian cancer, may need to be considered. Our literature review
identified three cases of ovarian teratoma and one case of ovarian serous
adenocarcinoma.
332
CASE OF ACUTE KIDNEY INJURY, LARGE KIDNEYS,
MEDIASTINAL LYMPHADENOPATHY DUE
TO IGG4RELATED
DISEASE- TUBULOINTERSTITIAL NEPHRITIS
Mohare K, Roberts S, Bodana S. Ochsner Clinic Foundation, New Orleans, LA.
Case Report:
Abstract: IgG4 related disease is a relatively new entity. IgG4RD is characterized by systemic involvement, dense infiltration of affected organs with
IgG4 positive plasma cells and characteristic storiform fibrosis on histopathology. Kidney involvement commonly causes tubulointerstitial nephritis,
shows good response to steroids and often has elevated IgG4 levels. As this
is a novel entity, it is still not highly suspected clinically, although once histologic diagnosis is established, it correlates clinically. Different measures are
ongoing to establish the diagnostic criteria.
A 50 year old African American man presented with acute kidney injury
on underlying chronic kidney disease. He had 40 lbs weight loss, fatigue,
shoulder pain, fever and altered behavior. On presentation he had creatinine
of 9, non-nephrotic range proteinuria, elevated total protein, mild hypercalcemia with a negative ANA screen. On imaging, kidneys were enlarged with
Right kidney at 14.8 cm and Left kidney at 13.7 cm. He also had mediastinal
lymphadenopathy. Serum ACE levels were normal. Clinically malignancy
was highly suspected. SPEP, UPEP were negative. A kidney biopsy was
performed which showed characteristic whorling dense fibrosis involving
90% of the tissue with polyclonal granular deposits in the tubular basement
membrane. Patient was started on systemic steroids and had a good response.
Discussion: IgG4 related disease is a recently recognized systemic immunemediated disease. IgG4-TIN is the most common renal manifestation which
can present as acute or chronic renal insufficiency, renal mass lesions, or both.
Histologically, plasma cell rich inflammatory infiltrate, storiform fibrosis, tubular basement membrane immune complex deposits are common. Other lesions
like membranous glomerulonephritis, IgA nephropathy, membranoproliferative
glomerulonephritis were not observed in the biopsy. IgG4 levels were not
obtained initially as the diagnosis was not suspected. Proposed diagnostic criteria for IgG4-TIN by Raissian et al and Japanese Society of Nephrology criteria
for IgG4-RKD will help diagnose more cases. As noted in this case, malignancy
is highly suspected at the time of presentation due to similarities in signs and
symptoms but biopsy helps solve the puzzle.
333
CASE OF ACUTE KIDNEY INJURY ALONG WITH SEVERE
THROMBOCYTOPENIA, HYPERBILIRUBINEMIA DUE TO
LEPTOSPIROSIS INFECTION IN NEW ORLEANS AREA
Mohare K, Bodana S, Blemur P. Ochsner Clinic Foundation, New Orleans, LA.
Case Report: 27 years old man without any significant past medical history
presented with acute illness. He had high grade fever associated with severe
myalgia, nausea, vomiting, headache and poor oral intake. During his almost
a week long illness, he developed jaundice, cough, rusty sputum production.
His admission labs were remarkable for creatinine of 4.5, Total bilirubin 22,
Direct bilirubin 15, normal liver enzymes, LDH 343. Remarkably he had serum potassium of 2.6. Also, of note he had severe thrombocytopenia of
22,000. He was still holding his blood pressure without any episode of hypotension. Peripheral smear was negative for schistocytes although clinically he
was strongly suspected to have thrombotic thrombocytopenic purpura. Urine
analysis was negative for any hematuria. Initially plasmapheresis was offered
till other results became available. However, peripheral smear continued to
506
remain clear of any schistocytes. ADAMTS13 assay came back normal.
Hence other etiologies were considered. Infectious disease work up was sent
for various possible differential diagnoses including Brucellosis, Rickettsial
disease, Ehrlichiosis, leptospirosis. Diagnosis was not very obvious as
thrombocytopenia was severe and is not classic feature of leptospirosis. Finally diagnosis became clear when serology for leptospirosis came back positive at 1:100.
Patient was a resident of New Orleans. He was a wildlife game inspector.
His work involved working in the marshy swamp areas with close contact
with various wild life creatures including alligators. Plus he had been hunting
ducks and fishing in Buras, LA the week prior to this presentation. This also
involved wading in the pond water.
Discussion: Leptospirosis is a zoonosis with protean clinical manifestations
caused by pathogenic spirochetes of the genus Leptospira. Here it was considered as one of the differential diagnoses however severe thrombocytopenia is not a typical feature of the disease. Severe hypokalemia could have
been from tubular dysfunction. Patient responded appropriately once started
on Doxycycline. Overall it is felt this diagnosis should be considered more
often in the appropriate setting as it can be potentially fatal. But with the correct use of antibiotic in time mostly there is recovery.
334
UNEXPLAINED HYPOTENSION: A CASE OF
SEVERE HYPOCALCAEMIA
Paccione R, Khalique S, Nimkevych O, Fremin K, Soltani Z. LSU Health
Sciences Center, New Orleans, LA.
Case Report: Hypocalcaemia after thyroidectomy is a common finding. It
may be transient or a permanent post-operative complication if removal of
the parathyroid occurred incidentally during thyroidectomy. Hypocalcaemia
should be considered as a cause of hypotension in these patients. Here we
discuss a case of severe hypotension secondary to hypocalcaemia.
Case Report: A 72 year old Caucasian female with a history of hypertension, hypothyroidism status post thyroidectomy two months prior, chronic alcohol abuse and depression was found down in her home. On physical exam
vitals: HR 96 bpm, respirations 21 breaths/min on mechanical ventilation,
BP 71/31 mmHg, temperature 98.6 F. Of note, she was sedated at the time
of the physical exam, but bilateral upper extremity twitching was present.
The remainder of the physical exam was unremarkable. Pertinent laboratory
findings included ionized Ca 0.65 mmol/L, calcium 3.6 mg/dl, magnesium
1.6 mg/dl, phosphorus 10.2 mg/dl, potassium 2.5 mmol/L, BUN 68 mg/dl,
creatinine 5.3 mg/dl with baseline normal kidney function. PTH <5.0 pg/ml,
cortisol WNL and the remainder of the work up was normal. Blood and urine
cultures were negative. Due to profound hypocalcaemia, the patient was placed
on continuous renal replacement therapy. After three days, the calcium normalized and the patient no longer required vasopressor support. Her mental status
and kidney function improved. The patient was sent home off dialysis and on
vitamin D and calcium supplements.
Discussion: Hypocalcaemia following near total of total thyroidectomy is a
common complication. It can be a result of a disruption of the blood supply
to the parathyroid gland or total removal of the parathyroid. The hypocalcaemia can range from asymptomatic to severely symptomatic. Hypocalcaemia traditionally manifests as tetany, seizures, altered mental status and/or
arrhythmias. However, it can also present as refractory hypotension, as was
the case in our patient. Once traditional calcium supplementation has been
implemented and fails to adequately replace the calcium, dialysis should be
considered.
Conclusion: Hypocalcaemia should be considered among the differential as
the cause of severe hypotension.
335
DEVELOPMENT OF A CLINICAL PRACTICE GUIDELINE
FOR VANCOMYCIN DOSING AND MONITORING
Palm S1, Belt E1, DeLeon S1, Balan A2, Hagemann T3. 1University of Oklahoma
Health Sciences Center, Oklahoma City, OK; 2University of Okahoma Health
Sciences Center, Oklahoma City, OK and 3University of Okahoma Health
Sciences Center, Oklahoma City, OK.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: Over the course of a 2-month period in the summer of
2012 at The Children’s Hospital at OU Medical Center (TCH), three patients
receiving vancomycin experienced drug-induced acute kidney injury (AKI).
Subsequently, a chart review of patients on vancomycin at TCH revealed inconsistent dosing and laboratory monitoring of levels and renal function.
Vancomycin-induced AKI is becoming an important topic on a national scale
due to increasing trough goals, although no official pediatric dosing or
monitoring guidelines were found when contacting other major children’s
hospitals. This project aims to develop a clinical practice guideline to assist
medical care providers and pharmacists in making decisions regarding
vancomycin dosing and monitoring to prevent drug-induced AKI.
Methods Used: A multidisciplinary team was assembled, including General
Pediatrics and Infectious Disease faculty, pediatric residents, clinical pharmacy, hospital nursing, laboratory staff, and TCH’s Senior Quality Coordinator. Collaboratively we established cost effective dosing and vancomycin
trough/serum creatinine monitoring guidelines, which should either eliminate
or allow early identification of patients with vancomycin-induced AKI. The
guidelines include monitoring for patients receiving additional nephrotoxic
medications and for pharmacy or infectious disease consults to guide physicians
in appropriate dosing and monitoring in more complicated cases.
Summary of Results: The Clinical Practice Guidelines are currently being
distributed to physicians, residents, nurses, and pharmacists via email, educational meetings, and postings in work areas. A comparison study to evaluate the incidence of drug-induced AKI before and after the implementation
of the guidelines will then be completed.
Conclusions: With close, consistent monitoring of vancomycin troughs and
serum creatinine in patients on vancomycin, the incidence of AKI should significantly decrease after guideline implementation. As vancomycin is not the
only nephrotoxic drug used regularly, an expansion of AKI prevention is also
in development process.
336
METABOLIC HEALTH MODIFIES THE ASSOCIATION
OF BODY MASS INDEX AND RISK OF END-STAGE
RENAL DISEASE
Panwar B1, Hanks LJ1, Tanner RM1, Muntner P1, Kramer H2, McClellan W3,
Warnock DG1, Judd SE1, Gutiérrez OM1. 1University of Alabama,
Birmingham, AL; 2Loyola, Chicago, IL and 3Emory, Atlanta, GA.
Purpose of Study: Obesity has been associated with poor outcomes in the
general population. However, recent studies have demonstrated that the association of body mass index (BMI) with outcomes depends in part on clustering of metabolic risk factors. We examined whether metabolic health
modifies the association of BMI with end-stage renal disease (ESRD) within
the REGARDS study, a cohort of 30,239 black and white adults.
Methods Used: The primary exposures were BMI and metabolic health. Metabolic health was defined according to the harmonized criteria for metabolic
syndrome - persons with ≥3 metabolic syndrome factors were categorized as
metabolically unhealthy and those with <3 as metabolically healthy. The outcome was ESRD. Cox models were used to estimate the hazard ratio of ESRD
as a function of BMI and metabolic health, separately. We tested for effect modification of metabolic health on the association between BMI and ESRD.
Summary of Results: We excluded participants with missing data, who did
not fast for the study visit, or were on dialysis, leaving 21,840 persons in analyzed sample. 218 participants developed ESRD over <6 years of follow-up.
Metabolic health modified the association of BMI with incident ESRD
(P-interaction=0.004). When we stratified the analysis according to categories of weight and metabolic subtype, as compared to metabolically
healthy normal weight persons, higher BMI was associated with lower risk
of ESRD in those who were metabolically healthy, whereas higher BMI
was associated with higher risk of ESRD in those who were metabolically
Hazard ratios (95% confidence intervals) of incident ESRD by weight and metabolic subtype
categories
Southern Regional Meeting Abstracts
unhealthy in analyses adjusted for demographic variables, lifestyle factors,
and co-morbidities. (Table).
Conclusions: Metabolic risk factors modify the association of BMI with risk
of incident ESRD. BMI alone is a poor marker of future risk of ESRD. Elevated BMI may be protective against the development of ESRD among metabolically healthy individuals.
337
CALCIPHYLAXIS IN END STAGE RENAL
DISEASE PATIENTS FROM THE UNITED STATES
RENAL DATA SYSTEM
Saith S1,2, Huber L1,2, Nahman NS1,2, Kheda M1,2, Baer S1,2, Colombo R1,2,
Kintziger K1. 1Georgia Regents University, Augusta, GA and 2Charlie
Norwood VA Medical Center, Augusta, GA.
Purpose of Study: Calciphylaxis (CPX) is a rare condition usually seen in
ESRD patients and is associated with significant mortality. It is characterized
by subcutaneous vascular calcification and cutaneous necrosis. Its etiology is
thought to be multifactorial but unclear. Given its low prevalence, few studies are available investigating CPX. Our study queried the United States Renal Data System (USRDS) to investigate the co-morbidities and outcomes
associated with CPX.
Methods Used: All prevalent ESRD patients in the USRDS were queried.
CPX was defined using the ICD-9 diagnosis code 275.49 (other disorders of
calcium metabolism) plus at least one of 7 additional relevant diagnosis codes
identifying skin lesions common to the disease (i.e. nodules, ulcers, skin necrosis, etc) during the same inpatient visit as the CPX diagnosis. Basic descriptive
statistics were used to assess frequencies and percentages for categorical
variables and mean and standard deviation (SD) for continuous variables.
Summary of Results: A total of 2,133,440 individuals with ESRD diagnosed between 1963 and 2009 were identified and 13,560,760 hospital
records from 1977 through 2008 analyzed. 459 (0.02%) patients met the criteria for CPX. For this group: mean age was 50.4 years (SD 15.7) with 20.3%
> 65 years, 71.0% female, 59.0% Caucasian and 37.3% African-American.
Treatment modality at the time of CPX diagnosis: center-based hemodialysis
(HD) (78.7%), peritoneal dialysis (14.8%) and transplant (2.4%). Comorbidities associated with CPX included obesity (79.1%), diabetes mellitus
(65.8%), coronary artery disease (47.7%), bacteremia (43.1%) and history of
parathyroidectomy (28.1%). The median survival time for individuals with
CPX was 1.1 years and 50% of the deaths were within 87 days of their CPX diagnosis. Most common causes of death were cardiac arrest (23.9%), septicemia
(20.6%), other identified causes (19.9%), withdrawal from dialysis/uremia
(5.7%) and MI (3.3%).
Conclusions: Although rare, CPX is associated with a high mortality. It is
more common in obese, diabetic, female and in-center HD patients. Cardiovascular disease and severe mineral bone disorders may also be present. Bacteremia is a common complication and may contribute to the high mortality.
338
PERSISTENT METABOLIC ACIDOSIS IN DIALYSIS PATIENT
AFTER UNINTENTIONAL SALICYLATE OVERDOSING
Schwartz DW. University of Tennessee, Memphis, TN.
Case Report: Metabolic acidosis is a common and important complication
of Chronic Kidney Disease and End Stage Renal Disease. Correcting the acidosis in CKD patients has been shown to slow progression of kidney disease. It must be monitored and managed appropriately. There are numerous
causes of both non anion gap and anion gap acidosis. We present a case
where a dialysis patient was found to have an anion gap acidosis of unknown
cause, that when resolved, persisted due to a second cause. Patient is a 63yo
M with h/o ESRD who had been having persistent diarrhea that had been refractory to medications. Patient has a h/o chronic pancreatic insufficiency
and has been on pancrelipase. Patient denied blood in stool, F/C, abdominal
pain. He was noted on monthly lab work to have a serum bicarbonate of 10
with an anion gap of 20. Pt was otherwise at baseline and the thought was the
diarrhea was causing the AG, and we did not believe he was taking heavy
amounts of salicyaltes or ingesting methanol. Patient relates on next HD session that he has been taking several packets of BC Powders every day. It was
explained to him that this contains too much aspirin to safely take. He also
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
507
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
noted mild tinnitus when questioned about it. Patient told to stop BC
powders and was indeed found to have elevated salicylate levels. Patient
was no longer taking BC powers but diarrhea has persisted. Regardless, the
patient continued to have a significant acidosis. Bicarbonate increased in HD
to 34, and he is told he cannot miss HD for now (he has a h/o of mild noncompliance). The acidosis persisted secondary to the diarrhea. A thorough
evaluation of acidosis is often required to come to the etiology, as there are
often several causes of an acid-base disorder present at the same time that
can cloud the evaluation. Preventing chronic acidosis in renal patients is important given the long-term complications of acidosis, such as decreased cardiac output and renal disease progression.
339
IV HYDRALAZINE FOR TREATMENT OF HOSPITALIZED
HYPERTENSIVE CHILDREN: HOW EFFECTIVE ?
Shatat I1, Ostrye J2, Jones J1, Hailpern S3, Egan B4, Chessman K2. 1MUSC
Children’s Hospital, Charleston, SC; 2Medical University of South Carolina,
Charleston, SC; 3Independent Consultant, Palo Alto, CA and 4MUSC,
Charleston, SC.
Purpose of Study: IV hydralazine is frequently used for treatment of hypertension in the hospitalized child. it’s safety and efficacy in treating hypertension in hospitalized children has not been examined.. The objective of this
study was to determine the efficacy and safety of the first dose of intravenous
hydralazine at the Medical University of South Carolina Children’s Hospital.
Methods Used: A retrospective chart review was conducted to evaluate the efficacy, safety, and appropriate use of intravenous hydralazine in hospitalized
children over a 3-year period. The patient population included children ages
birth to ∼ 18 years admitted to one of the non-critical care areas of the hospital.
patient’s blood pressure and heart rate prior to and up to 8 hours after each dose
was given were collected. Other admission-specific and patient demographic
data were also collected, along with documented adverse effects.
Summary of Results: 110 hospital admissions were included in this review.
The mean age was 8.65 years, 57.3% were male, and 35.5% were white. Pre
dose systolic and diastolic BPs were 146.4 and 92.0, respectively. Almost
one-third (30.0%) of patients received an inappropriate dose of hydralazine
(∼ 0.1 mg per kg per dose). All patients received intravenous hydralazine appropriately. Only 8 patients (7.3%) achieved BP control after one hydralazine
dose, with 9.1% of patients having documented adverse effects. In a multivariate regression models examining percent change in systolic and diastolic
BP, younger age, appropriate dose, and receiving two or more concurrent antihypertensive agents were significantly associated with decreases in diastolic, but not systolic BP.
Conclusions: In hospitalized children, younger age, appropriate dose, and
receiving hydralazine in addition to chronic hypertensive management is associated with a significant percent decrease in diastolic, but not systolic BP.
Intravenous hydralazine is well tolerated in children.
340
A RARE CASE OF LOW-SOLUTE HYPONATREMIA
IN A NON-ALCOHOLIC: A CASE REPORT AND
LITERATURE REVIEW
Srisung W, Mankongpaisarnrung C, Anaele C, Dumrongmongcolgul N,
Ahmed V. Texas Tech University Health Sciences Center, Lubbock, TX.
Case Report: Euvolemic hyponatremia poses a diagnostic challenge to
physicians. Among this group, low-solute hyponatremia is relatively uncommon. Classic cases were described in alcoholics as beer potomania, which is
characterized by hyponatremia in the setting of low-solute intake and heavy
beer drinking. We report a case of low-solute hyponatremia in a non-alcoholic
who was given solute load, and subsequently had excessive diuresis with resultant rapid increase in serum Na.
A 69-year-old African American man with HTN, seizure disorder but no
underlying psychiatric diagnoses presented with confusion and urinary incontinence. He had a serum Na of 117 mEq/L with urine osmolality of
132 mOsm/L and plasma osmolality of 250 mOsm/L. Urine Na was not
checked initially. He was given 1 L bolus of normal saline in the ER. Repeat
serum Na at 12 hr was 127 mEq/L. Urine output was 8 L in this first 12 hr.
5% Dextrose water was then started at 100 ml/hr. 15 hr after bolus, his Na
508
decreased to 123 mEq/L, urine osmolality was 315 mOsm/L and urine Na
was 128 mEq/L. Further history obtained when he regained consciousness
revealed inability to maintain oral intake due to poor appetite for 3 months,
but he could still hydrate himself adequately. He was an occasional alcohol
drinker. He has history of hospitalizations due to hyponatremia of unclear
etiology. Na level was monitored and the rate of D5W was adjusted to keep
the rate of increase in Na to no more than 8–10 mEq/L in 24 hr and 18 mEq/L in
48 hr. He was finally discharged at serum Na of 130 without any neurologic
sequelae.
Our case highlights the importance of detection of low-solute hyponatremia.
Its clinical picture can mimic SIADH as urine osmolality may exceed 100 mOsm/L
and urine Na may exceed 40 mEq/L. However, low-solute hyponatremia will
typically cause significant diuresis and rapid increase in Na after solute load
as opposed to worsening serum Na in SIADH. Rapid correction of Na can predispose to osmotic demyelination syndrome. However, if over-correction
occurs, lowering the Na with D5W is crucial. Management includes fluid restriction in mild or asymptomatic patients and finite amounts of IV fluids in
symptomatic cases, and maintenance of the solute load with oral intake.
341
AN UNCOMMON CAUSE OF BILATERAL ENLARGED
KIDNEYS: CASE REPORT AND LITERATURE REVIEW
Srisung W1, Mankongpaisarnrung C1, Warraich I2, Sotello D1, Yarbrough S1,
Laski M1. 1Texas Tech University Health Sciences Center, Lubbock, TX and
2
Texas Tech University Health Sciences Center, Lubbock, TX.
Case Report: Metastasis to the kidney is the most common etiology of
intrarenal malignancy, but it remains uncommon (12.6% in malignancy of
epithelial origin). Lung cancer is the most common primary lesion (19.823.3%), but only 22.5% of lung cancer spreads to kidneys. It can present unilaterally (46%) or bilaterally (54%), and either as an isolated mass or infiltrative
disease. We report a case of squamous cell lung carcinoma causing bilateral infiltrative kidney metastasis.
A 67-year-old White woman presented with a 6-month history of chronic
productive cough, abdominal bloating, and weight loss. Diminished breath
sounds were noted at the right lung base. Basal metabolic panel revealed
Na 127 mEq/L, BUN 32 mg/dl, and creatinine 2.4 mg/dl. Chest x-ray
showed a right lower lobe cavitary lesion with air-fluid level. Computed tomography (CT) of the lungs showed 8.6x6.2 cm and 1.6x1.4 cm cavities in
the right lower lung, multiple pulmonary nodules, and right-sided pleural effusion. Abdominal CT scan showed right adrenal mass and sclerotic changes
of T12 and L2 bony metastases, bilateral enlarged kidneys (right 8.7x12.5
cm and left 8.1x13 cm) with multiple small hypodense lesions, and retroperitoneal lymphadenopathy. Urinalysis showed RBC >20 and WBC 5-10. The
24-hour urine protein was 310 mg. Renal ultrasound showed an infiltrating
process without hydronephrosis. Renal biopsy showed squamous carcinoma
cells among a normal appearing glomerulus and atrophic tubules. Stains
were negative for CK7 and CK20. Transbronchial biopsy revealed squamous
cell carcinoma. The definite diagnosis was thus squamous cell carcinoma of
the lung with infiltrative renal metastasis.
In this case, the microscopic hematuria, azotemia, and non-nephrotic
range proteinuria resulted from metastatic lung cancer that diffusely infiltrated both kidneys. Enlarged kidneys in patients with suspected cancer elsewhere should raise concern for metastasis. Kidney biopsy remains the gold
standard for diagnostic workup for renal metastasis.
342
THE LONG-TERM OUTCOME OF KETOCONAZOLE
AND TACROLIMUS CO-ADMINISTRATION IN
KIDNEY TRANSPLANT PATIENTS
Umber A. Tulane, Metairie, LA.
Purpose of Study: To report our experience of combined use of Ketoconazole
& Tacrolimus in kidney transplant recipients.
To study the effect of combination therapy on rate of rejection in Kidney
Transplant recipients.
Methods Used: From 2006 to 2012, there were 450 adult patients who received a primary kidney transplant in our center. Among them, ketoconazole
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
was given in 199 patients after transplant surgery and was continued for at
least 1 year or until graft failure (group 1), while 149 patients did not receive
any ketoconazole (group 2).
The combination of tacrolimus, mycophenolate and steroid was used as maintenance therapy. Basic demographic data were similar between the 2 groups.
Summary of Results: The cumulative incidence of biopsy-confirmed and
clinically-treated acute rejection over 5 years was significantly higher in
group 1 than in group 2 (34% vs. 18%, p<0.001). The 5-year Kaplan-Meier
estimated patient and graft survival were not statistically different between
the 2 groups. Multivariable logistic regression analyses identified ketoconazole usage as an independent risk of acute rejection (OR 2.35, 95% Cl
1.41-3.90; p=0.001). Delayed graft function (OR 2.1, 95% Cl 1.15-3.68;
p=0.015) and infectious complication (OR 1.8, 95% Cl 1.05-3.10;
p=0.031) were also found to be significant risks of acute rejection.
Conclusions: Our study suggests that co-administration of Ketoconazole
and tacrolimus is associated with significantly higher incidence of acute rejection in kidney transplant recipients.
343
TOO MUCH OF A GOOD THING: WHEN
PREGABALIN MAKES YOU JERK
Wang HT1, Yu S2, Smalligan RD1. 1Texas Tech Univ HSC, Amarillo, TX and
2
Texas Tech Univ SOP, Amarillo, TX.
Case Report: A 51yo woman on hemodialysis (HD) presented with severe
leg pain that prevented her from walking. She denied trauma, heavy lifting,
prolonged immobility or leg swelling. PMH: HTN, ESRD, IBS, GERD;
Meds: amlodipine, aspirin, clonidine, dicyclomine, hydralazine, metoprolol,
ranitidine, and sevelamer. FH neg, no smoking, alcohol or drugs. PE: T98,
BP 131/76, and O2 95% on RA; alert, cardiac, pulmonary, abdominal, and
neurologic exams normal except for weakness in the lower extremities with
preserved reflexes. Labs: c/w her ESRD and Ca and CPK were nl.
Course: After DVT was ruled out pregabalin was given for neuropathic pain.
Next day her pain and strength had improved so her dose was increased. The
third day she developed choreoathetosis but no nystagmus, tremor, Chvostek
or Trousseau’s sign. The pregabalin was stopped and benztropine started. HD
continued on schedule. The choreoathetosis resolved over 2 days and the
patient went home able to walk.
Discussion: Pain from peripheral neuropathy is a common complaint heard
by internists. While gabapentin was excellent for treating it, pregabalin was
later found to be even more effective. Pregabalin is an isomer of GABA
which binds to Ca channels in CNS regions and increases GABA transport.
It is minimally metabolized and is excreted 90% unchanged in the urine. Side
effects include edema, dizziness, somnolence, ataxia, headache, and fatigue.
Case reports have revealed other side effects including dysautonomia, dyskinesia, extrapyramidal syndrome, etc. Our patient had been stable on her
home meds for more than 3 months making them unlikely culprits. In addition, no other meds besides pregabalin were started prior to her choreoathetosis and it faded after stopping pregabalin. This is supportive of the
hypothesis that pregabalin was the cause. Pregabalin is usually dosed at
150 mg daily or divided bid or tid for neuropathy. However, dose adjustment
is recommended in renal impairment. For patients with a CrCl ∼ 15 ml/minute,
the recommended dose is 25-75 mg as a single daily dose. This renal adjustment was overlooked in our patient. This case reminds physicians to consider the possibility of choreoathetosis when using pregabalin and the
importance of renal dose adjustment, particularly in hemodialysis patients.
344
RISK FACTORS FOR DEATH FOLLOWING A DIAGNOSIS OF
SPINAL EPIDURAL ABSCESS IN HEMODIALYSIS PATIENTS
White J1, Jin C1, Colombo R1, Baer S1,2, Afzal U1, Kintziger K1, Kheda M1,
Huber L1, Chebrolu P1, Nahman NS1,2. 1Georgia Regents University,
Augusta, GA and 2Augusta VAMC, Augusta, GA.
Purpose of Study: In previous work we showed that spinal epidural abscess
(SEA) is a serious infectious comorbidity in hemodialysis (HD) patients
(JASN, abstract in press). SEA was significantly associated with bacteremia,
Southern Regional Meeting Abstracts
MRSA infection and hepatitis C, however, the risk factors for mortality following a diagnosis of SEA remained undefined. The present study addressed
these questions via queries of the United States Renal Data System
(USRDS).
Methods Used: All incident HD cases from the USRDS for calendar years
2005–2008 were queried for a diagnosis of SEA. Survival and risk factors associated with mortality were also assessed. ICD-9 diagnosis codes were used
to identify diagnoses. Cox regression models were used to determine the relative hazard of death associated with an SEA diagnosis compared to other
known risk factors for death in ESRD patients. We used bivariate and multivariable models to assess these associations. Using backwards elimination of
non-significant variables, a final model was obtained, and the hazards ratios
(HR) and 95% confidence intervals (CI) determined.
Summary of Results: For the 4-year period of study, SEA was identified in
660/355,084 (0.19%) patients. The HR and 95% CI for death from SEA was
HR 1.20, CI 1.085, 1.328. Demographic variables that conferred a significant
risk for death included age > 65 years (HR 2.269, CI 2.243, 2.295) and the
presence of a dialysis catheter (HR 1.884, CI 1.850, 1.918). The top 4
comorbid conditions significantly associated with mortality in SEA positive
patients included cirrhosis (HR 1.715, CI 1.667, 1.765), decubitus ulcer (HR
1.669, CI 1.640, 1.699), bacteremia (HR 1.407, CI 1.390, 1.425) and TPN
(HR 1.376, CI 1.326, 1.429). Other significant co-morbidities included pancytopenia, candidemia, hepatitis C infection and C. difficile colitis (HR
range: 1.363 - 1.145).
Conclusions: SEA in HD patients is associated with an increased risk of
death. Older age, the presence of dialysis catheters, cirrhosis, decubitus
ulcers, bacteremia, and TPN constitute the greatest risk factors for death
and thus necessitate a comprehensive approach to management.
345
IS THERE A ROLE FOR ECULIZUMAB IN THE TREATMENT
OF SEVERE SHIGA TOXIN ASSOCIATED HEMOLYTIC
UREMIC SYNDROME?
Yanik MV, Feig D. University of Alabama at Birmingham, Birmingham, AL.
Case Report: Background: Hemolytic uremic syndrome is a thrombotic
microangiopathy characterized by hemolytic anemia, thrombocytopenia,
and acute kidney injury. A majority of pediatric cases are associated with a
diarrheal illness caused by shiga toxin producing E coli (STEC-HUS). Recent evidence suggests that in addition to direct endothelial injury and inflammation, activation of the complement system may be an important
mechanism of organ injury in STEC-HUS. Standard treatment of STECHUS is supportive care. The use of eculizumab, a monoclonal antibody that
inhibits C5, for treatment of STEC-HUS remains controversial.
Case: Patient is a 6 year old Caucasian male with a severe and complicated
course of STEC-HUS. Initially admitted with bloody diarrhea and dehydration, he developed thrombocytopenia and acute kidney injury within 24 hours
of hospitalization. By hospital day 2 patient was anuric and dialysis was initated
on hospital day 5. He developed neurologic symptoms with altered mental status and seizures, followed by shock and respiratory failure. Given his rapidly
progressing course, patient was given a dose of eculizumab on hospital day 7.
He continued to clinically worsen. He had a cardiac arrest and required ECMO
for combined cardiac and respiratory failure on hospital days 9–20. He developed abdominal compartment syndrome and bowel infarction requiring colonic
resection. He also sustained a right temporal and parietal stroke. Although over
several months patient gradually improved and was able to be discharged, he
continues to have stage II chronic kidney disease, cognitive deficits, and mild
left hemiplegia a year later.
Discussion: In this severe case of STEC-HUS there was no benefit of the administration of eculizumab, as the patient continued to deteriorate clinically.
A case series in the New England Journal of Medicine reported on 3 patients
with severe STEC-HUS who were treated with eculizumab, all of which had
dramatic improvement within 24 hours of the first infusion. There are
conflicting reports on the effects of eculizumab use during the 2011 STECHUS outbreak in Germany.
Conclusion: Further studies, especially randomized controlled trials, are
needed to evaluate the potential efficacy of eculizumab in the treatment of
STEC-HUS.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
509
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Southern Society for Clinical Investigation and Southern American
Federation for Clinical Research
Plenary Session
SSCI Young Investigator Award Finalists
SAFMR/SSCI/ Young Faculty Award
SAFMR/SSCI/ Trainee Research Award
8:00 AM
Friday, February 21, 2014
346
RECOVERY OF SMALL MYOCYTES AND REVERSE
REMODELING IN HYPERTENSIVE HEART DISEASE
Al Darazi F, Zhao W, Zhao T, Sun Y, Marion TN, Ahokas RA, Bhattacharya SK,
Gerling I, Weber KT. University of Tennessee Health Science Center, Memphis, TN.
Purpose of Study: Hypertensive heart disease (HHD) includes hypertrophied cardiomyocytes and widely scattered foci of microscopic scarring,
which have replaced necrotic myocytes. Bordering on these microdomains
of fibrosis are small myocytes that re-express β-myosin heavy chain (βMHC), a fetal gene. We hypothesized small myocytes represent an autologous population of cells which could be recovered for reverse remodeling
in HHD.
Methods Used: 8-wk-old male Sprague-Dawley rats received aldosterone/
salt treatment (ALDOST) for 4 wks followed by a 4-wk period of assisted recovery when oral antioxidants (ZnSO4 and nebivolol, a β3 receptor agonist)
were given.
Summary of Results: Compared to untreated age-/sex-matched controls:
mean arterial pressure (MAP, 162±2 vs. 99±2 mmHg) and left ventricular
(LV) mass (1043±23 vs. 883±32 g) were increased (p<0.05) while pressureindependent scarring appeared in the right and left heart at 4 wks ALDOST.
Bordering on these scars were α-smooth muscle actin+ myofibroblasts (myoFb)
and small myocytes revealing: i) re-expression of β-MHC and atrial natriuretic
peptide (ANP); ii) increased expression of reactive oxygen species; and iii) reduced nitric oxide. Cardiac tissue 8-isoprostane and redox-sensitive ligases
(atrogin-1 and MuRF1) of the ubiquitin protease system were each increased
(p<0.05) while E/A ratio and LV fractional shortening were reduced (p<0.05).
Assisted recovery led to normalized MAP, regression in LV mass, and reduction
in small myocytes (p<0.05) with restored redox balance and NO, neutralized protein degradation, together with improved diastolic and systolic function.
Conclusions: HHD includes microdomains of fibrosis where small myocytes
re-express a fetal gene program together with altered redox balance and protein degradation. Recovery of these myocytes, using neurohormonal withdrawal plus a regimen of antioxidants, is feasible and leads to reverse remodeling
with improved ventricular function in HHD. Whether heterocellular signaling
with myoFb exists and the potential origin of these small myocytes as progeny
of bone marrow or cardiac stem cells vs. dedifferentiated myocytes remains to
be determined.
347
ROLE OF ASPERGILLUS FUMIGATUS SIALIDASE IN
DISRUPTION OF PULMONARY ARTERY AND PULMONARY
MICROVASCULAR ENDOTHELIA
McGrath B1, Cioffi E2, Cioffi D3, Fortwendel J1. 1University of South
Alabama College of Medicine, Mobile, AL; 2University of South Alabama
College of Medicine, Mobile, AL and 3University of South Alabama College
of Medicine, Mobile, AL.
Purpose of Study: Aspergillus fumigatus (Af) is the primary causative species of invasive aspergillosis (IA), a deadly disease that affects immunocompromised patients. A better understanding of the molecular mechanisms
regulating invasive growth of Af is essential to the development of novel antifungal therapies. Pulmonary artery (PA) and microvascular (MV) endothelial cells, which form important barriers to hyphal angioinvasion and
subsequent bodily dissemination during IA infection, have been shown to
express sialic acids that contribute to the integrity of cell-cell interactions.
The current study tested the hypothesis that a sialidase enzyme secreted by
Af contributes to pathogenesis by facilitating the disruption of these endothelial barriers.
510
Methods Used: To generate a mutant strain lacking sialidase activity, the
sole Af sialidase gene, siaA, was deleted by homologous recombination in
a uridine auxotrophic strain using Af protoplast transformation. Standard Aspergillus culture and microscopy techniques were employed to analyze the
effect of siaA deletion on germination, hyphal growth and morphogenesis.
To determine the impact of siaA deletion on host-pathogen interactions, electric cell-substrate impedance sensing (ECIS) was utilized to measure cell-cell
barrier disruption in the presence of Af conidia.
Summary of Results: Deletion of siaA caused delayed germination and a
statistically significant reduction in radial growth rate, as compared to wild
type. However, the ΔsiaA strain developed morphologically normal hyphae.
ECIS analyses indicated reduced cell-cell barrier disruptions for both PA and
MV cell lines when cultured with the ΔsiaA mutant.
Conclusions: Taken together, our data suggest that siaA contributes to the
timing of Af asexual development and to the disruption of endothelial
barriers. Future studies will further define the role of siaA as a potential virulence determinant by delineating the individual contributions of sialidase
activity to Af growth, endothelial barrier disruption and virulence in a murine model of invasive aspergillosis.
348
NOVEL PROTECTIVE EFFECT OF HISTONE DEACETYLASE
9 AS A REPRESSOR OF ANGIOTENSINOGEN EXPRESSION
IN THE FEMALE RAT KIDNEY
Bourgeois C1,2, Rui P3, Sato R1,2, Prieto MC1,2. 1Tulane University, New
Orleans, LA; 2Tulane University, New Orleans, LA and 3Tulane University,
New Orleans, LA.
Purpose of Study: Young females are protected from the development of hypertension and related end-organ damage compared to males. Inappropriate
activation of intrarenal renin-angiotensin system (RAS) is a major contributor to hypertension and renal injury. During intrarenal RAS activation,
upregulation of angiotensinogen (AGT) in renal proximal tubular cells
(PTC) serves as a key mechanism. Females exhibit lower excretion of AGT
in urine, suggesting that intrarenal AGT production in females may be a protective factor against the development of hypertension. However, whether
epigenetic factors influence intrarenal AGT expression levels leading to the
sex disparities has not been delineated. In this study we aimed to determine
the epigenetic factors controlling AGT expression in PTC.
Methods Used: AGT mRNA and protein levels in renal cortex of male and
female Sprague Dawley rats were measured by real time RT-PCR and Western blot analyses. Furthermore, we used a chromatin modification enzyme
PCR array to examine if epigenetic factors which repress gene transcriptions,
particularly histone deacetylases (HDAC) and co-repressors, are determinant
for the low AGT expression in the kidney of females. To clarify the relationship between HDAC9 and AGT, gene silencing of HDAC9 was performed in
the rat PTC.
Summary of Results: AGT mRNA and protein levels were significantly
lower in renal cortex of females than males (0.15 ± 0.01 in mRNA, 0.41 ±
.03 in protein, ratio to male). In the HDACs and co-repressors tested in this
study, only HDAC9 exhibited higher expression in renal cortex of females
than males (7.09± 0.88, ratio to male). Western blot and immunohistological
analyses using rat renal cortex and cultured rat PTC revealed HDAC9 is
expressed in PTC. HDAC9 with Knockdown by siRNA augmented AGT
mRNA (1.92 ± 0.35, ratio to control) and protein (2.25 ± 0.50, ratio to control) levels compared to scrambled siRNA transfected cells.
Conclusions: HDAC9 is a novel suppressing factor of AGT in PTC, helping
to explain the low levels of intrarenal AGT in females. These findings help to
delineate the mechanisms underlying sex disparities in hypertension and renal injury associated with dysregulation of RAS.
349
ROSIGLITAZONE INCREASES CHOLESTEROL EFFLUX
CAPACITY IN PATIENTS WITH TYPE 2 DIABETES
Agarwala A, Givens E, McGuire DK, De Lemos JA, Rohatgi A. UT Southwestern Medical Center, Dallas, TX.
Purpose of Study: Thiazolidinediones (TZDs) are medications used in patients
with type 2 diabetes (T2DM) that activate peroxisome proliferator- activated
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
receptors (PPAR) γ, thereby improving insulin sensitivity and glycemic control.
In most patients, treatment with TZDs increases HDL-C, suggesting that TZDs
may have effects on atherosclerosis via the reverse cholesterol transport (RCT)
pathway. A few small reports without placebo control have shown that TZDs
may increase macrophage-specific cholesterol efflux capacity (CEC), the first
critical step in RCT. We tested the hypothesis that TZDs, specifically
rosiglitazone (rosi), increase CEC in patients with T2DM in a placebo-controlled trial.
Methods Used: 111 patients (57 rosi, 56 placebo) with T2DM and either the
presence of coronary disease or strong risk factors for coronary disease were
randomized to double-blinded treatment with rosi vs. placebo for 6 months.
CEC was measured at baseline and after 6 months treatment.
Summary of Results: Preliminary results from the first 65 patients (30 rosi,
35 placebo) show increased mean CEC in patients randomized to rosi vs. placebo (ΔR: 0.064 ± 0.31; ΔP: -0.045 ± 0.26, p=0.03). Paired comparisons of
baseline vs. treatment show an increase in the rosi arm (0.87 ± 0.54 vs. 0.93 ±
0.51, p 0.005) but no change in the placebo arm (0.79 ± 0.51 vs. 0.74 ± 0.51,
p 0.83) (Figure 1).
Conclusions: These findings from a randomized placebo-controlled trial
suggest that activation of PPAR γ increased CEC and that the effect of TZDs
on atherosclerosis may be mediated by their effects on cholesterol efflux.
FIGURE 1. Normalized cholesterol efflux at baseline and after 6
months of treatment.
350
THE ROLE OF LEUKOCYTE ASSOCIATED
IMMUNOGLOBULIN-LIKE RECEPTOR-1 (LAIR-1) IN
SUPPRESSING COLLAGEN-INDUCED ARTHRITIS
Kim S1, Price L1, Cullins D1, Park J1, Stuart JM1,2, Kang A1, Myers L3,1.
1
University of Tennessee Health Science Center, Memphis, TN; 2Veterans
Affairs, Memphis, TN and 3Le Bonheur Children’s Hospital, Memphis, TN.
Purpose of Study: The success of Abatacept (CTLA4-Ig) in treating rheumatoid arthritis (RA) suggests that targeting T cells can be an effective therapeutic strategy for RA. A possible new therapeutic target might be the
inhibitory receptor Leukocyte Associated Immunoglobulin-Like Receptor-1
(LAIR-1) which is found on peripheral mononuclear cells, including NK
cells and T cells. Our hypothesis was that stimulation of LAIR-1 will suppress cell signaling and lead to inhibition of autoimmune arthritis in the
collagen-induced arthritis (CIA) model.
Methods Used: DR1 transgenic mice that were LAIR-1 -/- mice (n=7) were
immunized with type II collagen (CII) to induce arthritis and compared to
DR1 LAIR-1 +/+ littermate controls (n=13). Each mouse was scored three
times weekly beginning 2 weeks post immunization and the mean severity
scores and percentage of arthritic limbs were recorded at each time point.
Summary of Results: When CII-reactive murine T cells were cultured in
vitro with the immunodominant determinant of CII we found that type I
Southern Regional Meeting Abstracts
collagen could suppress IL-17 responses in the presence of LAIR-1. In vivo,
mice that were immunized demonstrated that the LAIR -1 -/- group developed more severe arthritis than the wild type mice (severity scores 4.3 ±
2.7 vs. 2.2 ± 1.4, p=0.03). In addition, LAIR -1 -/- mice had a greater percentage
of limbs affected with arthritis than did the wild type group (33 ± 21 vs. 17 ± 10,
p=0.02). Antibody titers (IgG) to CII correlated with arthritis severity.
Conclusions: We have demonstrated that LAIR-1 -/- /DR1 mice develop arthritis that is more severe, with more arthritic joints than DR1 mice alone.
These data suggest that LAIR-1 stimulation is involved in the modulation
of CIA and that stimulation of LAIR-1 may serve as an effective treatment
for RA.
351
HEALTHY HIV-INFECTED INDIVIDUALS HARBOR HIV IN
THEIR ALVEOLAR MACROPHAGES
Cribbs SK1, Lennox J2, Caliendo A4, Brown L3, Guidot D1. 1Emory University and the Atlanta VA, Atlanta, GA; 2Emory University, Atlanta, GA;
3
Emory University, Atlanta, GA and 4Brown University, Providence, RI.
Purpose of Study: Alveolar macrophages (AM) are specialized innate immune cells that reside in the alveolus, a separate compartment site compared
to blood. The half-life of these cells is significantly longer than that of an activated lymphocyte. AM can be infected by HIV-1 as they express CXCR4
and CCR5. However, reports quantifying the burden of HIV in AM are
conflicting, and it is unclear what impact HIV infection has on AM function.
We hypothesize that the AM is an important reservoir for HIV, contributing
to cell-cell spread of the virus within the lung and exhibiting abnormal immune function that contributes to the increased risk of severe lung infections.
Methods Used: Healthy HIV-infected subjects underwent bronchoscopy and
bronchoalveolar lavage. AM were isolated and HIV-1 RNA was quantified
using the Abbott RealTime HIV-1 PCR Assay (Abbott Molecular Inc. Des
Plaines, IL). Proviral DNA was qualitatively measured using a modified version of the above-mentioned HIV-1 RNA viral load assay. To measure phagocytic function, AM were isolated and incubated for 1 hour with FITC-labeled
S. aureus in a 10:1 ratio. FITC-labeled bacteria containing cells were measured by flow cytometry. Phagocytic index was calculated as follows: (%
positive cells x mean channel fluorescence)/100.
Summary of Results: We enrolled 22 otherwise healthy HIV-infected
subjects (median CD4 count=409/μl, 82% with undetectable plasma viral
load). HIV-1 proviral DNA and/or quantitative RNA were detected in 82%
of the AM; 78% of this group had an undetectable plasma viral load and
94% were on anti-retroviral therapy. HIV-1 RNA levels ranged from 48 to
2305 copies/mL. Further, HIV-infected subjects with proviral DNA present
had decreased AM phagocytosis of FITC-labeled S. Aureus compared to
subjects without proviral DNA present [16.6 (IQR 6-45.2) vs. 33.1
(IQR12.2-172.1)].
Conclusions: Alveolar macrophages harbor HIV even in otherwise healthy
subjects with undetectable plasma viral loads, representing a potential reservoir for the virus. In addition, HIV viral replication within these macrophages may impair phagocytosis and other immune functions, leading to
an increased risk for lung infections.
Southern Society for Pediatric Research
Plenary Session
Young Investigator Award Finalists
8:00 AM
Friday, February 21, 2014
352
IMPROVING DISASTER AWARENESS AND PREPAREDNESS
AMONG FAMILIES OF CHILDREN WITH SPECIAL
HEALTHCARE NEEDS
Bagwell H 1 , Liggin R 1 , Thompson T 1 , Lyle K 1 , Anthony A 1 , Baltz M 1 ,
Melguizo-Castro M 2 , Nick T 2 , Kuo D 3 . 1 UAMS - ACH, Little Rock, AR;
2
UAMS - ACH, Little Rock, AR and 3 UAMS - ACH, Little Rock, AR.
Purpose of Study: Children with special healthcare needs (CSHCN) may
present unique challenges for disaster preparedness, as some of these children require care such as home oxygen, multiple medications, or gastrostomy
feeds that could be disrupted during a disaster. The purpose of this study is to
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
determine the disaster preparedness of families of CSHCN and the impact of
disaster preparedness education and intervention. It is anticipated that such
families will be underprepared, demonstrated by not having an emergency
information form (EIF) for their child or a disaster kit.
Methods Used: Families of CSHCN were consecutively enrolled between
May and September of 2013 at the Medical Home Clinic of Arkansas
Children’s Hospital, a comprehensive outpatient service for children with
complex medical needs. Each family was given a 24 question literaturebased survey about disaster preparedness. Following the survey, families received: 1) educational handouts on disaster preparedness; 2) a $15 disaster
supply starter kit, a backpack containing first aid supplies and a flashlight.
A follow-up phone survey was conducted 6-10 weeks later to determine
changes in disaster preparedness. McNemar’s test was used to assess the
differences between pre- and post-survey items.
Summary of Results: There were 250 families enrolled with 144 completing the post-survey. The median age of children was 3.1 years with a median
of 4 required medications; 55% required gastrostomy tubes, 23% required
home oxygen therapy, and 35% required a feeding pump. At pre-survey,
43% of families had an EIF for their child, increasing to 79% at the post-survey
period (p-value <0.001). Only 18% of the participants in the pre-survey period
had a disaster kit. At post-survey 143 of 144 respondents (99%) still had the
starter disaster kit, and 44% had added items such as water, clothes, or additional first aid supplies.
Conclusions: Families of CSHCN are underprepared for a disaster, but a
small disaster supply starter kit with education may increase disaster awareness and planning. Further investigation looking at long term improvements
in disaster preparedness and dissemination to other populations is needed.
353
EFFECT OF COLON TRANSECTION ON
SPONTANEOUS HIGH-AMPLITUDE-PROPAGATING
CONTRACTIONS IN CHILDREN
Jacobs C1, Wolfson S2, Di Lorenzo C2, Cocjin J2, Monagas J2, Hyman P2,1.
1
Louisiana State University, New Orleans, LA and 2Children’s Hospital, New
Orleans, LA.
Purpose of Study: After Hirschsprung’s disease (HD) surgery, many children suffer fecal incontinence caused by increased high amplitude propagating contractions (HAPCs). The aim of this study was to determine whether
children with HD have more HAPCs than children with colon transections
for reasons other than HD.
Methods Used: We reviewed 500 colon manometries. Children (7.6±5.1 yrs;
275 male) with functional constipation (n=237; 7.4±5.0yrs; 126 male) and
chronic abdominal pain (n=48; 9.8±5.8yrs; 25 male) served as controls compared to subjects with HD (n=56; 6.9±4.1yrs; 44 male) and colon transection
for other reasons (n=24; 6.1±5.8yrs; 12 male). We excluded 139 subjects
without HAPCs. We documented HAPCs during 1 h fasting and 1 h postprandial. Results are mean ± SD.
Summary of Results: During fasting, HD subjects had more HAPCs (2.2
±3.4/h) vs. functional constipation (0.8±2.2/h, p=0.0004) and chronic pain
(0.5±1.1/h, p=0.001), but not more than colon transection (1.9±3.2/h, p=1.0).
HD showed more postprandial HAPCs (4.0±5.4/h) than functional constipation
(1.5±2.5/h, p<0.0001) and chronic pain (0.9±1.6/h, p<0.0001), but not more
than colon transection (2.4±3.0/h, p=0.6). There were more HAPCs fasting
and post-prandial after colon transection (1.9 ± 3.2/h and 2.4±3.0/h) than functional constipation (0.8±2.2/h, p=0.3 and 1.5±2.5/h, p=1.0) and chronic pain
(0.5±1.1/h, p=1.0 and 0.9±1.6, p=1.0). HD subjects were divided by chief complaint: fecal incontinence or constipation. HD subjects with incontinence
(n=23) only had more HAPCs fasting (p=0.01) and post-prandial (p=0.01) than
HD subjects with constipation (n=28) only.
Conclusions: Increased HAPCs followed colon transection, regardless of
cause. HD subjects with incontinence had more HAPCs than subjects with
colon transection for other reasons.
354
INCREASING ENTERAL PROTEIN INTAKE IMPROVES
GROWTH IN VERY LOW BIRTH WEIGHT (VLBW) INFANTS
Maxted A1, Talati AJ1,2, Skeen A2. 1UTHSC, Memphis, TN and 2The Regional Medical Center, Memphis, TN.
512
Purpose of Study: Preterm infants are at a disadvantage of lacking adequate
nutrition because of early separation from mother and difficulty in establishing adequate enteral intake after birth. Human milk (HM) is the best form
of feeding for a newborn, but it may not be completely adequate for growth
of VLBW infants. Several different additives including human milk fortifiers
(HMF), liquid protein (LP) and medium chain triglycerides are added to HM
to improve caloric intake and growth. We proposed to evaluate the role of
adding LP to HM feeding and its effect on growth of VLBW infants during
their NICU stay.
Methods Used: A retrospective chart review was done in the NICU after
identifying VLBW infants receiving HM with fortification and added LP.
A comparable cohort of VLBW infants receiving HM with fortification
was identified. Data were collected for a 4 wk period after the infants were
off intravenous nutrition. Daily weight and intake of protein and calories
were calculated. A z score was calculated comparing the weekly weight
change from start of study and the difference in z score each week was also
calculated. Weekly total protein, albumin and other parameters of growth
were also recorded for 4 consecutive wk and at the time of discharge.
Summary of Results: A total of 32 VLBW infants were studied. 15 VLBW
infants (group 1) received LP in addition to HM +HMF while 17 infants
(group 2) received only HM+HMF during the study period. The mean BW
(672±148 vs 766±268g), GA (25.1±1.24 vs 25.9±2.2wk) and birth head circumference (22.1±1.14 vs 23.2± 2cm) were similar in both groups. As expected
the estimated average daily protein intake was higher in LP (group 1), while caloric intake was similar. The mean growth velocity was higher in group 1 (24.3
±12.5g vs 13.1±8.6g /d, p<.05) at the end of 4 week period. The weekly
change in z-score for weight improved significantly in HM+HMF+LP group
of infants by week 4 (-0.26 vs -1.4, p<.05) and at discharge (0.18 vs -0.99,
p<.05). The serum BUN levels were similar between the groups each week.
Conclusions: Additional protein intake improves weight gain in VLBW
infants fed exclusively with fortified HM. Longer follow up is needed to
see the benefit on brain growth and neurodevelopmental outcomes.
355
P2Y2 PURINERGIC RECEPTOR FUNCTION IS ESSENTIAL
FOR THE PATHOGENESIS OF POLYMICROBIAL SEPSIS
Arunachalam A1, Tackett B2, Maynard J2, Thevananther S2. 1Baylor College
of Medicine, Houston, TX and 2Baylor College of Medicine, Houston, TX.
Purpose of Study: Severe sepsis is a major cause of morbidity and mortality.
Cellular stress triggers ATP release into extracellular milieu. Extracellular
ATP, via activation of P2Y2 purinergic receptors, plays a pivotal role in inflammation and immunomodulation. However, the functional significance
of P2Y2 receptor activation in the modulation of inflammation, multi organ
injury, and death is not well understood. The overall goal is to test the hypothesis that P2Y2 purinergic receptor activation is essential for the induction of inflammatory cascades and multi-organ injury secondary to microbial infection.
Methods Used: Sepsis was induced by cecal ligation and puncture (CLP) in
adult (12-16 weeks) wild type (WT; C57BL6/J) and P2Y2-/- (KO; C57BL6/J)
mice. Tissues (liver, lungs) and blood were collected at 3,6,12, 21h and 7 days
after CLP. Tissues were analyzed for leukocyte infiltration, apoptosis, and proinflammatory cytokine/chemokine protein and RNA (quantitative RT-PCR)
expression.
Summary of Results: WT mice subjected to CLP developed symptoms of
severe sepsis and were moribund (100%) between 21-48 hr. However, KO
mice survived longer (70% survival at 7 days). In response to CLP, induction
of serum cytokines/chemokines was significantly attenuated in KO mice (IL-6,
67%; IFNγ, 91%; MCP-1, 96%; MIP-2, 75%), as compared to WT mice at
21 hr. CLP induced massive liver injury in the WT mice, with elevated serum
ALT, leukocyte infiltration into the hepatic parenchyma, hepatocyte injury,
and hemorrhagic necrosis at 21 hr. Liver injury was attenuated in the KO mice
in response to CLP at 21 hr, with decreased induction of TNFα (58%), IL-6
(72%), IL-1β (49%) and MCP-1 (90%) mRNA expression, as compared to
WT livers. Additionally, attenuation of markers of lung inflammation (TNFα,
76%) and kidney injury (blood urea nitrogen, 42%) were noted in the KO mice
as compared to WT.
Conclusions: P2Y2 receptor function is critical for sepsis-induced mortality
in mice. Early induction of hyper-inflammatory phase is dependent on intact
P2Y2 receptor expression. These findings highlight the functional significance
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
of purinergic signaling in the pathogenesis of sepsis and eventually will lead to
development of novel therapeutics.
356
ROLE OF ENDOTHELIAL MONOCYTE ACTIVATING
POLYPEPTIDE II (EMAP II) IN BRONCHOPULMONARY
DYSPLASIA (BPD)
Lal CV1, Awasthi N2, Xu H1, Schwarz A1, Schwarz R2, Savani R1, Schwarz M1.
1
University of Texas Southwestern, Dallas, TX and 2University of Texas
Southwestern, Dallas, TX.
Purpose of Study: The expression of EMAP II- a potent antiangiogenic and
proinflammatory factor, decreases during periods of vasculogenesis in fetal
lung development and increases in emphysema and BPD. We hypothesized
that exogenous EMAP II administration would cause BPD-like changes in
uninjured-normoxic mice, and that the administration of EMAP II blocking
antibody (EMAP II Ab) would rescue alveolar growth in mice exposed to
hyperoxia.
Methods Used: 1) From postnatal days (PN) 3-15, EMAP II protein or vehicle was administered to mice in 21% oxygen (normoxia) 2) PN 3-15, mice
were exposed to either 85% oxygen (hyperoxia) or normoxia and treated
with either EMAP II Ab or non specific rabbit IgG control (NsIgG). Lungs
were harvested on PN 15. Alveolar architecture was demonstrated by mean
linear intercept (MLI), cellular proliferation by Ki67 immunofluorescence,
and apoptosis by TUNEL assay. Vascular and epithelial lung markers were
analysed by real time PCR. Pulmonary function testing (PFT) was performed
at 6 weeks using Scireq Flexivent FX rodent ventilator.
Summary of Results: 1)Compared to vehicle-treated pups, normoxia pups
treated with EMAP II protein developed alveolar simplification consistent
with BPD (MLI: p<0.0001, t test), reduction in epithelial and vascular lung
markers and worsening lung functions. 2)Compared to normoxia controls,
mice exposed to hyperoxia between PN 3-15 demonstrated distal alveolar
dysplasia (MLI p<0.001 ANOVA), increased proliferation (p<0.01) and apoptosis (p<0.01). Compared to NsIgG-treated hyperoxia controls, hyperoxia
pups treated with EMAP II Ab had a marked reduction in MLI (p<0.01), alveolar cell proliferation (p<0.01), apoptosis (p<0.01) and had improved compliance. EMAP II Ab had no difference in MLI and alveolar cell proliferation
in normoxia pups as compared to NsIgG-treated normoxic controls.
Conclusions: In neonatal mice exogenous delivery of EMAP II protein
induces distal alveolar dysplasia consistent with BPD, while delivery of an
EMAP II function-blocking antibody rescues hyperoxia induced alveolar
dysplasia, reduces cellular hyperproliferation, apoptosis and improves pulmonary functions. This suggests that EMAP II blockade may be a potential
target for therapeutic intervention in BPD development.
357
GROUP B STREPTOCOCCI INDUCE PROINFLAMMATORY
RESPONSES VIA A PROTEIN KINASE D1
DEPENDENT PATHWAY
Upadhyay K1, Park J1, Kim Y1, Meals E1, Talati AJ1, English B2, Yi A1.
1
CFRI, UTHSC, Memphis, TN and 2Michigan State University, Grand
Rapids, TN.
Purpose of Study: Protein kinase D1(PKD1) plays a significant role in inflammatory process mediated by MyD88 dependent toll like receptors
(TLR). We proposed to study the role of PKD1 in Group B Streptococci
(GBS) mediated proinflammatory responses and to determine a molecular
mechanism by which GBS activates PKD1 in macrophages.
Methods Used: RAW 264.7 cells were stimulated with live GBS or penicillin G-killed GBS and activation of PKD1 was analyzed by in vitro kinase assay
and phospho-specific Western blot assay. Subsequently, MyD88-/- macrophages,
IRAK1-/-, or TRAF6 -/- macrophages were stimulated with GBS and activation
of PKD1 was analyzed. Next, to investigate whether PKD1 plays indispensable
role in GBS mediated proinflammatory responses, RAW264.7 cells and
C57BL/6 mice were stimulated with GBS in the presence of PKD inhibitor
Gö6976 and expression and production of the selected cytokines/chemokines
were analyzed by RT-PCR and ELISA. Finally, to determine contribution of
PKD1 in GBS-mediated septic shock-like death, D-galactosamine (D-GalN)
Southern Regional Meeting Abstracts
sensitized mice were challenged with antibiotic killed GBS in the presence of
PKD inhibitor and then viability of mice was observed.
Summary of Results: Both live and antibiotic killed GBS induced kinase
activity and phosphorylation of PKD1. PKD1 activation by GBS was completely abolished in MyD88-/- macrophages and IRAK1-/- macrophages
but not in TRAF6 -/-macrophages. GBS induced activation of signaling
modulators (i.e., JNK, ERK, p38, or NF-κB) and expression and production
of proinflammatory cytokines and chemokines (TNFα, IL-6, IL-10, IL-12p40,
IP-10, MCP-1, CCL5, MIP-1α, MIP-2, LIX, and KC) were either ablated or significantly inhibited in the presence of PKD inhibitor in vitro and in vivo. Furthermore, systemic administration of PKD inhibitor protected D-GalNsensitized mice from shock mediated death caused by GBS.
Conclusions: GBS induced activation of PKD1 via a MyD88 and IRAK1
dependent manner. PKD1 played an indispensable role in GBS-mediated activation of MAPKs and NF-κB and subsequent expression of proinflammatory cytokines and chemokines in vitro and in vivo. Furthermore,
inhibition of PKD1 activation protected mice from death due to exaggerated
proinflammatory response induced by antibiotic-killed GBS.
Cardiovascular Club II - Young Clinician Scientist Award
Competition
11:00 AM
Friday, February 21, 2014
358
MITOCHONDRIAL DNA HAPLOTYPE CONTRIBUTES
TO THE CYTOSKELETAL AND MITOCHONDRIAL
RESPONSES TO CYCLICAL STRETCH IN
ISOLATED CARDIOMYOCYTES
Guichard JL1, Benavides G1, Ballinger S1, Dell’Italia L1,2. 1University of
Alabama at Birmingham (UAB), Birmingham, AL and 2Birmingham Veteran
Affairs Medical Center, Birmingham, AL.
Purpose of Study: We have shown that humans with isolated mitral regurgitation and normal left ventricular systolic function have significant myofibrillar degeneration, aggregates of small mitochondria, and increased formation
of reactive oxygen and nitrogen species (ROS/RNS) within the cardiomyocytes. These findings suggest an interaction between the cytoskeleton and mitochondria. To determine if differences in mtDNA haplotype can contribute to
differences in VO disease susceptibility, we developed a novel mitochondrianuclear exchange (MNX) mouse model in which the mitochondria (mt)
from wild-type C57BL/6J (C57 WT) and C3H/HeN (C3H WT) mice have
been exchanged onto the respective nuclear (n) backgrounds (C57n:C3Hmt
or C3Hn:C57mt).
Methods Used: Cyclical stretch experiments were performed with isolated
cardiomyocytes. Cardiomyocytes were exposed to stretch (60 cycles per minute with 20% stretch for 3 hours) using the Flexcell® FX-5000™ Tension
System. Transmission electron microscopy (TEM) and immunohistochemistry
(IHC) analyses were used for cardiomyocyte structural assessment. The XF24
Extracellular Flux Analyzer from Seahorse Bioscience (North Billerica, MA,
USA) was used to measure real-time oxygen consumption and extracellular
acidification rates.
Summary of Results: TEM analysis showed that control cardiomyocytes
had tightly packed and electron dense subsarcolemmal, interfibrillar, and
perinuclear mitochondria. C57 WT and C3H MNX stretched cardiomyocytes
demonstrated extensive ultrastructure changes including mitochondrial disorganization and swelling, smaller and rounder mitochondria with a loss of
electron density, and structural breakdown of the myofilaments and Z-lines.
IHC analysis showed marked disruption of desmin in C57 WT and C3H
MNX stretched cardiomyocytes, which were in contrast to control cardiomyocytes and C3H WT and C57 MNX stretched cardiomyocytes. Mitochondrial bioenergetic analysis associated the C57 mtDNA haplotype with
bioenergetic dysfunction.
Conclusions: We have shown that mtDNA haplotype contributes to the
cytoskeletal and mitochondrial responses to cyclical stretch in isolated
cardiomyocytes.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
359
WNK1 ATIVATES BK CHANNEL ACTIVITY THROUGH
MAPK ERK1/2 SIGNALING PATHWAY
Liu Y, Shi Y, Caroti C, Bao H, Cai H. Emory University School of Medicine,
Atlanta, GA.
Purpose of Study: WNK (with no lysine) kinase is one of the serine/threonine
kinase family. We have previously shown that WNK4 inhibits BK channel activity through enhancing its degradation via a lysosomal pathway. WNK4 was
also found to inhibit BK channel activity which is kinase-dependent and via activating MAPK ERK1/2 and p-38 signaling pathways. However, little is known
on the role of WNK1 in the regulation of BK activity.
Methods Used: To determine the role of WNK1 in regulation of BK channel
activity, we have investigated the effect of WNK1 on BK channel activity using cell attached single channel recording technique, cell cultures, transfection, siRNA knock-down and western blot analysis.
Summary of Results: We have found that the knock-down of WNK1 expression in HEK 293 cells stably expressing alpha subunit of BK (HEK
BKα cells) channel significantly inhibited both BKα activity (NPo, 0.024
± 0.036, n=18, compared to the control group, 0.1316 ± 0.0573, n=36,
p =0.006) and open probability (Po, 0.0094 ± 0.0098, compared to the control group, 0.0512 ± 0.0573, p<0.001). Western blot analysis showed that
knock-down WNK1 significantly inhibited BKα total expression in HEK
BKα cells in a dose-dependent manner (1.0 ± 0, 0.848 ± 0.213, 0.422 ±
0.297, n=3, p<0.05). Over-expression of WNK1 was also shown to increase
BKα protein expression in a dose-dependent manner (1.0 ± 0, 1.723 ± 0.136,
2.729 ± 0.385, n=3, p<0.05) in HEK 293 cells transiently transfected with
myc-BKα and HA-WNK1. We also found that WNK1 decreased ERK1/2
phosphorylation in a dose-dependent manner.
Conclusions: These data suggest that WNK1 enhanced BKα activity and
total protein through inhibiting MAPK ERK1/2 signaling pathway.
360
RECOVERY OF SMALL MYOCYTES AND REVERSE
REMODELING IN HYPERTENSIVE HEART DISEASE
Al Darazi F, Zhao W, Zhao T, Sun Y, Marion TN, Ahokas RA, Bhattacharya SK,
Gerling I, Weber KT. University of Tennessee Health Science Center,
Memphis, TN.
Purpose of Study: Hypertensive heart disease (HHD) includes hypertrophied cardiomyocytes and widely scattered foci of microscopic scarring, which
have replaced necrotic myocytes. Bordering on these microdomains of fibrosis
are small myocytes that re-express β-myosin heavy chain (β-MHC), a fetal
gene. We hypothesized small myocytes represent an autologous population of
cells which could be recovered for reverse remodeling in HHD.
Methods Used: 8-wk-old male Sprague-Dawley rats received aldosterone/
salt treatment (ALDOST) for 4 wks followed by a 4-wk period of assisted recovery when oral antioxidants (ZnSO4 and nebivolol, a β3 receptor agonist)
were given.
Summary of Results: Compared to untreated age-/sex-matched controls:
mean arterial pressure (MAP, 162±2 vs. 99±2 mmHg) and left ventricular
(LV) mass (1043±23 vs. 883±32 g) were increased (p<0.05) while pressure-independent scarring appeared in the right and left heart at 4 wks
ALDOST. Bordering on these scars were α-smooth muscle actin+
myofibroblasts (myoFb) and small myocytes revealing: i) re-expression of
β-MHC and atrial natriuretic peptide (ANP); ii) increased expression of reactive oxygen species; and iii) reduced nitric oxide. Cardiac tissue 8isoprostane and redox-sensitive ligases (atrogin-1 and MuRF1) of the
ubiquitin protease system were each increased (p<0.05) while E/A ratio
and LV fractional shortening were reduced (p<0.05). Assisted recovery led
to normalized MAP, regression in LV mass, and reduction in small myocytes
(p<0.05) with restored redox balance and NO, neutralized protein degradation, together with improved diastolic and systolic function.
Conclusions: HHD includes microdomains of fibrosis where small myocytes
re-express a fetal gene program together with altered redox balance and protein
degradation. Recovery of these myocytes, using neurohormonal withdrawal
plus a regimen of antioxidants, is feasible and leads to reverse remodeling with
improved ventricular function in HHD. Whether heterocellular signaling with
514
myoFb exists and the potential origin of these small myocytes as progeny of
bone marrow or cardiac stem cells vs. dedifferentiated myocytes remains to
be determined.
361
URIC ACID IS ASSOCIATED WITH METABOLIC SYNDROME
IN CHILDREN AND ADULTS: THE BOGALUSA HEART
STUDY
Sun D1,2, Zhang X1, Li S1, Fernandez C1, Chen W1, Srinivasan SR1, Berenson GS1.
1
Tulane University, New Orleans, LA and 2Peking University Health Science
Center, Beijing, China.
Purpose of Study: This study assessed the hypothesis that uric acid (UA) is
associated with metabolic syndrome (MetS) components and their clustering
patterns in different age periods.
Methods Used: The study sample consisted of 2614 children (1577 whites,
1037 blacks; 1333 males; aged 4-18 years) and 2447 adults (1682 whites,
1765 blacks; 1072 males; aged 19-54 years) examined during 1987-2010.
The adverse levels of MetS components, including body mass index
(BMI), mean arterial pressure (MAP), triglycerides to HDL cholesterol ratio
(TG/HDLC), and homeostasis model assessment of insulin resistance
(HOMA), were defined as >75th percentile specific for age, race and sex.
Observed/expected (O/E) ratio and intra-class correlation (ICC) were used
as a measure of the degree of clustering of categorical and continuous
variables, respectively.
Summary of Results: UA was significantly positively associated with only
BMI in children, and with all four components in adults. Odds ratios of
UA associated with MetS were 1.74 in children and 1.92 in adults
(p<0.001 for both), adjusted for race, sex and age. Trends in O/E ratios with
UA quartiles are shown in the figure below. ICCs of 3- and 4-component
clusters increased with increasing UA quartiles in children; however, only
BMI-MAP-HOMA cluster showed an increasing trend of ICC in adults.
Conclusions: The results suggest an important role of UA in the development of MetS in pediatric and adult populations. The findings provide further
insights into the potential pathophysiological mechanisms of MetS and have
implications for identification and treatment of high risk individuals for MetS
in early life.
FIGURE 1. Race-, sex- and age-adjusted O/E ratios of MetS
clusters by uric acid quartiles (Q).
Adolescent Medicine and Pediatrics
Concurrent Session
2:00 PM
Friday, February 21, 2014
362
PEDIATRIC EMERGENCY DEPARTMENT USE BY
ADULTS WITH SPECIAL HEALTHCARE NEEDS LAURA A.
WRIGHT-SEXTON, MD, ROSINA CONNELLY, MD, MPH, FAAP
Wright L1, Connelly R2. 1University of South Alabama, Mobile, AL and
2
University of South Alabama, Mobile, AL.
Purpose of Study: To describe the use of a pediatric emergency department
(PED) by patients eighteen years of age and older over a ten-year period. We
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
hypothesized that adult patients with a special health care need (SHCN) presenting
to a PED were more likely to be seen by a pediatric healthcare provider.
Methods Used: This was a retrospective chart review of emergency department encounters by individuals 18 years and older resulting in care provided
by a pediatric provider at a tertiary pediatric hospital from January 1st, 2002December 31st, 2012.
Summary of Results: There were a total of 4,987 patient encounters during
the study period, 504 (10%) were by adult patients with SHCN. There were a
total of 261 adult patients with SHCN seeking care in the PED in this study
period. Gender was not associated with whether patients were seen (p=0.46),
however, having a tracheostomy and/or G-tube was significantly associated
with being seen in the PED (p=0.008). Of the 504 encounters seen by a physician in the PED, 63% of the visits were unrelated to their SHCN.
Conclusions: Pediatric patients with SHCN who transition into adulthood
used the PED in substantial numbers during our study period. More than
60% of these patient visits were unrelated to their SHCN. As this group of
patients continues to grow, PEDs should carefully consider their resources
and how best to provide care to adults with SHCN. Unless large-scale policy
decisions are made, pediatric hospitals should be prepared with adequate
resources and training to deal with these complex adult patients.
363
MANAGEMENT OF FEBRILE INFANTS IN PEDIATRIC
EMERGENCY DEPARTMENTS: 2004-2012
Verbrugghe DB, Goudie A, Onukwube J, Doshi P, Robbins J. Arkansas
Children’s Hospital, Little Rock, AR.
Purpose of Study: Up to one-third of all presentations to pediatric emergency
departments (ED) are documented with fever. Fever in young infants can be a
presenting symptom of infectious diseases ranging from a simple cold to devastating meningitis. While the incidence of bacterial meningitis among young
infants has significantly decreased since the introduction of the Haemophilus influenza and pneumococcal vaccines, recommendations for the management of
febrile infants have not significantly changed over the past 30 years.
The purpose of this study is to evaluate variation in the management of
febrile infants 0-30, 31-60, and 61-90 days of age across 35 pediatric emergency departments in the United States. Management practices are compared
to evolving recommendations from a multicenter and an individual facility
level perspective.
Methods Used: Data from 35 EDs participating in the Pediatric Health Information System (PHIS) for years 2004-2012 were used to identify infants
less than 3 months of age with a diagnosis code of fever. Medical diagnoses,
diagnostic tests performed, and medications prescribed are identified in PHIS
using ICD-9-CM and/or Clinical Transaction Classification (CTC) codes.
Summary of Results: 92,428 infants <3 months of age were identified in
PHIS with an ED diagnosis of fever. By age group, blood was drawn for lab
testing among 82.4%, 82.8%, and 64.9% of infants 0-30, 31-60, and 61-90 days
of age respectively; urine was obtained from 82.7%, 84.2% and 72.7%; lumbar
punctures (LPs) were performed in 76.4%, 52.2%, and 16.1% of infants; respiratory syncytial virus testing was performed in 28.2%, 27.3%, and 18.9%; and
chest X-rays were performed in 34.9%, 24.8%, and 31.5% of infants.
Wide variation was observed across the 35 institutions under study in the
management of febrile infants. Across institutions LP rates ranged from
10.4% to 73.7%, blood samples drawn from 42.4% to 90%, and urine
samples obtained from 41.9% to 90.7%.
Conclusions: There is great variability among individual institutions in how
febrile infants are managed upon presentation to the emergency department.
Management practices vary from evolving consensus recommendations in
the United States. Our future studies will determine whether management
practices are related to the outcomes of infants presenting with fever.
Southern Regional Meeting Abstracts
Purpose of Study: Limited data exist regarding influenza vaccination (IV)
programs in the pediatric emergency department (ED) setting. The objectives
of this study were to determine rates of influenza immunization among children
with a minor illness or injury treated in a pediatric emergency department (ED)
and parent willingness for their child to receive influenza vaccine (IV) during
future ED visits.
Methods Used: A 25-question survey was administered to parents of children aged ≥ 6 months evaluated in the ED in the summer of 2013. Exclusion
criteria include temperature ≥ 103°F, communication barrier, egg or IV allergy, or evaluation for non-accidental trauma. Demographic and visit data,
IV history, and intent and willingness to receive future IV were recorded.
Data were analyzed using standard descriptive statistics.
Summary of Results: Of 457 families approached, 119 (26%) declined participation, 53 (12%) were ineligible, and 285 (62%) were enrolled. Mean age
was 7.2 (±5) years; 46% were black; and 56% had ≥ 1 ED visit in the past
year. Fifteen (3%) were too young to have received IV in the prior season.
Of the remainder, 64% (95% confidence interval: 59%, 70%) reported receiving ≥ 1 dose of IV in the past year. The most common reasons for not
receiving IV were concerns about side effects (31%) and lack of time or interest (24%). 85% of parents report they will probably/ definitely vaccinate
their child prior to the upcoming influenza season; 83% report willingness
to receive IV at a future ED visit. Children with any current chronic medical
condition were more likely to report vaccination in the prior influenza season
(77% vs 60%, p<0.005). Among respondents for the 88 children who were
over 6 months during the previous influenza season yet not vaccinated, 55
(63%) report they would probably/definitely give IV to their child if offered
in the ED. Of these, 22 (25%) children had asthma or a high risk chronic
health condition.
Conclusions: Because influenza immunization rates for children are lower
than desired, our data support an IV program in the pediatric ED. Parents
are often concerned about side effects of influenza immunization. Primary
care providers should consider targeting education in this area to impact vaccination rates.
365
PERCEIVED PAIN WITH HPV VACCINE COMPARED TO
OTHER RECOMMENDED VACCINES FOR ADOLESCENTS:
WHAT CAUSES THE BIGGEST “OUCH”?
Salz N1, Lopez A2, Amin R3, Burns JJ1. 1Florida State University College of
Medicine, Tallahassee, FL; 2Sacred Heart Children’s Hospital, Pensacola,
FL and 3University of West Florida, Pensacola, FL.
Purpose of Study: The purpose of the study is to determine if the Human
papillomavirus vaccine (HPV) is more painful at the time of administration
than other recommended vaccines. Pain associated with the HPV vaccine
may contribute to incomplete vaccination status and explain higher rates of
syncope reported in some studies. No study has been done that surveys pain
from the patient receiving the recommended adolescent vaccines.
Methods Used: Consecutive patient visits from 04/24/2013 to 10/04/2013
for 10-18 year olds at a predominantly Medicaid insured residency teaching
clinic were assessed for pain associated with injection using the Wong-Baker
FACES Pain Rating Scale. Patients needing the HPV vaccine and at least one
other immunization were eligible. Patients were blinded to the administration
order. The participants were asked to rate their pain immediately after each
injection. The non-HPV vaccines used for comparison were Menactra,
Paired analysis of pain scale score: HPV vs. comparison vaccines
364
ASSESSING INTENT AND WILLINGNESS TO IMMUNIZE
CHILDREN AGAINST INFLUENZA IN THE PEDIATRIC
EMERGENCY DEPARTMENT
Stevenson M1, Levine A2, Bryant K1, Paul RI1. 1University of Louisville
School of Medicine, Louisville, KY and 2University of Louisville School of
Medicine, Louisville, KY.
*Wilcoxon Signed Ranks Test ** Statistically significant p ∼ 0.01 *** Menactra, Hepatitis
A, Varicella, Tdap.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Hepatitis A, Varicella and Tdap. Wilcoxon Signed Ranks test was used in
comparing the pain scores. To account for multiple analyses, a p-value of
<0.01 was used for significance using the Bonferroni correction.
Summary of Results: 150 participants were included with 47.3% females
and 52.7% males. A statistically greater number of HPV injections hurt more
on the pain rating scales than the average of the non-HPV injections given at
the same visit (p=0.000) (see Table). HPV was also found to hurt more often
than Menactra (p = 0.000) and Hepatitis A (p = 0.000) individually. There
were no significant differences for Tdap nor Varicella vs. HPV using
predetermined cut off of p<0.01.
Conclusions: HPV vaccine was perceived as more painful than the average
of 4 commonly administered vaccines and two individual vaccines.
366
ATTITUDES TOWARD HPV VACCINE: DIFFERENCES
AMONG PARENTS OF MALE AND FEMALE ADOLESCENTS
Roberts J1, Hale J2, Jacobson R3, Pope C1, Thompson D2, Darden P2.
1
MUSC, Charleston, SC; 2OUHSC, Oklahoma City, OK and 3Mayo Clinic,
Rochester, MN.
Purpose of Study: Recommendations for human papillomavirus (HPV) vaccination of adolescent males and females originated at different dates and for
different reasons. The purpose of this study is to identify differences in
attitudes toward HPV vaccine between the parents of adolescent males or
females.
Methods Used: In 2012, parents of 11-17 year-old adolescents were surveyed at any type of visit to 6 pediatric practices in OK and SC. Attitudes
were measured with an adapted Parent Attitudes about Childhood Vaccine
(PACV) survey originally developed by Opel et al. The 15-item survey is
not vaccine specific; it rates general vaccine hesitancy (on a scale of 0-100) with
higher scores indicating greater hesitancy. Medical records were reviewed and
adolescents were considered “due for HPV vaccine” if, at the end of the visit,
they had received <3 doses and were eligible for an additional dose. We compared “due for HPV vaccine,” with parents’ PACV score, and with individual
responses for each of the 15 items, by adolescent gender. We used χ2,
and t-tests for bivariate analyses and logistic regression for multivariable analysis.
Summary of Results: Data were analyzed from 375 parents and adolescents
(55% male). Adolescent males were more likely to be due for the HPV vaccine than females (65% v. 46%, p<0.001). The mean PACV score for parents
of males (22.5) was lower than for parents of females (26.5), P=0.02. The
PACV scale did not predict HPV vaccine status for either males (P=0.61)
or females (P=0.19). When analyzing the 15 PACV scale items individually,
two items differed by gender. Parents of males expressed less concern that
vaccine might not prevent disease (34% v. 47%; P=.02) and less concern that
vaccines might not be safe (35% v. 52%; P=.004).
Conclusions: Adolescent males were more likely to be due for HPV than
females. Parents of males were less hesitant about vaccines than parents of
females. The differences in attitudes found can guide strategies to improve
HPV vaccine acceptance in males. However, in this analysis PACV was not
associated with receipt of HPV vaccine.
367
COMPLIANCE WITH NATIONAL GUIDELINES FOR
PEDIATRIC PATIENTS WITH COMMUNITY-ACQUIRED
PNEUMONIA AFTER EDUCATION BY AN ANTIMICROBIAL
STEWARDSHIP PROGRAM
Jones P1, Stephens S1, Bagga B1,2, Lee K2,1, Arnold S1,2. 1University of
Tennessee Health Science Center, Memphis, TN and 2Le Bonheur Children’s
Hospital, Memphis, TN.
Purpose of Study: In 2011, guidelines for the management of communityacquired pneumonia (CAP) in children were published. For uncomplicated
CAP, recommendations are that healthy, immunized children receive a penicillin if local pneumococcal resistance patterns permit. Third generation
cephalosporins are reserved for children with moderate to severe or complicated CAP or those who are not fully immunized. The Antimicrobial Stewardship Program (ASP) at Le Bonheur Children’s Hospital conducted a
baseline review to ascertain which antibiotics were being used to treat
CAP. In addition, records for pneumococcal isolates from the preceding
2 years were reviewed to determine local susceptibility patterns before
recommendations were issued.
516
Methods Used: All pneumococcal isolates from the microbiology lab from
2010-11 were reviewed and were classified as susceptible, intermediate or resistant based on pneumonia breakpoints. Recommendations CAP management
were prepared in mid-2012 and were presented to hospital and emergency
physicians and residents through conferences and grand rounds. The Pediatric
Hospital Information Systems (PHIS) database was used to determine antibiotic
use in patients diagnosed with pneumonia before and after education. The database was also used to compare our antibiotic use to other pediatric hospitals.
Summary of Results: Among pneumococcal isolates, 99% were susceptible
or intermediate to penicillin (MIC ≤ 4 mcg/ml). As a result, ampicillin was
recommended for empiric therapy for uncomplicated CAP. During the three
years prior to guideline education, ceftriaxone was used in 90% of children
admitted for CAP and ampicillin in 1%. After education, ampicillin use increased to 50% during the 2012-13 respiratory season, and ceftriaxone use
decreased to 50%. Ampicillin use increased from 8% to 20% at the other
PHIS hospitals.
Conclusions: Local development of guidelines and ASP driven education of
medical staff led to a substantial increase in the use of a narrow spectrum antibiotic, ampicillin, and a reduction in the unnecessary use of ceftriaxone for
CAP. Daily ASP initiated monitoring will ensure further reduction in broadspectrum antimicrobials in patients admitted to our hospital with CAP.
368
TEEN DRIVING TOOLKIT FOR PEDIATRICIANS
Woodfin T2, Irons E3, King WD1, Crew M3, Monroe K1. 1univ of alabama,
Birmingham, AL; 2Univ of Alabama, Birmingham, AL and 3Childrens of
Alabama, Birmingham, AL.
Purpose of Study: The American Academy of Pediatrics (AAP) recommends pediatricians discuss safe passenger behavior. A convenience sample
survey of teens in our state revealed only 11.5% of teens recalled their pediatrician discussing safe driving with them. Knowledge and practice of state
pediatricians regarding safe teen driving laws and education were assessed
in this study.
Methods Used: Funded by the Allstate Foundation, the Alabama AAP created
a coalition of interested stakeholders, the Alabama Safe Teen Driving coalition.
A series of Grand Rounds were conducted across the state. A pre and posttest
were performed for the attendees at these conferences. A toolkit to assist
pediatricians was created and distributed. A follow up survey assessed the usefulness of the toolkit. Log likelihood ratios used for evaluation.
Summary of Results: 147 pediatricians attended the sessions (only 61%
aware of our state graduated drivers’ license law). Although, 35% of pediatricians surveyed see more than 70 adolescents in their practice each year,
nearly half, 49.3% reported that they never have discussions with teen
patients about safe driving. 42% were not familiar with driving contracts
and of those who were familiar, only 15% had ever recommended one to a
patient. The pre test indicated that only 21.0% of participants knew GDL
components before the educational intervention compared to 98.6% of post
test participants could correctly identify the GDL components (z=13.4,
p<0.001). In follow up survey (n=45), toolkit use was most common in the
primary care setting( 93% usage rate(G2 =7.4,p=0.007). The toolkit was also
used more in practices that see 20 or more teens per week( 84.6%) (G2 =
10.1, p=0.002).
Conclusions: Many state pediatricians were unaware of our state law regarding GDL. Despite seeing a number of adolescents in their practices, many
pediatricians were not familiar with driving contracts nor did they discuss
safe driving with their patients. A brief educational intervention greatly improved knowledge among participants. The toolkit to assist pediatricians
has been especially useful in primary care settings and in practices that see
a high number of teens per week.
369
UTILITY OF THE GAPS QUESTIONNAIRE AMONG HIV
INFECTED YOUTH
Benjamins L1, Beyda R1, Schulte C2, Rodriguez G3, Feldmann J4. 1University of Texas School of Medicine, Houston, Houston, TX; 2University of
Texas School of Medicine, Houston, Houston, TX; 3University of Texas
School of Medicine, Houston, Houston, TX and 4Baylor College of Medicine,
Houston, TX.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: Growing numbers of HIV positive children are surviving
into adolescence. Risk taking behaviors in this population are uniquely important as they may potentiate HIV transmission. Limited data exists on
the utility of screening tools specifically addressing modifiable risk behaviors
in adolescents with HIV. The AMA’s Guidelines for Adolescent Preventive
Services (GAPS) survey screens adolescents for social and behavioral risks
including depressive symptoms. We assessed the utility of the GAPS questionnaire among youth with HIV.
Methods Used: We conducted a retrospective chart review of patients ages
10 to 21 years old seen at the University of Texas Pediatric HIV clinic. Data
was collected from GAPS surveys completed routinely as part of the health
maintenance exam. Viral load, CD4 count and other clinical data were also
collected. We compared our results to the Youth Risk Behavior Survey.
Summary of Results: A total of 66 charts were reviewed; 92% of the youth
were perinatally infected, average age 14.86. Compared to the 2011 YRBS,
8% of study subjects vs. 17% of YRBS youth report carrying a weapon
and 33% vs. 47% report ever having sex. One third of the HIV+ youth had
a history of an STI. A greater proportion of teens with HIV reported depressive symptoms (38% HIV+ vs. 28.5% YRBS). Comparing GAPS variables
and viral load, we found greater reports of sadness (44%) in those with a detectable VL versus those who were undetectable (26%). Those with a detectable VL also had a statistically significant higher rate of suicidal ideation
or suicide attempts (6%) compared to those with an undetectable VL (0%),
p<0.05.
Conclusions: Our results are in keeping with previously published studies
revealing increased rates of depression among HIV positive youth. Other risk
behaviors were lower than national norms, but still of significance. Identifying and addressing risks enables earlier intervention and treatment. The
GAPS is useful tool for risk identification among HIV positive youth.
370
USE OF THE PEDIATRIC SYMPTOM
CHECKLIST TO SCREEN FOR
ATTENTION-DEFICIT/HYPERACTIVITY DISORDER
Kube DA, Whitaker TM, Palmer FB. University of Tennessee Health Science
Center, Memphis, TN.
Purpose of Study: This study was designed to determine if the attention
subscale of the Pediatric Symptom Checklist (PSC), a screening tool used
by primary care clinicians to detect possible behavioral disorders in children,
correctly identified children with ADHD when compared to a measure specific for ADHD, the Vanderbilt ADHD Diagnostic Parent Rating Scales
(VADPRS).
Methods Used: One hundred eleven children (ages 6 to18 years, 51% male)
admitted to a children’s hospital for acute illness were selected for behavioral
screening. A parent/caregiver completed the PSC and the VADPRS. The
PSC consists of 35 items for behavioral problems, rated on a severity scale
of 0-2. The PSC is positive for impairment if the total score is ≥28 and positive for ADHD if the five-item attention subscale score is ≥7. The VADPRS
consists of the 9 criteria from the Diagnostic and Statistical Manual of Mental Disorders-IV-TR used for diagnosing ADHD, predominantly Inattentive
Type (ADHD-I) and the 9 criteria for diagnosing ADHD, predominantly
Hyperactive-Impulsive Type (ADHD-H), scored on a severity scale of 0-3, with
ratings of 2 and 3 considered positive. The VADPRS is positive for ADHD-I if
≥6 specific criteria are met, ADHD-H if ≥6 specific criteria are met and ADHD,
Combined Type (ADHD-C) if criteria are met for both.
Summary of Results: The PSC was positive for behavioral impairment in
15 subjects (13 on the attention subscale). Of the 13 subjects on the attention
subscale, 4 subjects had a prior diagnosis of ADHD and 9 were newly identified. The VADPRS was positive for 14 subjects: 4 ADHD-I, 3 ADHD-H,
and 7 ADHD-C. PSC sensitivity for identifying ADHD diagnosed on the
VADPRS was 57.0%, specificity 94.2%, positive predictive value 61.5%,
and negative predictive value 93.2%. The PSC correctly identified all 7
subjects with ADHD-C.
Conclusions: The PSC was moderately sensitive and highly specific for
ADHD diagnosed by the VADPRS. The PSC correctly identified all subjects
with ADHD-C, making it a useful screening tool for this subtype. The PSC
can be useful as a screen for excluding ADHD due to its high specificity and
negative predictive value. Nine previously undiagnosed ADHD subjects
Southern Regional Meeting Abstracts
were identified by the PSC, supporting the idea that inpatient hospitalization
may provide an additional opportunity for behavioral screening.
371
VALIDATION OF AN ANTIBIOTIC STEWARDSHIP
PROGRAM’S NEONATAL INTENSIVE CARE UNIT
ANTIBIOTIC USE GUIDELINES USING STERILE SITE
CULTURE RESULTS
Phiapalath T1,2, Bagga B1,2, Talati AJ1,2, Arnold S1,2, Lee K1,2. 1Le Bonheur
Children’s Hospital, Memphis, TN and 2The University of Tennessee Health
Sciences Center, Memphis, TN.
Purpose of Study: In early 2012, the Antibiotic Stewardship Program (ASP)
at Le Bonheur Children’s Hopsital created and implemented neonatal intensive care unit (NICU) empiric antibiotic guidelines for early and late onset
sepsis, and necrotizing enterocolitis. Guidelines were based on extensive review of the literature, our hospital’s antibiogram, and discussion with neonatology staff. In 2013, concern was expressed that the implemented antibiotic
regimens were inappropriate for more resistant pathogens potentially found
in the NICU. The purpose of this study was to validate the use of the current
NICU empiric antimicrobials by studying all positive sterile site cultures
from our NICU over the past year.
Methods Used: Positive NICU cultures between June 2012 to May 2013
were extracted and all sterile site cultures were evaluated. Information collected included culture site, pathogen, and susceptibility. Each culture was
evaluated for susceptibility to the ASP guideline antibiotics. Patients with resistant cultures were further reviewed for risks for antibiotic resistance.
Summary of Results: 100 of the 179 analyzed cultures were from sterile
sites. 4% (4/100) were found to be resistant to guideline antibiotics. Two resistant cultures were E. coli in central line blood samples from one patient
transferred from another hospital where the same organism had previously
been cultured. Stenotrophomonas maltophilia grew in the other two samples.
One was from lung tissue isolated in broth only. The other was from a peripheral blood culture in a patient with previous identification of the organism in
a respiratory culture at the transferring hospital.
Conclusions: Our study demonstrates that the majority of sterile site cultures
from our NICU are susceptible to the current ASP recommended empiric
therapy. 96% of the positive sterile site cultures were susceptible to the
ASP-selected empiric antibiotics. Three of the four cultures resistant to the
recommended empiric antibiotics were from recently admitted patients with
these bacteria previously cultured at their transferring hospitals. These results
validate the utilization of our ASP-initiated NICU empiric antibiotic
guidelines at our pediatric hospital.
372
ESOPHAGEAL BOUGIENAGE VS SURGICAL MANAGEMENT
FOR ESOPHAGEAL COINS
Allie EH, Blackshaw A, Losek J, Tuuri RE. Medical University of South
Carolina, Charelston, SC.
Purpose of Study: Our objective was to report our pediatric emergency department (PED) experience with esophageal bougienage (EB) versus surgical
retrieval for esophageal coins with regard to clinical outcomes and costs.
Methods Used: We conducted a cross sectional observational review of all
children with esophageal coins presenting to the MUSC PED from January
2004 to October 2012. The following data were abstracted: presenting symptoms, age, sex, exclusion criteria for bougienage, efficacy and complications,
return visits, length of stay (LOS), and hospital charges. T-tests and 95% confidence intervals were used for comparisons of outcome measures between
the group undergoing successful EB and those patients undergoing surgical
retrieval.
Summary of Results: 245 patients presented to our PED with esophageal
coins. 135 patients had successful EB and 109 had surgical retrieval. 1 case
was excluded from the final analysis after being discharged with observation
after failed EB. 165 patients were eligible for EB. Of the EB-eligible, 129
had successful EB; 7 had unsuccessful EB followed by surgical retrieval;
and 29 underwent surgical retrieval first. Of the 80 ineligible for EB, 71
had surgical retrieval, and 9 had EB. Of the inappropriate EB there were
6 successful and 3 failed attempts. The mean length of stay (LOS) for
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
successful EB was 137 minutes (SD 54) and 769 (SD 535) for the surgical
cases. The difference in the means between treatment groups was 632 minutes,
95% CI for the difference was 723 to 541 (p = 0.000).
The mean hospital charges for successful EB were $984 (SD $576) and
$7022 (SD $3132) for the surgical cases (p=0.000). The difference in means
was $6,038, 95% CI $6,580 to $5,496 (p= 0.000). The mean age for the EB
cases was 4 yrs (SD 2) versus 2 yrs (SD 3) for those undergoing surgical retrieval (p=0.000). The EB cases had 19 minor and no major complications
and 5 return visits to clinics or the PED. One child who underwent EB had
a failure of coin passage beyond the pylorus and subsequently underwent
surgical retrieval. The surgical group had ten minor and two major
complications – a traumatic esophageal diverticulum and an intra-procedural
endotracheal tube dislodgement.
Conclusions: For eligible children, esophageal bougienage is a safe and
more cost-effective management strategy for lodged esophageal coins.
Allergy, Immunology, and Rheumatology I
Concurrent Session
2:00 PM
Friday, February 21, 2014
373
AUTOREACTIVE B CELL PHENOTYPE IN BXD2 MICE
USING B CELL TETRAMERS
Hamilton JA2,1, Li J1, Wu Q1, Yang P1, Luo B1, Hsu H1, Mountz , JD1,2,3.
1
UAB, Birmingham, AL; 2UAB, Birmingham, AL and 3VA Medical Center,
Birmingham, AL.
Purpose of Study: In patients with systemic lupus erythematous (SLE) and
rheumatoid arthritis (RA), defective B cell tolerance enables the generation of
pathogenic autoantibodies. BXD2 mice develop lupus and erosive arthritis characterized by production of high-affinity, class-switched autoantibodies. To facilitate
study of the mechanisms preventing autoreactive B cell activation in the context
of a full B cell repertoire, we have developed a B-cell tetramer-based strategy
for ex vivo detection and characterization of autoreactive B cells in BXD2 mice.
Methods Used: A PEPperPRINT Autoimmunity Microarray covering 2,733
linear B-cell autoepitopes from the Immune Epitope Database (IEDB) was
used to identify BXD2 autoepitopes. Tetramers were generated from top
peptides. B cells were characterized by 11-parameter flow cytometry using
live/dead analysis, non-specific and specific tetramer staining, and analysis
of 4 parameters for follicular (CD23+CD21-), germinal center (GL-7), marginal zone (CD21+CD23-), and type I interferon (IFN) induced activation
molecule (CD69).
Summary of Results: The dominant epitopes recognized by BXD2
autoantibodies are those commonly found in human SLE and RA patients,
including peptides of nucleoproteins, ribonucleoproteins and heat shock
proteins. The array further revealed reactivity with several citrulline-modified
peptides. Tetramer studies revealed an age-dependent 5-10 fold increase in
autoreactive B cells to fibromodulin-[cit] and snRNP in BXD2 compared to
B6 mice. Within fibromodulin-[cit] tetramer+ cells, there was a significant increase in marginal zone (29% vs 4%) and germinal center (7.3% vs 0%) B cells
in BXD2 vs B6 mice. There was also a significant increase in the % of CD69+
(99% vs 8.45%) tetramer+ B cells in BXD2 vs B6 mice.
Conclusions: Autoreactive B cells in BXD2 mice are enriched for activated
B cells that are derived from either MZ or GC B cells. These results are consistent with our previous analyses indicating that type I IFNs stimulate CD69
expression on MZ B cells to induce follicular migration of MZ B cells to
break immune tolerance to apoptotic self-antigens. These findings indicate
that targeted depletion of this subpopulation of autoreactive B cells may be
beneficial for treatment of rheumatic disease.
Spondyloarthritides (SpA). This study investigated the prevalence of CVD
risk factors, CHD and Cerebral Vascular accident in veterans with SpA (including Psoriatic Arthritis (PsA), Ankylosing Spondylitis (AS), and Reactive
arthritis (ReA) at the Jackson VAMC.
Methods Used: A retrospective chart review using ICD-9 codes for PsA,
AS, and ReA was performed at Jackson, VAMC. Data including age, race,
gender, lipid panel, HbA1c, presence of hypertension (HTN), smoking,
CVD, and cerebrovascular events were tabulated. Matched Controls were selected from VA clinics and 2:1 matching was performed. A Comparison of
CHD, stroke(s), and CVD risk factors (dyslipidemia (DLD), HTN, and diabetes Mellitus (DM) was made. Prevalence ration and odds ratio were calculated by standard method. Statistical significant (alpha <0.05) was calculated
using Chi-square and Fisher’s exact test.
Summary of Results: There were 81 patients, 79 were male and 2 female.
With a mean age of patients with PsA, AS ,and ReA being 61,8, 60.4, and
56, respectively. There was a significant increase in the prevalence of CHD
and its risk factors in the SpA, PsA, and AS patients when compared with
the AHA data, and General Male vets from the 2003-04 control population.
When compared to the matched controls the risk was increased but did not
achieve statistical significance. There was an insufficient number of black
patients in the cohort to assess effect of ethnicity.
Conclusions: Our results suggest that patients with SpA have an increased
risk of developing CHD as well its risk factors. Our study is unique as increased risk and it quantification have not been previously reported in the
U.S. veteran population. The increased CHD risk attributable to the increased
prevalence of risk factors cannot be determined in this study but has been
suggested to be over and above them.
375
LOSS OF SURROGATE LIGHT CHAIN
PROTEINS DEREGULATES THE MATURE B CELL
CDR-H3 REPERTOIRE
Khass M1, Watkins L2, Zhuang Y2, Burrows P3, Schroeder H2. 1UAB,
Birmingham, AL; 2UAB, Birmingham, AL and 3UAB, Birmingham, AL.
Purpose of Study: Successful association of the μ heavy chain and the surrogate light chain proteins, λ5 and VpreB, creates the pre B cell receptor (Pre
BCR) and establishes a critical checkpoint in B cell development. Failure to
create the Pre BCR arrests B cell development at the early PreB cell stage, a
situation that has been suggested to be a factor promoting human acute lymphoblastic leukemia (ALL). We previously showed that the B cell repertoire
is controlled by the sequence characteristics of the complementary determining region heavy chain (CDR-H3), which lies at the center of the antigen
binding site. Using a bioinformatics system we developed for CDR-H3 analysis, we sought to examine the mature B cell CDR-H3 repertoire in mice
lacking λ5 expression, to test whether absence of Pre BCR selection can affect its composition.
Methods Used: We analyzed CDR-H3 based on its length, reading frame usage and average amino acid hydrophobicity content.
Summary of Results: Our results show that λ5 knock out transitional and
follicular B cells have an increase in the use of disfavored highly hydrophobic and/or charged CDR-H3 sequences with increased use of the normally
underrepresented reading frame 2. Peritoneal cavity B1a cells from the λ5
KO mice showed the same features.
Conclusions: These findings suggest that a lack of control of the repertoire
due to a defect at the PreB cell stage can impact the mature B cell compartments, potentially promoting abnormal autoantibody responses and contributing to the transformation of B cells. This insight may help to explain
one mechanism for the pathogenesis of human ALL.
374
376
PREVELANCE OF CORONARY HEART DISEASE AND ITS
RISK FACTORS IN VETERANS WITH
SPONDYLOARTHRIDITIDES
THE ROLE OF LEUKOCYTE ASSOCIATED
IMMUNOGLOBULIN-LIKE RECEPTOR-1 (LAIR-1) IN
SUPPRESSING COLLAGEN-INDUCED ARTHRITIS
Mains TB, Majithia V. University of Mississippi Medical Center, Jackson, MS.
Purpose of Study: An increased prevalence of cardiovascular disease (CVD)
and coronary heart disease (CHD) has been reported in patients with
Kim S1, Price L1, Cullins D1, Park J1, Stuart JM2,1, Kang A1, Myers L3,1.
1
University of Tennessee Health Science Center, Memphis, TN; 2Veterans
Affairs, Memphis, TN and 3Le Bonheur Children’s Hospital, Memphis, TN.
518
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Purpose of Study: The success of Abatacept (CTLA4-Ig) in treating rheumatoid arthritis (RA) suggests that targeting T cells can be an effective therapeutic strategy for RA. A possible new therapeutic target might be the
inhibitory receptor Leukocyte Associated Immunoglobulin-Like Receptor-1
(LAIR-1) which is found on peripheral mononuclear cells, including NK
cells and T cells. Our hypothesis was that stimulation of LAIR-1 will suppress
cell signaling and lead to inhibition of autoimmune arthritis in the collageninduced arthritis (CIA) model.
Methods Used: DR1 transgenic mice that were LAIR-1 -/- mice (n=7) were
immunized with type II collagen (CII) to induce arthritis and compared to
DR1 LAIR-1 +/+ littermate controls (n=13). Each mouse was scored three
times weekly beginning 2 weeks post immunization and the mean severity
scores and percentage of arthritic limbs were recorded at each time point.
Summary of Results: When CII-reactive murine T cells were cultured in
vitro with the immunodominant determinant of CII we found that type I collagen could suppress IL-17 responses in the presence of LAIR-1. In vivo,
mice that were immunized demonstrated that the LAIR -1 -/- group developed more severe arthritis than the wild type mice (severity scores 4.3 ±
2.7 vs. 2.2 ± 1.4, p=0.03). In addition, LAIR -1 -/- mice had a greater percentage of limbs affected with arthritis than did the wild type group (33 ±
21 vs. 17 ± 10, p=0.02). Antibody titers (IgG) to CII correlated with arthritis
severity.
Conclusions: We have demonstrated that LAIR-1 -/- /DR1 mice develop arthritis that is more severe, with more arthritic joints than DR1 mice alone.
These data suggest that LAIR-1 stimulation is involved in the modulation
of CIA and that stimulation of LAIR-1 may serve as an effective treatment
for RA.
Southern Regional Meeting Abstracts
378
REGIONS ON CHROMOSOME 2, 6, AND 10 MAY
CONTRIBUTE TO THE SPECTRUM OF CVID
Hwangpo T1, Szymanska Mroczek E1, Brand M1, Burrows P1, Brown E2,
Reynolds R1, Schroeder H1. 1University of Alabama, Birmingham, AL and
2
University of Alabama at Birmingham, Birmingham, AL.
Purpose of Study: In our PID Clinic, we have cared for more than 400
patients with recurrent infections and depressed immunoglobulins. We have
enrolled a majority of them in a protocol to identify regions within the genome that may contribute to the spectrum of their disease. We are interested
in patients with CVID as well as a spectrum of patients who do not meet
CVID criteria.
Methods Used: We included a total of 1,100 controls and 189 cases of European American ancestry; of which, 189 cases were either CVID, intermediate CVID (ICR), or hypogammaglobulemic (IgG <500) patients. Relative
risk was estimated using odds ratios and corresponding 95% confidence
intervals as implemented in PLINK as well as logistic regression association
model with 10 principal component analysis were used in the analysis. Linkage
disequilibrium was also assessed using Haploview and LocusZoom.
Summary of Results: The strongest gene associations with CVID were
mapped to several loci on chromosomes 2, 6, and 10. Specifically, on chromosome 6, rs2523535, localized to HLA-B, was associated with CVID, ICR, and
hypogammaglobulemia (p=6.5 × 10-6). Within chromosome 10, SNP
rs110112086 lies upstream of microRNA MIR4675 (p=5.9 × 10-8). Within
chromosome 2, SNP rs12998521 lies within IL1R1 gene (p=5.3 × 10-5).
Conclusions: This study exposes new and unexplored regions of interest
within chromosomes 2, 6, and 10 in our CVID and CVID spectrum of
patients. Further study of these areas may show new gene(s) that may contribute to their immunodeficiency.
377
ROLE OF DΒ GERMLINE SEQUENCE ON CONSTRAINING
TCR CDRΒ3 DIVERSITY
Levinson M, Schroeder H, Silva-Sanchez A, Zhuang Y. University of
Alabama at Birmingham, Birmingham, AL.
Purpose of Study: Diversity of the T cell receptor is mainly developed
through V(D)J rearrangement and N addition during TCR development.
The product of V(D)J rearrangement is the CDRβ3, a region of high variability that recognizes antigen and includes all of the D gene. The Dβ sequence is highly conserved across various species, from trout to mouse to
humans. This suggests that there are some natural constraints on the TCR;
these constraints are thought to limit deleterious T cells from reaching the periphery. We hypothesize that altering the D region will have an effect on the
development of thymocytes.
Methods Used: In order to test our hypothesis, mice were generated with an
alteration in their Dβ gene segments. The alterations are a Dβ2 KO (Dβ1), a
replacement of the Dβ locus with a charged Dβ (DβDKRQ), and a replacement of the Dβ locus with a hydrophobic DH (DβYTL). Both developing
thymocytes and mature splenic T cells were sorted from these mice by flow
cytometry based on their expression of either CD44, CD25 and CD28 or
CD4 and CD8. RNA from the thymocytes was extracted and the VDJ-beta
genes were sequenced using primers to the VB13-1 and to the VBC1. In
frame sequences were analyzed using IMGT junction analysis program.
Summary of Results: We have data comparing the T cell repertoires of WT
and altered Dβ mutants. When compared to WT mice, the mutant Dβ mice
have an altered T cell repertoire in both CDRβ3 amino acid composition and
CDRβ3 length and these differences can be attributed to the changes in the
germline sequence. Changing the Dβ also changes the total T cell number
in both developing and mature T cells, with an altered Dβ being selected
against. Preliminary functional data has shown that the T-dependent B cell
response is diminished in the charged Dβ mice.
Conclusions: The Dβ germline sequence is clearly affecting the TCR repertoire. In DβDKRQ mice, the repertoire is skewed toward a shorter, more
charged distribution. In DβYTL mice, CDRβ3 repertoire appears skewed towards a more hydrophobic, longer, BCR-like distribution. However, the altered
TCR repertoire isn’t as hydrophobic as the BCR repertoire, suggesting mechanisms of somatic selection by preferring a less charged CDRβ3. Ongoing
experiments using varied TCR Dβ locus mutants will elucidate the role of the
germline sequence on the development of thymocytes.
379
DIAGNOSTIC CRITERIA FOR MUCOUS RECIRCULATION
SYNDROME OF CHRONIC RHINOSINUSITIS
Patel A, DeShazo R. University of Mississippi Medical Center, Brandon, MS.
Purpose of Study: In clinical practice, nonallergic rhinosinusitis is a diagnosis of exclusion. One incompletely defined condition, masquerading as nonallergic rhinosinusitis, is characterized by persistent post nasal drip and
mucoid rhinorrhea caused by abnormal mucous circulation within the nasopharynx. The underdiagnosis of this condition is due in part to a lack of diagnostic criteria. This investigation sought to better characterize mucus
recirculation syndrome and to establish diagnostic criteria for it.
Methods Used: To develop a study cohort, we first established inclusion criteria and performed a computerized search of the medical literature
published in English using the terms “mucous OR mucous AND recirculation” and “mucoid AND rhinitis”. Clinical data from these (8) patients were
organized and then combined with that of 4 patients with mucous recirculation syndrome from our practice. Data from the cohort were analyzed to develop diagnostic criteria.
Summary of Results: The majority of the study group of 12 patients were
female (58%) with an average age of 53 years. The most common presenting
symptoms were persistent post nasal drip and nasal obstruction. Recirculation through the maxillary sinus occurs in the majority (83%) of the cohort.
The more prevalent mechanism for mucous recirculation was a misplaced
middle metal antrostomy (50%) with recirculation between the natural sinus
ostium and the antrostomy. Recirculation between the natural ostium or
antrostomy and an accessory ostium (25%) was the second most common
mechanism. Mucous recirculation resolved when the abnormal ostium was
enlarged to incorporate the natural ostium.
Conclusions: The diagnosis of mucous recirculation syndrome may be made
if (1) A patient has symptoms of chronic rhinosinusitis characterized by post
nasal drip and nasal obstruction that is refractory medical treatment, (2)
Recirculating mucous is visualized on nasal endoscopy and (3) Symptoms
resolve when the antrostomy or accessory ostea is incorporated into the natural osteum. Mucous recirculation syndrome should be differentiated from
non-allergic rhinosinusitis and considered in patients with chronic nasal
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
519
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
obstruction and mucoid post-nasal drip, especially if previous sinus surgery
has been performed.
380
DEMOGRAPHIC IMPACT ON ASTHMA &
HEALTH-RELATED QUALITY OF LIFE - A PILOT STUDY
Caruthers C1, Shah H1, Solanky T2, Kamboj S1, Kumar P1. 1LSU Health
Sciences Center, New Orleans, LA and 2Univeristy of New Orleans, New
Orleans, LA.
Purpose of Study: Asthma is a chronic inflammatory disorder with significant morbidity and mortality. It is a medically managed disease affected by
proper medication reconciliation. Presumably, the higher the compliance,
the better control of asthma and improved health-related quality of life
(HRQOL). We analyzed the impact of demographic factors on a patient’s
ability to access medications and disease comprehension
Methods Used: IRB approval was obtained. Adults with asthma were studied at Allergy-Immunology clinics. Informed consent was obtained from
all research subjects. Asthma control test (ACT) and HRQOL, via SF-36
standard quality of life scores, were assessed and demographics collected.
Compliance was determined by ascertaining proper medication usage.
Summary of Results: 49 bronchial asthmatic patients were studied - 14 in
charity clinics and 35 in private clinics. Insured patients had mean SF-36
of 42.40 compared to uninsured patients who had mean of 35.94 (p=0.04).
Analysis of the different income levels with ANOVA model showed statistical significance (p=0.01) for SF-36. Upon further analysis between income
levels of less than $25,000 and more than $50,000, the mean was 38.66
and 47.58 (p=0.01). Analysis of clinic location, age and education level were
not statistically significant, although there was a trend in SF-36 and ACT
scores. In private clinics, the mean SF-36 and ACT scores were 42.27 and
16.69, while in charity clinics, 39.02 and 14.64. Older age groups had a
lower SF-36 when comparing younger age group. For patients aged 18-25,
mean SF-36 was 45.33, 25-50 years old was 41.73, and older than 50 was
40.69. Higher education level demonstrated improved SF-36 and ACT
scores. Patients who did not complete high school had mean SF-36 and
ACT scores of 37.90 and 14.14, high school graduates were 39.94 and
15.43, and college graduates were 44.31 and 17.63.
Conclusions: Patients with health insurance and higher income have an improved quality of life and better asthma control. Higher education was associated with better quality of life and asthma control, although the differences
were not statistically significant.
381
CIGARETTE SMOKING, AGE, AND MALE SEX
ARE INDEPENDENT RISK FACTORS FOR
OCULAR SARCOIDOSIS
Walker M1, Janot A1,4, Huscher D2,3, Grewal H1, Yu M1, Lammi MR1, Saketkoo LA1.
1
LSU Health Sciences center, New Orleans, LA; 2CGerman Rheumatism REsearch Centre, Berlin, Germany; 3Charite University Hospital, Berlin, Germany
and 4Medical College of Virginia, Richmond, VA.
Purpose of Study: Sarcoidosis is a multi-organ system granulomatous disease of unknown origin with an incidence of 1-40/100,000. Ocular Sarcoidosis (OS) affects 25-50% of patients with sarcoidosis and can lead to
blindness. To date, no studies have sought to determine risk factors for OS.
Methods Used: A retrospective chart review was conducted at a single institution with inclusion criteria of biopsy-proven sarcoidosis with a duration of
>1 year and a documented smoking status. Variables collected were ages at
time of diagnosis (of any organ) and at time of chart review, race, sex, smoking status, quantity of tobacco exposure, and presence (or history) of OS.
Disease duration is defined as the difference of age at diagnosis and age time
of chart review. Group comparisons were done by t-test and by MannWhitney tests where applicable. Univariate and multivariate regression analysis were done to identify independent risk factors.
Summary of Results: Of 269 charts reviewed, 109 patients met inclusion
criteria. In the OS group, the portion of smokers (71.4%) was significantly
higher than in the group without OS (42.0%, p=0.027). There was no significant difference (p=0.605) in the median number of pack years between
smokers with OS (13 [7, 28]) and those without OS (16.5 [7, 25]).
520
Furthermore, the OS group consisted of more male patients (57.1% versus
26.1%, p=0.009). Median disease duration of sarcoidosis was also higher
in patients with OS (10 years versus 4 years, p=0.031). Through multivariate
regression analysis, tobacco exposure (OR=5.24, p=0.007, 95% CI 1.5817.41), male sex (OR=7.48, p=0.002, 95% CI 2.152-26.006), and age at time
of chart review (OR=1.114, p=0.002, 95% CI 1.04-1.19) were found to be
independent risk factors for OS.
Conclusions: Male sex, age at chart review, and tobacco exposure (regardless of pack years) were found to be independent risk factors for OS. Disease
duration did not withstand multivariate analysis in this moderately sized
group. However, screening for OS should not remit in patients with known
sarcoidosis until defined in larger prospective populations.
382
SMOKING AND SECONDHAND SMOKE AMONG
PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS
AND CONTROLS: ASSOCIATIONS WITH DISEASE
AND DISEASE DAMAGE
Minkin SJ, Slan SN, Gilkeson G, Kamen D. Medical University of South
Carolina, Charleston, SC.
Purpose of Study: Previous reports suggest smoking may be a risk factor for
developing systemic lupus erythematosus (SLE). This study explores the impact of tobacco smoke on SLE patients compared to controls and on disease
characteristics among patients.
Methods Used: Data from a cohort of SLE patients and controls was utilized. Medical history, smoking and secondhand smoke exposure history,
SLE Disease Activity Index (SLEDAI) and SLICC-Damage Index (SDI)
scores were collected at an in-person enrollment visit and confirmed by chart
review. Statistical analysis used chi square testing and multivariate logistic
regression.
Summary of Results: There were 545 SLE patients and 386 controls with
data available for analysis (Table). At enrollment, the mean age was 37.6 +/14.7 years for patients and 42.0 +/- 15.4 years for controls. Differences between
current and never smokers (p=0.51) and ever and never smokers (p=0.70) were
not significantly different between patients and controls. Compared to unrelated
controls, African American patients were significantly more likely to be exposed in the home to secondhand smoke before the age of 18 (OR 1.81, 95%
CI 1.13-2.89).
Damage by SDI (SDI > 0) was significantly associated with ever smoking
(OR 3.08, 95% CI 1.4-6.6), current smoking (OR 3.17, 95% CI 1.1-9.1), and
secondhand smoke exposure in childhood (OR 1.91, 95% CI 1.0-3.6). No
significant relationship was found between smoking status and active disease
at enrollment (SLEDAI ≥ 6) or dsDNA autoantibodies. Discoid rash was significantly associated with ever smoking (OR 2.74, 95% CI 1.5-5.1) and current
smoking (OR 4.85, 95% CI 2.2-10.5).
Conclusions: Our study suggests that secondhand smoke during childhood
may be a risk factor for SLE. Secondhand smoke during childhood, current
smoking and past smoking contribute significantly to disease damage among
patients with SLE.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Cardiovascular I
Concurrent Session
Southern Regional Meeting Abstracts
important for EH than for CH, indicating the importance of preventive strategies for LV hypertrophy in early life.
2:00 PM
Friday, February 21, 2014
383
VARIATION IN GEOGRAPHIC DISTRIBUTION OF
IN-HOSPITAL MORTALITY ASSOCIATED WITH ACUTE
MYOCARDIAL INFARCTION: A NATIONWIDE ANALYSIS
Pant S1, Deshmukh A1, Mehta K2, Mehta JL1. 1UAMS, Little Rock, AR and
2
University of Pittsburgh Medical Center, Pittsburgh, PA.
Purpose of Study: Mortality from acute myocardial infarction (AMI) has
significantly declined nationwide due to various reasons. Data regarding geographic variation in mortality is however limited.
Methods Used: Using the Nationwide Inpatient Sample (NIS), we investigated the trends in in-hospital mortality, risk factors and procedures
performed in different regions of the country from 2000-2010. The hospital’s
census region was obtained from the AHA Annual Survey of Hospitals.
Summary of Results: There were a total of12,958,452 cases of AMI from
2000 to 2010. We noted an overall decline in mortality in all regions over
this time span. However, the overall in-hospital mortality per 100,000 cases
was highest in the South followed by Mid-west and North-east, with least
in the West (Figure 1). Baseline characteristics analysis showed significantly
higher occurrence of traditional risk factors (diabetes mellitus, hypertension,
smoking, obesity and hyperlipidemia) in the South compared to other
regions (Table 1). Median household income was significantly lower in this
region as indicated by higher distribution of quartile 1 and quartile 2 incomes
(Table 1). Finally, racial variation analysis showed higher race-specific mortality in the South compared to the rest (Figure 2). This race-wise mortality
variation in the South is more pronounced among African-American and
Hispanics (Figure 2).
Conclusions: Although mortality from AMI is trending down across all
regions of the US, it is disproportionately higher in the South, likely due to uneven distribution of socio-economic and traditional risk factors in the South.
384
IMPACT OF LONG-TERM BURDEN OF OBESITY AND
ELEVATED BLOOD PRESSURE ON LEFT VENTRICULAR
REMODELING PATTERNS: THE BOGALUSA HEART STUDY
Lai C1,2, Cen R1, Li S1, Fernandez C1, Chen W1, Srinivasan SR1, Berenson GS1.
1
Tulane University, New Orleans, LA and 2Peking Union Medical College Hospital, Beijing, China.
Purpose of Study: This study assessed the hypothesis that long-term cumulative burden and trends of obesity and elevated blood pressure (BP) from
childhood are associated with adult left ventricular (LV) hypertrophy and
geometric remodeling patterns.
Methods Used: The longitudinal study cohort consisted of 1061 adults (717
whites and 344 blacks, aged 24 to 46 years) who have been examined 4 or
more times for BMI and BP from childhood to adulthood, with an average
follow-up period of 28.0 years. The area under the curve (AUC) was calculated as a measure of long-term burden (total AUC) and trends (incremental
AUC) of BMI and BP. Four LV geometric types were defined as normal, concentric remodeling (CR), eccentric (EH) and concentric hypertrophy (CH)
using LV mass index and relative wall thickness.
Summary of Results: Compared with whites, blacks had higher prevalence
of eccentric (17.7% vs 8.9%, p <0.001) and concentric (9.1% vs 3.5%,
p <0.001) hypertrophy. Higher values of BMI, systolic (SBP) and diastolic
(DBP) blood pressure of childhood, adulthood, total AUC and incremental
AUC were all significantly associated with higher LV mass index and LV hypertrophy in linear and logistic regression analyses, respectively, when adjusted for race, sex age and lipid variables. The association of geometric
remodeling patterns with BMI and SBP is shown in the table below. Of interest, all values of BMI had a consistently greater influence on EH than SBP.
DBP showed similar association parameters with LV remodeling patterns.
Conclusions: The findings suggest that adverse influence of obesity and elevated BP levels on LV hypertrophy begins in childhood, and obesity is more
OR different from 1: *p<0.05, **p<0.01, † p=0.08.
385
THE PREVALENCE OF DIASTOLIC DYSFUNCTION AMONG
PATENTS WHO UNDERGO ECHOCARDIOGRAMS AND ITS
SIGNIFICANCE - DIABETES IS A STRONG CORRELATE OF
DIASTOLIC DYSFUNCTION
Panikkath R, Jenkins L. Texas Tech University Health Sciences Center,
Lubbock, Lubbock, TX.
Purpose of Study: The prevalence and progression of diastolic dysfunction
among patients who undergo echocardiography in an echocardiographic center is not known
Methods Used: This study includes all adult patients (>20years) with diastolic dysfunction who had an echocardiogram at a university hospital echocardiographic center in January 2012. If the subject had a previous
echocardiogram, the oldest one was evaluated for the presence and severity
of LV diastolic dysfunction. Controls were subjects with normal systolic
and diastolic function in absence of exclusion criteria.
Summary of Results: Of the 483 consecutive subjects who underwent echocardiography, 223 had LV diastolic dysfunction. Of those, 190 (39.4%)
subjects met the inclusion criteria. Mean age of cases and controls was
67.7 ± 13.4 years (50.5% males) and 54 ± 15 (57% males) respectively
(p=0.01 for age & 0.67 for sex). Diabetes (43.7% vs. 22.5%; p∼0.001), hypertension 85.7% vs. 70.1%; p =0.02) and coronary artery disease (44.2%
vs. 35.0%; p=0.06) were the common comorbidities in cases and controls, respectively. In logistic regression, only age [odds ratio (OR) 1.05 (95% CI
1.01-1.10)] and diabetes [odds ratio (OR) 61.22 (95% CI 12.60-120.56) were
significantly associated with diastolic dysfunction. Only 20 (10.5%) patients
with diastolic dysfunction had been diagnosed with congestive heart failure.
Seventy one subjects with diastolic dysfunction had a previous echocardiogram available. Over a mean duration of 49.5 ± 35.4 months, the diastolic
dysfunction remained the same in 46 (64.8%), progressed by one grade in
17 (23.9%) and two grades in 4 (5.6%) patients. In logistic regression, hypertension [odds ratio (OR) 1.1 (95% CI 0.1-12.7)], diabetes [OR 0.45 (95% CI
0.2-1.4)] or BMI [OR 0.99 (95% CI 0.92-1.1)] were not significantly associated with progression of diastolic dysfunction.
Conclusions: Diastolic dysfunction in the presence of normal systolic function was seen in 39.4% of adult patients who underwent echocardiograms. Of
those, only 10% had a diagnosis of diastolic heart failure. Diabetes is a
strong correlate of diastolic dysfunction. In the subjects who had a previous
echocardiogram, the majority had no progression of diastolic dysfunction
over a mean duration of 4 years.
386
THE EFFECT OF KIDNEY TRANSPLANTATION ON
LEFT VENTRICULAR SYSTOLIC FUNCTION AND
MECHANICAL DYSSYNCHRONY BY GATED
MYOCARDIAL PERFUSION IMAGING
Crosland W, Aggarwal H, Mehta S, Mannon R, Heo J, Iskandrian A, Hage F.
University of Alabama-Birmingham, Birmingham, AL.
Purpose of Study: We have previously demonstrated that patients with
end-stage renal disease (ESRD) and left ventricular mechanical dyssynchrony (LVD) have a worse prognosis and phase analysis readily evaluates
LVD via gated SPECT myocardial perfusion imaging (MPI). Whether kidney transplantation influences mechanical function and dyssynchrony
remains unclear.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
521
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Methods Used: We identified patients from the renal transplantation database maintained at the University of Alabama at Birmingham who underwent MPI for clinical indications before and after renal transplantation and
who had normal perfusion on both sets of stress imaging. Patients with
ESRD at time of second MPI were excluded. MPIs were reprocessed to derive LV ejection fraction (EF), and LVD indices (phase bandwidth [BW]
and standard deviation [SD]). The primary outcomes were change in LVEF,
BW, and SD before and after transplantation.
Summary of Results: The study population consisted of 30 patients (55%
male, 51 ± 12 years, 55% African American, 61% diabetic). Of these, 12
(40%) had not initiated dialysis prior to first MPI and the rest had initiated
dialysis for 2±2 years. The second MPI was performed at 3 ± 2 years after
transplantation. LVEF (72 ± 10% vs. 67 ± 11%, p = 0.002) improved after
transplantation but not phase SD (21.6 ± 14.3° vs. 21.5 ± 11.4°, p = 0.98)
or BW (58.2 ± 36.0° vs. 57.6 ± 29.6°, p = 0.93). On pre-transplant MPI, 9
(27%), 14 (42%), and 15 (46%) had abnormal LVEF, SD, and BW, respectively. The corresponding numbers after transplantation were 3 (9%), 12
(36%), and 14 (42%), respectively.
Conclusions: This study shows that renal transplantation favorably
influences LVEF but does not improve abnormal LVD indices.
387
(2 mg/kg/day) for 28 days, the hearts were isolated and used for quantification of fibrotic markers using real time quantitative reverse transcriptionpolymerase chain reaction (qRT-PCR) and Western blot analyses. Together,
systolic blood pressure (SBP), heart weight-to-body weight (HW/BW) ratio, left ventricular end-diastolic dimension (LVEDD), left ventricular
end-systolic dimension (LVEDS), and percent fractional shortening (FS)
were analyzed.
Summary of Results: The Npr1-/- null mutant mice showed 6-fold induction of fibrosis in the hearts. The expression of a number of fibrotic markers
was markedly induced, including connective tissue growth factor (CTGF,
5-fold), α-smooth muscle actin (α-SMA, 4-fold) and TGF-βRI (4-fold), TGFβRII (3.5-fold) and SMAD proteins (SMAD-2, 5-fold; SMAD-3, 3-fold) in
Npr1-/- mice hearts compared with wild-type control mice. The treatment with
TGF-β inhibitor, GW788388, significantly prevented the development of cardiac fibrosis and potentially down-regulated the expression of fibrotic markers
and SMAD proteins in Npr1-/- mice compared to vehicle-treated wild-type
animals.
Conclusions: The results of the present study suggest that the development
of cardiac fibrosis in Npr1-/- gene-disrupted mice might be regulated through
TGF-β mediated SMAD-dependent pathways.
389
EFFECT OF DECREASED RENAL FUNCTION ON LONG
TERM SURVIVAL IN PATIENTS WITH LEFT ANTERIOR
DESCENDING CORONARY DISEASE
Bissett JK. Central Ark VA Hosp, Little Rock, AR.
Purpose of Study: The study was designed to compare the results of therapy
for left anterior descending disease in patients with and without renal disease. Despite the presence of stenosis of a major coronary artery these
patients would usually be excluded from randomized clinical trials.
Methods Used: A computerized medical record system was used to follow
the clinical course of 100 patients with left anterior descending disease form
2003-2013. The system documents and allows review from multiple
hospitals throughout the United States. The patients were divided into 2
groups on the basis of a glomerular filtration rate of 60 millimeters per minute per 1.73 meters squared. Coronary angioplasty, coronary bypass surgery,
and measurement of fractional flow reserve were applied when appropriate.
The patients were placed on standard medications and treated by primary
care, nephrology, and cardiology and offered pacemakers, defibrillators,
and dialysis as needed. No patient was excluded from data collection for
any reason.
Summary of Results: (1) In this pilot study of a small group of patients in a
national health care system renal disease was a significant predictor of mortality with a 10 year hazard ratio of 3.39 (confidence interval 1.68-6.85,
p<0.01) (2)Death occurred in 22/35 patients with a reduced glomerular filtration rate at baseline versus 19/46 patients with normal renal runction
(p<0.01). (3) These results include all deaths from multiple medical
conditions.
Conclusions: (1) Patients with coronary disease with left anterior descending
stenosis can experience increased mortality from renal disease despite medical
and interventional cardiac therapy (2) Additional studies are needed to design
methods for preservation of renal function in patients with vascular disease.
388
DISRUPTION OF GUANYLYL CYCLASE/NATRIURETIC
PEPTIDE RECEPTOR-A GENE INDUCES CARDIAC FIBROSIS
THROUGH TGF-Β/SMAD DEPENDENT PATHWAY
ADVANCED GLYCATION END PRODUCTS (AGES) AND
ASSOCIATIONS WITH SYSTEMIC LUPUS ERYTHEMATOSUS
Toney MM, Kamen D. Medical University of South Carolina, Charleston, SC.
Purpose of Study: Oxidative stress plays a role in disease activity and premature atherosclerosis seen in patients with systemic lupus erythematosus
(SLE). Advanced glycation end products (AGEs) are prevalent in the Western diet. The accumulation of serum AGEs disrupts protein function, and interaction with its receptor induces production of reactive oxygen species and
activation of vascular endothelial cells, leading to increased oxidative stress.
Our hypothesis is that serum AGE levels, indicators of dietary habits, correlate with inflammation and potentially impact autoimmunity.
Methods Used: Serum samples from 80 Gullah African American participants recruited prospectively into the SLE in Gullah Health (SLEIGH)
study were assayed with AGE Competitive ELISA kit from Cell Bio Labs.
The cohort consisted of 50 patients (25 with no history of cardiovascular disease, and 25 with history of cardiovascular disease or renal disease) and 30
controls (15 related controls, 15 unrelated healthy controls). Student’s T-test
was used to compare AGE levels among SLE patients to controls. We used
logistic regression models to examine predictors of autoantibody status and
a nested case-control design to compare AGE levels between different disease subsets of patients.
Summary of Results: As shown Table 1, mean AGE levels were higher in
the control group than the patient group. Interestingly, among controls,
ANA positivity significantly correlated with higher AGE levels (p=0.0008),
even when adjusted for age (years) (OR 1.09, 95% CI 1.02-1.17, p=0.011).
Among patients, smoking status (p=0.004) and hydroxychloroquine
(p=0.016) use were consistent with higher AGE levels and statistically significant. Though not statistically significant, history of stroke among patients
correlated with higher AGE levels (p= 0.08).
Conclusions: Although there was not a statistically significant difference in
AGE levels between SLE patients and controls, the AGE levels were significantly higher with ANA positivity among controls. This finding suggests
that serum AGE levels may play a significant role in autoimmunity and
warrants further study.
Subramanian U, Gogulamudi V, Pandey KN. Tulane University, New
Orleans, LA.
Purpose of Study: Targeted-disruption of guanylyl cyclase/natriuretic peptide
receptor-A (GC-A/NPRA) gene (Npr1) in mice provokes high blood pressure,
cardiac hypertrophy, fibrosis, and remodeling associated with congestive heart
failure. The objective of this study was to determine the mechanisms regulating
the development of fibrosis in Npr1 gene-disrupted mice hearts.
Methods Used: The Npr1 null mutant (Npr1-/-, 0-copy), heterozygous
(Npr1+/-, 1-copy), and wild-type (Npr1+/+, 2-copy) mice were orally administered with transforming growth factor-β1 receptor inhibitor GW788388
522
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
390
INSULIN-LIKE GROWTH FACTOR 1 REGULATES LIPID
ACCUMULATION IN MACROPHAGES - A POTENTIAL
MECHANISM FOR INSULIN-LIKE GROWTH FACTOR 1
MEDIATED ATHEROPROTECTION
Higashi Y, Shai S, Sukhanov S, Kim C, Snarski P, Delafontaine P. Tulane
University, New Orleans, LA.
Purpose of Study: Lipid accumulation and foam cell formation is a hallmark
of atherogenesis, whereas apoptosis of foam cells leads to necrotic core formation and plaque vulnerability. Insulin-like growth factor-1 (IGF-1) is antiatherogenic, and loss of IGF-1 signaling in macrophages not only increases
plaque burden, but also induces a vulnerable plaque phenotype with enlarged
necrotic cores and thin fibrous caps. We investigated potential mechanisms
for accelerated atherosclerosis in macrophage specific IGF-1R knockout
mice (MΦIgf1rKO).
Methods Used: MΦIgf1rKO was created on Apoe-deficient background.
Animals were fed on a high-fat diet for 8 wks, and atherosclerotic burden
and aortic gene expressions were assessed. Peritoneal macrophages were isolated and assessed for lipid transport activities.
Summary of Results: MΦIgf1rKO had an increase in atherosclerotic burden
(57±15% increase by aortic sinus cross-section analysis vs. Igf1r floxed
mice, n=8, p<0.05), and plaque composition was altered: MΦIgf1rKO had
an increased macrophage content (Mac3 staining, by 39±13%, P<0.05) and
a decrease in smooth muscle cell content (α-smooth muscle actin staining,
by 34±17%, P<0.05). Aortic gene expression analysis showed that
MΦIgf1rKO downregulated ABCA1 and ABCG1 (by 32±14% and 45
±19%, P<0.05, n=10), transporters responsible for lipid efflux from
macrophages, and upregulated ACAT-1 and DGAT-2 (by 58±16% and 115
±55%, P<0.05, n=10), lipid metabolism enzymes responsible for lipid storage; however MΦIgf1rKO did not alter Msr1, CD36, or LOX-1 expression.
Furthermore, MΦIgf1rKO suppressed PPARγ and LXRβ (by 47.6±2.5%
and 43.0±10.8%, P<0.01), consistent with changes in expression of lipid
transport and lipid metabolism related genes. In cultured macrophages we
assessed the effect of IGF-1 on lipid transport and found that MΦIgf1rKOmacrophage shows reduced lipid efflux to apolipoprotein A1 and HDL
(P<0.01).
Conclusions: Our findings suggest that loss of IGF-1 signaling in macrophages promotes lipid accumulation, thereby enhancing foam cell formation and necrotic core enlargement, which account for a vulnerable plaque
phenotype. IGF-1 regulation of lipid accumulation provides a novel therapeutic strategy to treat atherosclerotic lesions and to promote plaque
stabilization.
Southern Regional Meeting Abstracts
placebo (ΔR: 0.064 ± 0.31; ΔP: -0.045 ± 0.26, p=0.03). Paired comparisons
of baseline vs. treatment show an increase in the rosi arm (0.87 ± 0.54 vs.
0.93 ± 0.51, p 0.005) but no change in the placebo arm (0.79 ± 0.51 vs.
0.74 ± 0.51, p 0.83) (Figure 1).
Conclusions: These findings from a randomized placebo-controlled trial
suggest that activation of PPAR γ increased CEC and that the effect of TZDs
on atherosclerosis may be mediated by their effects on cholesterol efflux.
FIGURE 1 Normalized cholesterol efflux at baseline and after 6
months of treatment
392
HIGH-SENSITIVITY TROPONIN FOR DIAGNOSIS OF
OBSTRUCTIVE CORONARY ARTERY DISEASE
Siegfried JS, Panagopoulos G, Garratt KN, Coplan NL. Lenox Hill Hospital,
New York, NY.
Purpose of Study: Small elevations in troponin values may be caused by
several clinical conditions. In urgent care settings such elevations often lead
to a presumptive diagnosis of active coronary artery disease (CAD). We
sought to determine the relationship between small troponin elevations and
presence of significant CAD.
Methods Used: Records were reviewed for 250 consecutive patients admitted from the ED with suspected cardiac ischemia who underwent cardiac
catheterization during hospitalization. Unstable patients and those with
STEMI or creatinine >2.0 were excluded. Data collected included high sensitivity troponin I level and presence of significant obstructive CAD (defined
as ≥70% stenosis in an epicardial vessel or equivalent criteria), as well as
data regarding cardiac function and risk factors. Quantitative assessment
was performed using computerized quantitative coronary angiography
(QCA). The data were used to generate a receiver operating curve (ROC)
for use of troponin to diagnose obstructive CAD.
Summary of Results: A troponin above the lowest detectable level, 0.015
ng/mL, had a sensitivity of 71.3% and specificity of 45.8% for detection of
an obstructive coronary lesion. Specificity of 80.4%, 89.7% and 95.3%
was achieved at troponin 0.075, 0.437 and 4.485 with sensitivity of 44.8%,
30.8% and 19.6% respectively. The area under the ROC was 0.67. Presence
of obstructive CAD, increasing age, left ventricular end diastolic pressure,
lower ejection fraction, and degree of stenosis by QCA were all significantly
correlated with elevated troponin. Logistic regression analysis showed that
when adjusted for risk factors and cardiac function, troponin remained a
weak but significant predictor of obstructive CAD (OR 1.146, 95% CI
[1.021-1.287], p=0.021).
Conclusions: Troponin is a relatively sensitive marker for the presence of
obstructive CAD and is fairly specific at low levels of elevation. An undetectable troponin does not rule out the presence of a significant coronary lesion
and further testing may be required to delineate disease. Patients being evaluated for suspected cardiac ischemia, even with very small troponin
elevations, should be evaluated thoroughly and effectively for active CAD.
391
ROSIGLITAZONE INCREASES CHOLESTEROL EFFLUX
CAPACITY IN PATIENTS WITH TYPE 2 DIABETES
393
Agarwala A, Givens E, McGuire DK, De Lemos JA, Rohatgi A. UT Southwestern Medical Center, Dallas, TX.
Purpose of Study: Thiazolidinediones (TZDs) are medications used in
patients with type 2 diabetes (T2DM) that activate peroxisome proliferatoractivated receptors (PPAR) γ, thereby improving insulin sensitivity and
glycemic control. In most patients, treatment with TZDs increases HDL-C,
suggesting that TZDs may have effects on atherosclerosis via the reverse
cholesterol transport (RCT) pathway. A few small reports without placebo
control have shown that TZDs may increase macrophage-specific cholesterol
efflux capacity (CEC), the first critical step in RCT. We tested the hypothesis
that TZDs, specifically rosiglitazone (rosi), increase CEC in patients with
T2DM in a placebo-controlled trial.
Methods Used: 111 patients (57 rosi, 56 placebo) with T2DM and either the
presence of coronary disease or strong risk factors for coronary disease were
randomized to double-blinded treatment with rosi vs. placebo for 6 months.
CEC was measured at baseline and after 6 months treatment.
Summary of Results: Preliminary results from the first 65 patients (30 rosi,
35 placebo) show increased mean CEC in patients randomized to rosi vs.
THE PROGNOSTIC VALUE OF TRANSIENT ISCHEMIC
DILATION OF THE LEFT VENTRICLE WITH
REGADENOSON MYOCARDIAL PERFUSION IMAGING
Lester D, Bleich S, El-Hajj S, Heo J, Iskandrian A, Hage F. University of
Alabama School of Medicine, Birmingham, AL.
Purpose of Study: Transient ischemic dilation (TID) of the left ventricle on
myocardial perfusion imaging (MPI) is generally considered a sensitive and
specific marker of severe and extensive coronary artery disease and is
thought to provide important prognostic information. The prognostic value
of TID obtained using regadenoson, a selective adenosine A2A receptor agonist, as a stress agent for MPI has not been well studied.
Methods Used: An automated software program was used to measure TID
on consecutive patients with normal and abnormal perfusion pattern who underwent regadenoson MPI at our institution. The primary outcome was allcause death.
Summary of Results: The study population consisted of 199 patients (44%
male, 59±12 years, 37% diabetes, 13% with coronary revascularization) with
normal and 688 patients (65%, 63±12 years, 51%, 53%) with abnormal
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Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
523
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
perfusion pattern. TID was not different between the 2 groups (1.04±0.15 vs.
1.06±0.01, p=NS). None of the patients with normal perfusion pattern underwent revascularization within 90 days of index MPI. The proportion of
patients with abnormal perfusion that underwent early revascularization increased with increasing quartiles of TID (11%, 13%, 16%, 23%, p=0.02).
During a follow-up of 44±17 months, there were 51 (26%) and 229 (33%)
deaths among patients with normal and abnormal perfusion, respectively.
In patients that did not undergo early revascularization TID was not associated with overall mortality whether perfusion was normal or abnormal (Figure). These findings were unchanged in patients with diabetes or history of
coronary revascularization.
Conclusions: TID to regadenoson is associated with early referral to coronary revascularization but not with long-term mortality.
Gastroenterology I
Concurrent Session
2:00 PM
Friday, February 21, 2014
394
EFFECT OF COLON TRANSECTION ON
SPONTANEOUS HIGH-AMPLITUDE-PROPAGATING
CONTRACTIONS IN CHILDREN
Jacobs C1, Wolfson S2, Di Lorenzo C2, Cocjin J2, Monagas J2, Hyman P2,1.
1
Louisiana State University, New Orleans, LA and 2Children’s Hospital, New
Orleans, LA.
Purpose of Study: After Hirschsprung’s disease (HD) surgery, many children suffer fecal incontinence caused by increased high amplitude propagating contractions (HAPCs). The aim of this study was to determine whether
children with HD have more HAPCs than children with colon transections
for reasons other than HD.
Methods Used: We reviewed 500 colon manometries. Children (7.6±5.1 yrs;
275 male) with functional constipation (n=237; 7.4±5.0yrs; 126 male) and
chronic abdominal pain (n=48; 9.8±5.8yrs; 25 male) served as controls compared to subjects with HD (n=56; 6.9±4.1yrs; 44 male) and colon transection
for other reasons (n=24; 6.1±5.8yrs; 12 male). We excluded 139 subjects
without HAPCs. We documented HAPCs during 1 h fasting and 1 h postprandial. Results are mean ± SD.
Summary of Results: During fasting, HD subjects had more HAPCs (2.2±3.4/h)
vs. functional constipation (0.8±2.2/h, p=0.0004) and chronic pain (0.5±1.1/h,
p=0.001), but not more than colon transection (1.9±3.2/h, p=1.0). HD showed
more postprandial HAPCs (4.0±5.4/h) than functional constipation (1.5±2.5/h,
p<0.0001) and chronic pain (0.9±1.6/h, p<0.0001), but not more than colon
transection (2.4±3.0/h, p=0.6). There were more HAPCs fasting and post-prandial after colon transection (1.9 ± 3.2/h and 2.4±3.0/h) than functional constipation (0.8±2.2/h, p=0.3 and 1.5±2.5/h, p=1.0) and chronic pain (0.5±1.1/h, p=1.0
and 0.9±1.6, p=1.0). HD subjects were divided by chief complaint: fecal incontinence or constipation. HD subjects with incontinence (n=23) only had more
HAPCs fasting (p=0.01) and post-prandial (p=0.01) than HD subjects with constipation (n=28) only.
524
Conclusions: Increased HAPCs followed colon transection, regardless of
cause. HD subjects with incontinence had more HAPCs than subjects with
colon transection for other reasons.
395
INCREASED RATE OF GASTRIC EMPTYING AFTER
PYLOROPLASTY CORRELATES WITH REDUCTION OF
GASTROPARETIC SYMPTOMS
Onate EJ1, Sarosiek I1, Liu K2, Dwivedi AK2, McCallum R1. 1Texas Tech
University Health Sciences Center, El Paso, TX and 2Texas Tech University
Health Sciences Center, El Paso, TX.
Purpose of Study: It is suggested that there is a poor correlation between
gastroparetic (GP) symptoms and gastric emptying time (GET). Gastric
Electrical Stimulation (GES) improves GP symptoms, but there is a minimal
or no effect on acceleration of GET. Hence we have added pyloroplasty (PP)
as a surgical procedure in GP patients receiving GES. Our Aim was to assess
the correlation of changes in GET in GES patients after PP with improvement of GP symptoms.
Methods Used: Seventeen (13F) drug refractory GP patients (13- Post-Surgical,
4-Idipoathic) received PP with GES. Total GP symptoms scores (TSS)
assessing severity (S) and frequency (F) of nausea, early satiety, bloating,
vomiting, postprandial fullness, epigastric pain and burning were obtained by
using a 5-point Likert scale at baseline and at last follow up visit, a mean of
6 months (3–18). The GETwas conducted before and after surgery with GP defined as > 60% retention of isotope at 2 hrs and >10% at 4 hrs. Paired t-test, oneway ANOVA and Pearson correlation coefficient was used to analyze data.
Summary of Results: GES & PP improved severity of TSS from 20 to
11points and frequency from 21 to 12 (P<0.001). GET retention was reduced
from 75% to 39% at 2hrs and from 47% to 12 at 4hrs (P<0.001). There was a
significant correlation between reduction in severity and frequency of postprandial fullness with acceleration in GET: (p=0.032) and (p=0.022) respectively. There was also a strong correlation between reduction in frequency of
vomiting, epigastric pain, and epigastric burning and faster 2 hrs GET:
(p=0.051), (p=0.024), (p=0.040) respectively. Additionally, there was a significant correlation of improvement in S and F of nausea and early satiety with
changes in GET at 4 hrs: nausea- S (p=0.043), F (p=0.067); early satiety-S
(p=0.03) and F (p=0.031).
Conclusions: 1) Overall, in drug-refractory GP the addition of PP to GES
markedly accelerated gastric emptying, while also significantly improving
GP symptoms; 2) The increased rate of gastric emptying after PP correlated
with improvements in many GP symptoms. 3) GET remains a valuable
marker for diagnosis and management of GP patients.
396
HISTOLOGICAL EVIDENCE OF INFLAMMATORY
CHANGES IN THE MUSCULARIS PROPRIA OF GASTRIC
SMOOTH MUSCLE IN IDIOPATHIC GASTROPARESIS
PATIENTS
Saadi M1, Torabi A2, Sunny J1, Sarosiek I1, Davis BR3, Moraveji S1, McCallum R1.
1
TTUHSC- El Paso, El Paso, TX; 2TTUHSC- El Paso, El Paso, TX and
3
TTUHSC- El Paso, El Paso, TX.
Purpose of Study: Idiopathic gastroparesis (ID-GP) patients are thought to
represent a heterogenous population. Recent data indicating structural
changes in their gastric smooth muscle with reduced numbers of interstitial
cells of Cajal (ICC) and enteric neurons as well as increased immune
infiltrates in the myenteric plexus suggest a gastroenteritis-infection based
etiology. Our aim was to investigate inflammatory cell markers on full thickness surgical biopsies of the antrum in ID-GP patients.
Methods Used: Five female patients, aged 19 to 58 years, with ID-GP
who underwent full thickness gastric (antrum) biopsy during surgery to
place the gastric electrical stimulation system and pyloroplasty. Similar biopsies were obtained at surgery from 4 female non-gastroparetic chronically vomiting patients (“vomiting controls”), aged 19 to 61 years, with
superior mesenteric artery syndrome (3) and Rumination Syndrome (1).
The biopsy samples were stained with H&E, trichrome for fibrosis, S100
for myenteric plexus, and C- Kit (CD117) for ICC and examined under
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
light microscopy for the presence of inflammatory infiltrate, macrophages
or fibrosis in the myenteric plexus.
Summary of Results: See Table:
Two out of the five ID-GP patients (40%) had reduced ICC numbers, and
two had an infiltrate of neutrophils in and around the nerve bundles. One had
evidence of apoptotic neuronal changes. There were no inflammatory cells
identified in the biopsies from the “vomiting control” patients.
Conclusions: 1) This study shows that cellular abnormalities are found in
the gastric muscularis propria in most ID-GP patients (4 out of 5). 2) The
neutrophil infiltrates in the myenteric plexus observed in two patients have
not been previously described and provide more support for the concept that
post-infection neural injury could be the underlying pathophysiology in
patients termed as “idiopathic” gastroparetics.
397
CORRELATION OF SIGMOIDOSCOPY FINDINGS IN
CHILDREN WITH MILK PROTEIN ALLERGY
Patel B. University of South Alabama, Mobile, AL.
Purpose of Study: Milk protein allergy, an immunologic reaction to cow’s
milk proteins, is becoming a chronic problem in children upto one year of
age. It affects up to 3% of children in the United States of America. It can
cause skin, lung, and gastrointestinal manifestations via IgE or non-IgE mediated response. The objective of this study was to determine if children less
than one year of age who presented with symptoms of milk protein allergy
had positive sigmoidoscopy findings. In addition, we sought to correlate
the symptoms of milk protein allergy with sigmoidoscopy findings of
lymphonodular hyperplasia (LNH) and eosinophils count per high power
field (eosinophils/hpf) in the rectosigmoid.
Methods Used: This was a retrospective chart review of patients referred
to the University of South Alabama Pediatric Gastroenterology clinic from
outlying clinics with symptoms of milk protein allergy including blood per
rectum, fussiness, vomiting, gas/constipation, poor weight gain, wheezing
and rash.
Summary of Results: Most children were less than six months old and there
was an even distribution of males and females included in this study. The
commonly reported symptoms were fussiness, vomiting, constipation and
rectal bleeding. Our study was divided into two groups, those with microscopic or macroscopic rectal bleeding and those without. The LNH and eosinophil/hpf were analyzed in these two groups. It was found that patients
with rectal bleeding had the highest number of eosinophils/hpf. However,
there was no statistical significance between the number of eosinophils/hpf
or presence of LNH in either group.
Conclusions: Vomiting was the most common symptom associated with eosinophil/hpf and wheezing was the least. While 63% of the patients in both
the rectal bleeding and non rectal bleeding groups were on a protein hydrolysate formula prior to their sigmoidoscopy, they had lower eosinophils/
hpf, and the other 37% of patients who were on non protein hydrolysate formulas (excluding amino acid formulas) had higher eosinophils/hpf. Thus we
conclude that sigmoidoscopy may not be useful in infants who have already
been on protein hydrolysate formulas. Further studies are needed to look for
causes of LNH, as it may be due to other cells involved such as T-cells. Future studies may show that these cells instead of eosinophils/hpf in food hypersensitivity reactions may be of better diagnostic value.
398
SIGNIFICANT GASTROESOPHAGEAL REFLUX
DISEASE IN INFANCY IS ASSOCIATED WITH
ESOPHAGEAL REFLUX IN CHILDHOOD
Ramasamy P, Gremse D. University of South Alabama Children’s & Women’s
Hospital, Mobile, AL.
Southern Regional Meeting Abstracts
Purpose of Study: Limited available evidence suggests that presence of gastroesophageal reflux disease (GERD) in infancy may be associated with GERD
symptoms later in life (Winter et al., Scand J Gastroenterol 2011;46:1157-68).
Our aim is to determine the incidence of GERD in children who had GERD during infancy confirmed by esophageal pH monitoring.
Methods Used: Infants born between 1990 through 2008 who underwent
esophageal pH study were followed for GERD symptoms during childhood.
Infants with GERD who had no follow up records or who had co-morbidities
were excluded from further analysis. Data were collected from query of electronic patient database. The presence of GERD in children who had an abnormal pH study in infancy was compared to those with normal pH study
results. Continuous variables were compared using student’s t-test & discrete
variables were analyzed by chi-square with Yates’ correction.
Summary of Results: Examination of patient records identified 371 infants
with abnormal pH studies (positive group) & 394 infants with normal pH
studies (control group). 241 infants in positive group & 256 infants in negative group were excluded from further analysis due to lack of follow up
records. In addition, 9 infants in the positive group & 14 infants in the negative group were removed due to co-morbid conditions like genetic
syndromes or previous gastrointestinal surgery. Of the 206 patients in the final analysis, 38/105 subjects in the positive group & 20/101 subjects in the
control group were found to have GERD in childhood (36% vs. 20%,
p=0.0139). The 2 groups were similar in terms of age at follow up (9.2 ±
5.2 vs. 9.7 ± 5.34 yr., x ± SD, p=0.14), gender (55% vs. 46% male,
p=0.16) & race (60%/40% vs. 38%/60%/2%, Caucasian/African-American/
Asian).
Conclusions: We conclude that children who have significant GERD in infancy are more likely to experience GER symptoms during childhood. We
speculate that abnormal esophageal pH study results in infancy may be prognostic of an increased risk of GERD later in childhood.
* Patients may have had more than 1 diagnostic study.
399
A HEALTHIER BODY FAT DISTRIBUTION AFTER GASTRIC
BYPASS SURGERY IS ASSOCIATED WITH IMPROVEMENTS
IN INSULIN SENSITIVITY AND ADIPOKINE LEVELS
Mitchell PW1,2, Marks-Shulman P2, Shang M2, Tyree R2, Tamboli R2, Abumrad N2.
1
LSUHSC New Orleans School of Medicine, New Orleans, LA and 2Vanderbilt
University School of Medicine, Nashville, TN.
Purpose of Study: Body fat distribution is an important determinant of
health risks, and is dependent upon hereditary factors and hormones. Android obesity (central or “apple-shaped”), which includes visceral fat, is correlated with increased risk of cardiovascular disease and type 2 diabetes,
whereas gynoid obesity (lower body or “pear-shaped”), is associated with
lower disease risk and may even be protective. Roux-en-Y gastric bypass
(RYGB) is to most effective means to achieve massive weight loss and
improvements in metabolic diseases. We hypothesized that an improved
body fat distribution after RYGB would be associated with improvements
in insulin sensitivity and metabolic hormone profiles.
Methods Used: To test this hypothesis, we utilized data from our cohort of
42 RYGB subjects (all females) collected prospectively up to 2 years after
RYGB. Body fat distribution was assessed by waist and hip circumferences
and dual-energy x-ray absorptiometry (DXA), which measured percent fat
in the android and gynoid regions.
Summary of Results: Body fat distribution improved after RYGB to a more
gynoid fat distribution. A decrease in the ratio of android-to-gynoid percent
fat (AGR) after RYGB had a significant effect on improvements in hepatic
and peripheral insulin sensitivity and leptin levels after RYGB in addition
to weight loss. The increase in adiponectin after RYGB was associated with
a decrease in AGR but was not associated with weight loss. Body fat distribution before RYGB had no effect on changes in body weight, insulin
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
525
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
sensitivity, or metabolic hormones after RYGB. Additionally, our data indicate that waist-to-hip ratio is not a precise substitute for AGR in obese and
post-RYGB subjects.
Conclusions: This data suggests that individuals with android and gynoid
obesity experience the same weight loss and metabolic benefits after RYGB;
however, the location of fat mass loss is a determinant of the degree of metabolic improvements after RYGB.
400
CONSTIPATION DIAGNOSIS AND MANAGEMENT:
WHAT ARE OUR PRACTICES?
Lowry M, Lukens E, Monroe K, Hitch M, Morris J, Pruitt C, Nichols MH.
University of Alabama at Birmingham, Birmingham, AL.
Purpose of Study: The purpose of this study was to investigate the diagnostic techniques and management used to assess a pediatric patient with
suspected constipation by Pediatric Emergency Medicine (PEM) and Pediatric Gastroenterology (GI) Health Care Providers (HCP) and Pediatric
Residents. This study follows another study examining 621 ED patients with
discharge diagnosis of constipation where 72% of patients had x-rays, 35%
treated with enemas, 51% with ED intervention (19% no documented treatment results), and 36% with dietary recommendations.
Methods Used: A survey was emailed to 125 physicians and nurse
practitioners regarding their individual practice in the diagnosis and management of abdominal pain related to constipation. This survey questioned their
use of imaging, digital rectal exams, enemas, oral laxatives and patient
counseling. The study was approved by our Institutional Review Board.
Summary of Results: 95 HCP completed the survey: 17 PEM Attendings,
8 PEM Fellows, 2 PEM Nurse Practitioners, 6 GI Attendings, 3 GI Fellows,
59 Pediatric Residents. When HCP were asked if they obtain x-rays in
patients with abdominal pain, 16% HCP responded usually, 44% sometimes,
24% occasionally, 15% rarely. Main reasons for x-rays included suspicion of
obstruction (86%) or intussusception (57%), persistent vomiting (44%), or
generalized pain with negative workup (54%). Digital rectal exams were usually performed by 32% of HCP, 24% sometimes, 19% occasionally, 20%
rarely. Preferred treatment for constipation included enema 56%, Miralax
90%, dietary recommendations 49%. GI HCP were more likely than PEM
HCP to administer an enema and counsel on structured toileting. When
asked if results were documented if ED intervention, 31% responded always,
33% usually, 14% sometimes, 7% occasionally, 11% rarely. HCP believed an
ED discharge order set (95%) and prompts to document results if rectal or
treatment performed (77%) would be beneficial.
Conclusions: When comparing the practices of HCP with an ED retrospective study of patients with discharge diagnosis of constipation, x-rays were
obtained more frequently than reported practice by HCP. Enemas are a common treatment practice of HCPs. Documentation of treatment results and discharge recommendations could possibly be improved with HCP education
and ED chart prompts and discharge order sets.
401
frequently prescribed medicines were laxatives in 42% and gastric acid
suppressants in 27% of the sample. Other medicines included anticholinergics, antidepressants, prokinetics, appetite stimulants, peppermint oil, fiber,
and probiotics.
Follow-up visits varied in number and chronology, from one to 9 visits,
based on clinical status. Five subjects (3%) withdrew because their diagnosis
changed: 2 Crohn’s disease, 2 celiac disease. Forty-four subjects were lost to
follow -up. One year after the initial visit, we interviewed 95 subjects.
Twenty-one subjects (22%) were asymptomatic, 37 (39%) were improved,
26 (27%) were unchanged, and 6 (6%) were worse. (No response from 5).
Age, sex (regardless of the onset of puberty), IBS subtype, BMI, and co-morbid
functional disorders did not predict outcome.
Conclusions: We conclude that there was no consensus treatment for IBS.
The relatively common use of acid inhibitors may reflect attempts to treat
co-morbid dyspepsia, or treat IBS with a safe placebo. A majority of IBS
children attending GI clinic miss school because of symptoms. The natural
history of IBS is uncertain, and it is unclear whether treatment alters outcome. The absence of consensus about drug treatment casts doubt on drug
efficacy.
402
CHRONIC USE OF ADVIL CAUSING
COLLAGENOUS COLITIS
Moti D, Ahmed M. Texas Tech Univ Health Sciences Center, Amarillo, TX.
Case Report: Collagenous colitis is characterized by chronic watery diarrhea
which typically occurs in middle aged patients. Several etiologies have been
suggested, one of them to be NSAIDs. In this report, we present case of chronic
diarrhea due to collagenous colitis associated with a long term use of Advil.
An 83 year old white female presented with diarrhea of 2 months duration. The diarrhea was watery and non-bloody. The daily frequency was 9-12
motion per daily. The diarrhea was mostly post-prandial. There was no abdominal pain, fever or chills. Patient denied any nausea or vomiting and any significant weight loss. No recent history of travel. Patient had history of chronic
arthritis and was taking at least 800 mg of Advil daily for more than 5 years.
At presentation, patient showed signs of dehydration. The stool examination
was non-revealing. A colonoscopy with biopsy revealed chronic colitis consistent with Collagenous Colitis. Patient was rehydrated, and was advised to discontinue the Advil. She was started on budesonide. Within a short period of time, her
symptoms ceased, and she remained disease free on subsequent follow up.
Collagenous Colitis is one of the two different types of microscopic colitis which typically occurs with middle aged patients. It is characterized by
chronic, watery diarrhea associated with fecal urgency. Several theories on
the etiology of collagenous colitis have been suggested. It is possible that
the underlying causes may differ among patients. Collagenous colitis may
represent one clinical presentation of a group of collagenous mucosal inflammatory diseases of the gastrointestinal tract that affect not only the colon but
also the small intestine and stomach. The diagnosis of Collagenous Colitis is
established by biopsy of the colonic Mucosa. Therapy should consist of stopping NSAIDs and other drugs that have been associated with these disorders.
Steroid is the mainstay of treatment.
THERE’S NO PREDICTING! NATURAL HISTORY OF
IRRITABLE BOWEL SYNDROME IN CHILDREN
Lee ES2, Hyman P1,2,3. 1Children’s Hospital New Orleans, New Orleans,
LA; 2LSU Health Sciences Center - New Orleans, New Orleans, LA and 3Digestive Health Alliance Children’s GI Research Network, Milwaukee, WI.
Purpose of Study: The purpose of this study was to prospectively gather information concerning symptoms, treatment, and school absence in a cohort
of children with IBS.
Methods Used: We enrolled 144 subjects (97 female) aged 4-17 years. All
met symptom based Rome criteria for IBS on the QPGS-RIII questionnaire.
Summary of Results: We found subtypes: constipation predominant-IBS (cIBS) in 43, diarrhea-predominant IBS (d-IBS) in 60, alternating IBS (a-IBS)
in 31, and no subtype in 10. BMI was >85% in 36%. We found dyspepsia,
defined by frequent upper abdominal discomfort, in 71 IBS subjects. Pain
exacerbations lasting hours to days resulted in an abdominal migraine diagnosis in 39 subjects. At the initial visit 64% had missed school within the
past month because of abdominal pain. Physicians prescribed medicine in
90%, cognitive behavioral therapy in 18%, and no treatment in 9%. The most
526
403
IMPACT OF SOCIAL MEDIA ON GASTROENTEROLOGISTS
IN THE UNITED STATES
Davis ED1, Tang S1, Zebedee Z1, Glover P1, Wu R2, Wang A2. 1University of
Mississippi, Flowood, MS and 2UT Southwestern, Dallas, TX.
Case Report: Social media is pervasive in the mobile and connected world.
As of 2012, Facebook® has more than one billion users and Twitter® has
about 300 million users.
Major academic gastroenterological societies in the US; such as the
AGA, ASGE, ACG, and their affiliatedscientific publications all have active
pages among social media.
To assess the current impact of social media on healthcare and gastroenterology in the US and the attitude of gastroenterologists toward social media.
We sent web-based invitations to all 183 gastroenterology fellowship
program directors and 6,600 practicing gastroenterologists in the US.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
265 invitees completed the survey. We were interested in areas such as:
the amount of time physicians spent on social media, which medium,
and how much of their supplemental educational material was derived
from these sources.
The majority of study participants were not interested in social media as a
way to obtain continuing medical education. 82.1% of respondents did not
access AGA, ASGE, ACG social media pages for educational materials.
47.7% of gastroenterologists never utilized any form of social media. In addition, most respondents thought that social media will not positively impact
medicine, with respect to the accurate dissemination of medical information.
Smart phones are the most popular device used for e-mails and web browsing. In 2013, the majority of American gastroenterologists were not interested in social-media-based medical education and did not believe social
media would positively impact medicine.
Responses to the question: “How do you feel social media will improve healthcare”
Southern Regional Meeting Abstracts
Methods Used: Retrospective chart review.
Summary of Results: Results: All patients presented with refractory constipation between 1-3 years. Group 1 included 65 patients, 52% male, diagnosed via endoscopy in infancy with MPI (>10 eos/hpf in duodenum (ED)
&/or >5 eos/hpf in rectosigmoid colon (ERS)) with lymphonodular hyperplasia (LNH), a frequent endoscopic finding in MPI, present in 65%, ED
in 64%, and ERS in 97%. There was no association between the presence
of ED or ERS and symptoms of hard stools, fecal occult blood (FOB), visible
blood, visible mucus, abdominal pain, abdominal distention, bloating,
vomiting, straining with defecation or the presence of more than two
symptoms (>2SS). There was a significant association between LNH and
hard stools (p<0.046). Of note, 22% were already on, or asked to be on a
cow’s milk-free diet (CMFD) due to the severity of the constipation and
17% were asked to be on a soy protein-free diet (SPFD). Our second group
included 27 patients, 55% male, who were scoped due to refractory constipation and were diagnosed with MPI between 1 & 3 years of age with LNH in
85%, ED in 68%, and ERS in 100%. There were significant correlations between ED and FOB (p< 0.037) and ED and the presence of >2SS (p<0.004).
In this group, all patients were asked to be on a CMFD and in 26% the constipation resolved, 48% didn’t improve, and 26% had no follow-up or compliance with the CMFD. Lastly, 44% of patients were asked to be on a
SPFD and in 50% of them the constipation resolved, 33% didn’t improve,
and 16% had no follow-up or non-compliance with the SPFD.
Conclusions: Although intestinal eosinophilia is used for the diagnosis of
MPI, few correlations with symptomatology exist in patients with refractory
constipation and MPI in the toddler age group whether diagnosed as infants
or toddlers, but at least 50% improved on a CMFD or CMFD plus SPFD.
The primary limitations were the small numbers and the variability in dietary
compliance.
Hematology and Oncology I
Concurrent Session
2:00 PM
Friday, February 21, 2014
405
T(8;9)(P22;P24): A SIMPLE TRANSLOCATION OR A
NEW DISEASE?
404
CORRELATION BETWEEN ENDOSCOPIC FINDINGS
AND CLINICAL SYMPTOMATOLOGY IN TODDLERS
WITH REFRACTORY CONSTIPATION AND MILK
PROTEIN INTOLERANCE
Alkilani A, Connelly R, Crissinger K. University of South Alabama
Children’s and Women's Hospital, Mobile, AL.
Purpose of Study: Refractory constipation in toddlers may be due to milk
protein intolerance (MPI) that is diagnosed by increased eosinophils in the
duodenum &/or colon. The purpose of the study was to determine wherher
intestinal eosinophilia correlates with the severity of constipation and associated clinical symptoms.
Bitar C1, Saba J2, Safah H3. 1University of Balamand, Beirut, Lebanon;
2
Lebanese University, Beirut, Lebanon and 3Tulane University, New
Orleans, LA.
Purpose of Study: Janus kinase 2 (JAK2) gain of function mutation is a
well-known genetic anomaly in myeloproliferative neoplasms (MPN). Recently, t(8;9)(p22;p24) has been described to activate JAK2 resulting in a variety of diseases that surpasses MPN to include cases of acute leukemia,
lymphoma, and myelofibrosis. At the molecular level, t(8;9)(p22;p24) results
in new fusion gene product, putting pericentriolar material 1 (PCM1) gene
located on 8p22 and JAK-2 located on 9p24 together. This leads to continuous activation of JAK2 tyrosine kinase, with subsequent hematological malignant effects. PCM1-JAK2 positive diseases are characterized by an
aggressive disease course and resistance to most available therapies. In this
study, we review all reported cases of t(8;9)(p22;p24) and discuss issues related to diagnosis and therapy.
Methods Used: An extensive literature search on PubMed/MEDLINE was
conducted using the keywords “PCM1” and “JAK2”. 27 published articles
with a confirmed t(8;9)(p22;p24) were identified. Data were gathered and
tabulated regarding the following 10 variables: age, gender, presentation, diagnosis, peripheral blood, bone marrow, cytogenetics, FISH, therapy, and
outcomes.
Summary of Results: 27 cases were identified. Median age was 47 years
(range, 12-75 years), with a striking male predominance (M:F =4.4; M=22,
F=5). Myeloid neoplasm was by far the most common diagnostic entity
(24/27), this includes atypical CML n=6; AML n=5; CEL n=5; MDS n=4;
MPN- unclassified n=3; acute erythroid leukemia n=1. The rest was lymphoid malignancies (3/27): one pre-B-ALL, one Burkitt’s leukemia, and
one T-cell lymphoma. The choice of therapy was based on disease entity,
and can be divided into 3 categories: cytotoxic chemotherapy, allogeneic stem
cell transplant, and tyrosine kinase inhibitors. The latter was a well-tolerated
therapy and induced favorable responses. Long follow-ups were unavailable
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
527
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
in most cases, and death was reported in12 cases secondary to disease progression or complications of therapy.
Conclusions: t(8;9)(p22;p24) remains a rare translocation resulting in an aggressive disease. Emerging tyrosine kinase inhibitors have promising roles,
but need to be validated in the context clinical trials.
406
PRIMARY TREATMENT FOR CLINICALLY LOCALIZED
PROSTATE CANCER: DOES AGE OR RACE MATTER?
Saltzman A1,2, Luo S3,4, Hudson M1. 1Ochsner Clinic Foundation, New
Orleans, LA; 2Louisiana State University, New Orleans, LA; 3Washington
University School of Medicine, St. Louis, MO and 4John Cochran Veterans
Affairs Medical Center, St. Louis, MO.
Purpose of Study: To determine whether racial and/or age differences exist
for the primary treatment type received by veterans with equal insurance access for prostate cancer detected by PSA > 4 mg/mL.
Methods Used: We identified a cohort of 20,694 African American (AA)
and white veterans aged 40-70 years with PSA >4 ng/mL between 10/1/2000
and 9/30/2007 within VHA. 6,223 underwent prostate biopsy and 3,030
veterans were diagnosed with prostate cancer. We performed a retrospective
chart review and/or obtained data from the Veterans Administration Central
Cancer Registry on patients with clinically localized disease. We report the primary treatment received, stratifying by age and race. Treatment categories included active surveillance, surgery, radiation, androgen-deprivation therapy
alone, other (photon beam, cryotherapy), and refused/no treatment.
Summary of Results: 2479 veterans with clinically localized prostate cancer
were analyzed. Overall, AA veterans were most likely to undergo radiation
therapy (p<0.0001) while whites were most likely to undergo surgery
(p<0.0001). When stratifying by age alone, veterans aged 40-49 and 50-59
were most likely to undergo surgery (p<0.0001) while veterans ages 60-69
were most likely to undergo radiation (p<0.0001). When stratifying by age
and race, only the 50-59 age group showed racial differences, with AA
veterans most commonly treated with radiation and white veterans most
commonly treated with surgery (p<0.001). Comorbidity indices, using the
Romano adaptation of the Charlson comorbidity index, were significantly
higher in the 50-59 year old AA veterans than their white counterparts
(p=0.0017), but D’Amico risk status and Gleason score were not significantly different (p=0.1245 and p=0.0877 respectively).
Conclusions: Age and comorbidities are associated with primary treatment
type for localized prostate cancer, and may in part explain observed racial
differences in primary treatment type received for clinically localized prostate in veterans.
407
RED BLOOD CELL TRANSFUSION AND RED BLOOD CELL
EXCHANGE IN SICKLE CELL DISEASE
McNaughton J, Mayhall G, Schmid J. Tulane University School of Medicine,
New Orleans, LA.
Purpose of Study: Red blood cell (RBC) transfusion and RBC exchange are
procedures used to treat sickle cell disease (SCD). Information was gathered
on each to educate sickle cell patients and families.
Methods Used: A literature review was performed to examine aspects of
sickle cell disease and transfusion therapy, including the history of SCD
and therapeutic apheresis, the effects of transfusion on hemoglobin S, the
risks and benefits of RBC transfusion and RBC exchange, and transfusion
reactions, including alloantibody formation.
Summary of Results: Sickle cell crises often occur due to occlusion of
blood vessels by high levels of Hemoglobin (Hb) S cells. RBC transfusion
helps improve blood flow and preserve oxygen delivery to tissues by increasing the amount of normal HbA, which decreases the proportion of HbS.
Transfusion and exchange are indicated to treat and prevent many of the serious events that can occur in SCD, including stroke and acute chest syndrome. Simple transfusion is the receipt of a unit of healthy donor RBCs.
Exchange is the separation of a patient’s whole blood by an apheresis machine. The RBCs are removed with subsequent return of the remaining blood
and donor RBCs. There are benefits and risks to both. Transfusion and exchange transfusion decrease the levels of HbS and provide HbA, although
528
transfusion can lead to iron overload. Exchange prevents iron overload, but
exposes the patient to more donor RBC units. When a marked reduction of
HbS is needed, exchange will be more effective than transfusion. Transfusion
reactions rarely occur and are most commonly hives or fever. More serious
reactions can happen, such as hemolytic transfusion reactions, although
transfusion services have many steps in place to prevent these. Alloantibody
formation is common in sickle cell patients. Many transfusion services provide extended phenotype matching for RBC antigens to prevent antibody
formation and all provide antigen negative blood in cases where an antibody
has been made.
Conclusions: Transfusion and exchange provide excellent results in the
treatment and prevention of vaso-occlusive crises in SCD. There are risks
to each, although transfusion services work to prevent serious events, so that
the benefits of transfusion and exchange in SCD outweigh the risks.
408
STROMAL CELLS PROVIDE SUPPORTVE NICHE FOR
TUMORIGENESIS AND METASTASIS IN ESOPHAGEAL
ADENOCARCINOMA
Zhang X1, Myers T2, Cordova J2, Green H2, Davis N3, Bolton J2,
Margolin D2, Li L1. 1Ochsner Clinic Foundation, New Orleans, LA;
2
Ochsner Clinic Foundation, New Orleans, LA and 3Ochsner Clinic Foundation, New Orleans, LA.
Purpose of Study: The incident of esophageal adenocarcinoma (EAC) has
risen 600% over the last 30 years. The 5-year survival rate is about 15%.
Lymph node (LN) is the first metastatic site for EAC. Positive LN involvement in EAC is one of the strongest negative indicators for patient outcome.
It is known that LN stromal cells not only physically construct the LN architecture but also are essential for regulating cell migration and recruiting. To
elucidate the molecular mechanism underlying EAC metastasis, we investigated the role of stromal cell in EAC growth and metastasis using noninvasive imaging approaches in a human-in-mouse xenograft model.
Methods Used: EAC tissue samples were obtained from 8 consented patients
undergoing esophageal resection and digested using collagenase for single cell
suspension. The characteristics of EAC patient cancer cells and human EAC
cell line OE33 were compared by flow cytometry and immunohistochemistry.
Luciferase-tagged OE33 or patient cancer cells were subcutaneously implanted
in the flanks of NOD/SCID mice. EAC tumorigenesis and/or metastasis in the
absence or presence of stromal cell HK or HK cell conditioned-media were
monitored by bioluminescence imaging system.
Summary of Results: We established a reproducible human-in-mouse xenograft models that developed spontaneous metastasis. The xenografts from
OE33 cells and EAC patients’ cancer cells share similar architecture,
biomarkers, and histopathologic morphology with primary tumor in EAC
patients. Addition of HK cells or HK cell conditioned-media not only significantly facilitates the EAC cell growth in vitro, but also supports primary tumor growth and mouse liver and lung metastasis.
Conclusions: We have developed a robust approach for imaging of EAC
growth and dissemination, permitting both macroscopic and microscopic
analysis of EAC progress. Our data suggested that LN stromal cells provide
a direct support to EAC development and metastasis. Targeting signals from
stromal cells in combination with chemotherapy may provide a promising
therapeutic strategy for EAC.
409
PULMONARY EMBOLISM IN A PREVIOUSLY HEALTHY
YOUNG WOMAN
Wallis NK1,2, Northrip K1, Radulescu VC1. 1University of Kentucky,
Lexington, KY and 2University of Kentucky, Lexington, KY.
Case Report: An obese 16 year-old female presented in severe respiratory
distress that developed over the course of 5 days. She complained that her
heart was “racing” and her throat “throbbed” and was unable to speak full
sentences or walk even a few steps. A CT scan of the chest revealed large bilateral pulmonary emboli and significant right heart strain. ECHO verified
RV dilation with systolic dysfunction and tricuspid regurgitation. Right heart
catheterization confirmed elevated right heart filling pressures and she was
successfully treated with ultrasound-enhanced thrombolysis. Coagulopathy
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
work-up included Factor V Leiden, Factor VIII, protein C and S, prothrombin,
homocystine, Beta-2 glycoprotein and anticardiolipin. All were within normal
limits except the lupus anticoagulant (LA), which was strongly positive. Upper
and lower extremity ultrasound did not show evidence of DVT. The patient was
treated with Lovenox as a bridge to Coumadin and was discharged home after
9 days. Her only medical problem prior was menorrhagia for which she was
started on the OCP, Tri-Previfem, 3 months prior. Her family history was negative for significant blood clots or pregnancy losses.
Pulmonary embolization has become more common in the adolescent
population over the past couple of years. Possible causes include the increased use of oral contraceptives, the obesity epidemic, and improved
diagnostics. Obesity and OCPs independently increase the risk of thrombosis
and the combination of both lead to a 24-fold higher risk. Our patient had
these risk factors plus a positive test for LA, an antiphospholipid antibody.
Persistent antiphospholipid antibodies further increase the risk of both arterial and venous thrombosis. After diagnosis, the patient was counseled to
avoid all estrogen-containing contraceptives. She will remain on life-long
anticoagulation if repeat testing reveals persistent LA as she would then meet
the diagnostic criteria for antiphospholipid antibody syndrome. This case
highlights the need to carefully consider the risks and benefits prior to
starting a patient on OCPs and reminds clinicians of the complex set of
factors in determining a patient’s risk for thrombosis.
410
ACUTE PROMYELOCYTIC LEUKEMIA PRESENTING
WITH GINGIVAL HYPERPLASIA
Eubanks J, Herrin V. University of Mississippi Medical Center, Jackson, MS.
Case Report: Acute promyelocytic leukemia (APL) is a distinct subtype of
acute myeloid leukemia resulting from the promyelocytic leukemia-retinoic
acid receptor α (PML-RARα) fusion gene that typically presents with pancytopenia, bleeding, and coagulopathy. We report a case of an unusual presentation of APL involving gingival infiltration.
A 43 year old man with a history of pulmonary embolism, non-compliant
with warfarin, presented with a four day history of progressive gum swelling,
bleeding and tenderness with associated difficulty speaking. Examination
showed pronounced gingival hyperplasia and bleeding. A complete blood
count showed a white blood count of 57 × 109/L, anemia, and thrombocytopenia with a peripheral blood smear showing promyelocytes and blasts. No
coagulopathy was present. Bone marrow biopsy showed hypercellularity
with promyelocytes comprising >80% of the marrow cells. Cytogenetics
revealed t(15;17)(q24;q21) and fluorescence in situ hybridization showed
the PML-RARα gene fusion, establishing the diagnosis of APL. He was
treated with all-trans-retinoic acid (ATRA) plus cytarabine and idarubicin.
Aminocaproic acid solution improved the gingival bleeding. His course
was complicated by differentiation syndrome treated with dexamethasone
and a brief interruption in ATRA therapy. At completion of induction, there
was vast improvement in gingival hyperplasia.
Southern Regional Meeting Abstracts
While oral bleeding is often present at diagnosis of APL, leukemic infiltration of the gingival tissue is rare. This is more commonly seen with monocytic variants of acute myeloid leukemia and cases reported of gingival
involvement in APL have been associated with relapse rather than initial diagnosis. This case underscores the potential for a gingival manifestation of
APL at presentation.
411
ANALYSIS OF EPIDERMAL GROWTH FACTOR
RECEPTOR (EGFR) MUTATIONS AND ECHINODERM
MICROTUBULE-ASSOCIATED PROTEIN-LIKE
4-ANAPLASTIC LYMPHOMA KINASE (EML4-ALK)
GENE REARRANGEMENT IN LUNG ADENOCARCINOMA
PATIENTS: A 3-YEAR EXPERIENCE OF THE
LOUISIANA STATE UNIVERSITY (LSU) PUBLIC
HOSPITAL IN NEW ORLEANS
Narmala SK, Puligothram S, Jahangir SZ, Boulmay B. LSU - New Orleans,
New Orleans, LA.
Purpose of Study: The National Comprehensive Cancer Network recommends that all patients with lung adenocarcinoma (LA) be tested for EGFR
mutation and EML4-ALK gene rearrangement (EGFR/ALK). EGFR mutations are found in approximately 10% of white patients and up to 50% of
Asian patients with LA. The prevalence of EML4-ALK gene rearrangement
is between 2-7% in LA patients. Both are more common in non-smokers. We
analyzed the frequency of EGFR mutations and EML4-ALK gene rearrangements from the biopsy samples submitted for these analyses.
Methods Used: We identified patients from LSU diagnosed with metastatic
LA whose specimens were sent for EGFR/ALK from January 1, 2009 to
June 30, 2013. Data collected included number of specimens sent for EGFR/
ALK, number of samples with inadequate tumor, biopsy technique utilized
and number of re-biopsy attempts for EGFR/ALK.
Summary of Results: 54 patients were evaluated in the study time period:
58 individual biopsy specimens were sent for EGFR/ALK. 34/58 (58%) of
specimens were found to be adequate for EGFR and/or ALK. 6/34 samples
were inadequate for EGFR, but adequate for ALK, 2/34 were inadequate
for ALK, but adequate for EGFR. In 12/34 samples only EFGR was tested,
ALK was not tested. Only 1 of the 28 samples tested for EGFR had the mutation and none of the 22 samples tested for EML4-ALK had the gene
rearrangement.
Conclusions: A substantial proportion of initial LA diagnostic biopsies were
inadequate for EGFR/ALK analysis. EGFR mutations and EML4-ALK rearrangement are rare in LA patients seen at LSU public hospital. Most of
patients in the analyzed tend to be black and almost all had an extensive
smoking history which could explain the low frequency of EGFR mutations
and EML4-ALK rearrangement.
412
INTERIM LSU HOSPITAL (ILH) STUDY 2012: EVALUATING
ADHERENCE TO SUPPORTIVE CARE GUIDELINES FOR
PATIENTS ADMITTED TO ILH FOR NEUTROPENIC FEVER
Castillo E, Dhillon N, Puligothram S, Boulmay B. LSUHSC, New Orleans, LA.
Purpose of Study: The purpose of this quality improvement project was to
identify if growth factors and prophylactic antibiotics had been included in
the treatment plan of the patients admitted with neutropenic fever in 2012.
Methods Used: At LSU Interim Hospital in New Orleans, the Hematology/
Oncology team evaluated the number of neutropenic fever admissions and
whether growth factors and antibiotics had been administered per guidelines.
A chart review of these admissions was performed in order to give recommendations to the cancer committee as applicable.
Summary of Results: A total of 21 charts of patients admitted for neutropenic
fever as a primary diagnosis were reviewed.
- Eleven patients (six on hyper-CVAD and five on 7+3) received prophylactic antibiotics; they all had an indication for antibiotic prophylaxis
according to commonly accepted guidelines.
- Fourteen patients (6 on hyper-CVAD, 3 on R-CHOP, 3 on 7+3 and 2 of
DCF) received growth factor as part of their treatment. Per guidelines, it was
only indicated in 11 of them.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
529
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
- Seven patients (1 on FOLFOX, 1 on weekly Cisplatin + radiotherapy, 2
on DCF, and 2 on Cisplatin + Etoposide) did not receive prophylactic
antibiotics or growth factors, also consistent with commonly accepted treatment guidelines.
Conclusions: After evaluating the characteristics of chemotherapy related
neutropenic fever admissions to the LSU Interim Hospital, we have come
to the conclusion that guidelines for prophylactic antibiotics and growth
factors use were followed. Based on this data, we have no recommendations
to the cancer committee, other than to encourage clinicians to use the treatment templates provided in EPIC, which are built with commonly accepted
supportive care agents.
413
HODGKIN’S LYMPHOMA PRESENTING WITH EPIDURAL
SPINAL INVOLVEMENT
Evans J, Herrin V. University of Mississippi Medical Center, Jackson, MS.
Case Report: Hodgkin’s lymphoma typically presents with painless lymphadenopathy, however in minority of cases can have extranodal spread of disease.
We report a case of newly diagnosed classical Hodgkin’s lymphoma with epidural spinal involvement resulting in severe lumbar spinal canal stenosis.
A 32yo female presented with complaints of shortness of breath,
palpitations, subjective fevers, night sweats, and unintentional weight loss
over the past several weeks. Initial CT scan of chest showed extensive lymphadenopathy in supraclavicular, hilar, and mediastinal lymph nodes, therefore
patient was transferred to our institution. On physical exam patient was
found to have large palpable soft tissue mass at base of left neck with normal
neurological exam. Initial labs were significant for leukocytosis of 12,000,
hemoglobin of 6.5 g/dL, and platelet count of 207,000. Patient was admitted
to the hospital and underwent subsequent CT imaging of her neck and abdomen that revealed wide spread lymphadenopathy, as well as L5 vertebral lesion. She then underwent a MRI of her lumbar spine confirming involvement
of L5 vertebra as well as an epidural mass extending from L3-4 to S2-3 that
resulted in severe lumbar spinal canal stenosis. Surgery was consulted and
performed excisional biopsy of cervical lymph node that was consistent with
classical Hodgkin’s lymphoma, nodular sclerosis subtype. While in the hospital patient had recurrent fevers up to 103 degrees Fahrenheit with complaints of pain in lower back requiring intravenous narcotics, but did not
express signs of spinal cord compression. She also received 2 units of packed
red blood cells for treatment on anemia with improvement in shortness of
breath and palpitations. After results of biopsy patient was discharged from
the hospital with plans to undergo outpatient bone marrow biopsy for staging
purposes prior to initiation of chemotherapy.
Epidural spinal involvement of Hodgkin’s lymphoma is a relatively rare
phenomenon found most often in disseminated advanced stage disease. Its
occurrence has been described between 0.5 and 3%. Unless it causes signs
of cord compression, treatment typically involves systemic chemotherapy
without surgical intervention.
Infectious Diseases I
Concurrent Session
2:00 PM
Friday, February 21, 2014
414
GROUP B STREPTOCOCCI INDUCE
PROINFLAMMATORY RESPONSES VIA A
PROTEIN KINASE D1 DEPENDENT PATHWAY
Upadhyay K1, Park J1, Kim Y1, Meals E1, Talati AJ1, English B2, Yi A1.
1
CFRI, UTHSC, Memphis, TN and 2Michigan State University, Grand
Rapids, TN.
Purpose of Study: Protein kinase D1(PKD1) plays a significant role in inflammatory process mediated by MyD88 dependent toll like receptors
(TLR). We proposed to study the role of PKD1 in Group B Streptococci
(GBS) mediated proinflammatory responses and to determine a molecular
mechanism by which GBS activates PKD1 in macrophages.
530
Methods Used: RAW 264.7 cells were stimulated with live GBS or penicillin G-killed GBS and activation of PKD1 was analyzed by in vitro kinase assay
and phospho-specific Western blot assay. Subsequently, MyD88-/- macrophages,
IRAK1-/-, or TRAF6 -/- macrophages were stimulated with GBS and activation
of PKD1 was analyzed. Next, to investigate whether PKD1 plays indispensable
role in GBS mediated proinflammatory responses, RAW264.7 cells and
C57BL/6 mice were stimulated with GBS in the presence of PKD inhibitor
Gö6976 and expression and production of the selected cytokines/chemokines
were analyzed by RT-PCR and ELISA. Finally, to determine contribution of
PKD1 in GBS-mediated septic shock-like death, D-galactosamine (D-GalN)
sensitized mice were challenged with antibiotic killed GBS in the presence of
PKD inhibitor and then viability of mice was observed.
Summary of Results: Both live and antibiotic killed GBS induced kinase
activity and phosphorylation of PKD1. PKD1 activation by GBS was completely abolished in MyD88-/- macrophages and IRAK1-/- macrophages
but not in TRAF6 -/-macrophages. GBS induced activation of signaling
modulators (i.e., JNK, ERK, p38, or NF-κB) and expression and production
of proinflammatory cytokines and chemokines (TNFα, IL-6, IL-10, IL12p40, IP-10, MCP-1, CCL5, MIP-1α, MIP-2, LIX, and KC) were either ablated or significantly inhibited in the presence of PKD inhibitor in vitro and
in vivo. Furthermore, systemic administration of PKD inhibitor protected DGalN-sensitized mice from shock mediated death caused by GBS.
Conclusions: GBS induced activation of PKD1 via a MyD88 and IRAK1
dependent manner. PKD1 played an indispensable role in GBS-mediated activation of MAPKs and NF-κB and subsequent expression of proinflammatory
cytokines and chemokines in vitro and in vivo. Furthermore, inhibition of
PKD1 activation protected mice from death due to exaggerated proinflammatory response induced by antibiotic-killed GBS.
415
PREVALENCE AND AGE-RELATED ACQUISITION OF
ANTIBODIES AGAINST GROUP A STREPTOCOCCAL
M-RELATED PROTEINS
Agbaosi T1,2, Niedermeyer SE1,2, Penfound TA1,2, Dale JB1,2. 1University of
Tennessee Health Science Center, Memphis, TN and 2Veterans Affairs Medical Center, Memphis, TN.
Purpose of Study: Group A streptococcal (GAS) infections are common in
children but less common in adults. Immunity against type-specific M
proteins of GAS protects against subsequent infection with the same M type.
The relative resistance of adults to infection has been attributed to immunity
to multiple M types, among other factors. An additional explanation is immunity to shared, cross-protective GAS antigens that provides broader protection later in life. M-related proteins (Mrp) of GAS are expressed by
83% of GAS isolates and contain opsonic epitopes,. Unlike M proteins,
Mrp’s are highly conserved and are grouped into three structurally related
families represented by Mrp2, Mrp4, and Mrp49. In the current study we
evaluated the prevalence and age-related acquisition of Mrp antibodies in
children.
Methods Used: Serum samples from 88 subjects (ages 1-19) were obtained
from the clinical laboratories of a pediatric hospital. Purified, recombinant Nterminal peptides of Mrp2, 4, and 49 were used to assess Mrp antibody levels
by ELISA.
Summary of Results: Significant levels of antibodies against any of the
three Mrp’s were observed in 49/88 serum samples (56%). Mrp4 antibodies were the most prevalent (37/88), followed by Mrp 2 (29/88) and
Mrp49 (12/88). 25% of serum samples contained antibodies against one
Mrp, 17% against two and 14% against all three. There was a clear association between the subjects’ age and prevalence of antibodies against
Mrp’s (r=0.32, p<0.05). Significant age-related trends were also observed
with Mrp4 and Mrp2. Additional studies are underway to examine the
type-specific M antibody levels in the same cohort to assess the spectrum
of GAS infections in relation to age.
Conclusions: Mrp antibodies are prevalent and are acquired in an age-related
manner. Taken together with our recent studies showing that N-terminal
peptides of Mrp’s contain opsonic epitopes, these results suggest that Mrp
antibodies in combination with M antibodies may contribute to the relative resistance to GAS infections later in life. These findings also have implications for
the development of broadly protective GAS vaccines.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
416
M-RELATED PROTEINS AND M PROTEINS OF GROUP A
STREPTOCOCCI EVOKE BACTERICIDAL ANTIBODIES
AGAINST GROUP C AND G STREPTOCOCCI
Niedermeyer SE1,2, Penfound TA1,2, Hysmith ND1,2,3, Dale JB1,2. 1University of Tennessee Health Science Center, Memphis, TN; 2Veterans Affairs
Medical Center, Memphis, TN and 3St. Jude Children’s Research Hospital,
Memphis, TN.
Purpose of Study: Non-group A β-hemolytic streptococcal infections have
emerged as prominent causes of invasive disease. In recent studies, the incidence of serious infections involving group C and G streptococci belonging
to the species Streptococcus dysgalactiae subspecies equisimilus (SDSE)
exceeds that of group A streptococci (GAS). Efforts to develop safe and effective streptococcal vaccines have virtually ignored the significant global
burden of disease caused by SDSE. In the current study, we evaluated the
bactericidal activity of antisera raised against protective antigens of GAS,
M-related proteins (Mrp) and M proteins, against various SDSE emm types.
Methods Used: BLAST analysis was used to identify sequence homologies
among GAS and SDSE surface proteins. Rabbit antisera were raised against
Mrp and the 30-valent M protein-based vaccine and used in whole-cell
ELISA and bactericidal tests with various SDSE representing multiple
emm types.
Summary of Results: Significant sequence homologies were identified between some M proteins of SDSE and GAS Mrp’s and M peptides contained
in the 30-valent vaccine. Mrp and 30-valent vaccine antisera reacted with
multiple emm types of SDSE in whole-cell ELISA. In bactericidal tests, 5/5
SDSE tested to date were opsonized and killed in human blood containing either Mrp or 30-valent vaccine antisera. Studies are in progress to assess the extent of potential cross-protection between GAS vaccine antigens and clinical
isolates of SDSE.
Conclusions: The observation that antisera raised against protective antigens
of GAS promote bactericidal killing of SDSE suggests that vaccines originally designed to prevent GAS infections could potentially elicit broader immunity against serious invasive streptococcal diseases. These results are
consistent with previous observations of lateral exchange of genetic material
between GAS and SDSE. Future studies will be designed to test combination
vaccines containing GAS antigens to determine the breadth of potential coverage against SDSE.
417
THE INCIDENCE OF HERPES ZOSTER AMONG VETERANS
Moanna A1,2, Rentsch C1, Rimland D1,2. 1Veterans Affairs Medical Center,
Decatur, GA and2Emory University School of Medicine, Atlanta, GA.
Purpose of Study: The incidence of herpes zoster (HZ) in the U.S. has been
estimated to be 1 million cases or more annually with a higher rate in adults
older than 60 years of age. The morbidity of the disease including postherpetic neuralgia imposes significant impacts on quality of life. Previously,
we found that the incidence of HZ had increased from 3.10 episodes per
1000 veterans in 2000 to 5.22 in 2007. Here, we present a follow-up study
to evaluate the incidence of HZ in veterans after the introduction of the zoster
vaccine in late 2007.
Methods Used: To evaluate the continuing trend in the annual incidence of
herpes zoster since 2007, we derived incidence rates using the Veterans
Health System (VHA) Decision Support System (DSS) reports of HZ by
ICD-9 codes and the corresponding denominator data for all veterans in care.
The annual rates of HZ were calculated by dividing unique patients with HZ
by the total number of veterans seen that year. The same method was used to
calculate the rate by age groups. Chi square for trend was calculated for the
total and age specific rates over the entire time period. We used data from
DSS to present the use of zoster vaccine nationally.
Summary of Results: The total number of veterans seen increased from
5,292,175 in 2007 to 5,919,203 in 2012. The number of zoster episodes increased from 28,529 episodes in 2007 to 39,503 in 2012. Since 2007, the annual incidence of HZ increased from 5.39 per 1000 veterans to 6.67 in 2012
(R2 =0.6510, p=0.0524) .This increasing rate was seen in both men and
women for all age groups. There were 114,126 doses of the zoster vaccine
administered at the VA between 2007 and 2012. The estimated coverage
Southern Regional Meeting Abstracts
for veterans 50 years or older was 2.49% and that for veterans 60 or older
was 3.14%.
Conclusions: The incidence of HZ continues to increase in our veteran population despite the introduction of the zoster vaccine in 2007. The uptake of
this vaccine remains low in the population age 50 and older.
418
ANTIBIOTICS FOR EARLY ONSET SEPSIS
PROPHYLAXIS- TIME FOR STEWARDSHIP?
Rampon K1, Gaston K2, Talati AJ1,2. 1UTHSC, Memphis, TN and 2The Regional Medical Center, Memphis, TN.
Purpose of Study: Recent advances in prenatal care and maternal prophylaxis for GBS colonization have decreased the incidence of group B streptococcal (GBS) infection and early-onset sepsis (EOS), yet it remains a feared
complication of birth. Prophylactic antibiotics (abx) are justified in some
situations. Neonates with clinical symptoms of sepsis and/or abnormal laboratory values may be continued on abx beyond the empirical 48-hour mark of
sterile cultures. However, antimicrobial therapy in infants without other
indications should be promptly discontinued. Various maternal indications
have been used in the past to predict risk for the infant of developing EOS.
Likewise, clinical symptoms and lab tests of the infant appropriately increase
the suspicion of infection. We proposed to evaluate the reasons for starting
abx in a neonate immediately after birth and duration/reasons of prolonging
abx in event of a sterile blood culture in an asymptomatic infant.
Methods Used: Retrospective chart review of all neonates born during the
4 mths prior to June 2013 was done in a level 3 NICU. Infants with positive
blood culture were excluded. Medical records were reviewed after IRB approval and data collected from neonates who were started on abx included
demographics, maternal history, duration and type of abx, laboratory values
and clinical symptoms.
Summary of Results: 218 infants received abx soon after birth. Mean birth
weight was 2666 gm with gest. age 35 wk. Mean duration of abx therapy
was 88:52 ±19:57 h. For infants ≤ 1500gm the mean duration was longer
95±20 h compared to infants >1500 gm 87±20h. Maternal risks included
chorioamnionitis (27%), positive GBS status (16.5%), UTI (10%) and
PROM (15%). 184/218 (85%) received abx for >48h. Of these 184 infants
110 (59%) had normal lab values. A reason was not documented for continuing abx for 64 (30%) patients. 84(45%) had reason documented as elevated
CRP and 24(13%) as maternal chorioamnionitis.
Conclusions: Several asymptomatic infants continue to receive abx beyond
48 h in spite of no obvious reason. A “hardstop” by pharmacy at 48h may
prevent unnecessary additional doses of abx. This could save exposure to
infants of hundreds of doses of abx/year with significant cost savings. Continuation of abx in face of abnormal lab values in asymptomatic infants
should also be re-evaluated.
419
A POPULATION-BASED INVESTIGATION OF
OUTCOMES OF CANDIDEMIA
Kabbani S1,2, Stein B1,2, Hollick R4, Harrison LH4, Farley M1,2,3. 1Emory
University School of Medicine, Atlanta, GA; 2GA Emerging Infections Program (EIP), Atlanta, GA; 3VA Medical Center, Atlanta, GA and 4Maryland
Active Bacterial Core surveillance (EIP), Baltimore, MD.
Purpose of Study: Describe risk factors associated with mortality from
candidemia.
Methods Used: Demographic and clinical data were collected prospectively
between 3/1/08 and 2/28/13 for candidemia cases identified through active,
population-based, laboratory surveillance in Georgia and Maryland EIP. Univariate analysis of risk factors associated with mortality at 30 days in children and adults was performed; p<0.05 was significant.
Summary of Results: Overall, 3,782 candidemia cases were identified with
a mean annual incidence of 14.5/100,000 population. While only 64.9% of
cases were hospital-onset (HO), 93.1% had at least one of the following risk
factors: use of central venous catheter (CVC) (84.6%), total parenteral nutrition (TPN) (34%), or antibiotics within 14 day (78.8%). Death occurred in
917 (26.4%) adults and 32 (13.8%) children. Fluconazole resistance was
found in 7.2% of cases and was not associated with mortality. Among adults,
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
mortality was significantly higher with age ≥45 (OR 2.28 CI 1.82-2.84), HO
(OR 1.86, CI 1.57-2.21), antibiotics(OR 1.6, CI 1.31-1.97), CVC (OR 1.46,
CI 1.17-1.83), ICU stay (OR 3.85, CI 3.18-4.65), renal disease (OR 1.72, CI
1.43-2.06), liver disease (OR 1.74, CI 1.42-2.13), malignancy (OR 1.43, CI
1.18-1.72), prior antifungal use (OR 1.37, CI 1.11-1.69), and infection with
Candida krusei (OR 2.21, CI 1.3-3.76) or C. tropicalis (OR 1.38, CI 1.081.75). Black race (OR 0.8, CI 0.69-0.94), TPN administration (OR 0.84,
CI 0.72-0.99), CVC removal within 7days (OR 0.25, CI 0.21-0.3), antifungal
treatment (OR 0.2 CI 0.16-0.25) and C. parapsilosis (OR 0.54, CI 0.43-0.69)
were associated with lower odds of death. In children, death was significantly
associated with male gender, CVC, antibiotics, ICU stay, and malignancy; antifungal treatment and CVC removal were associated with lower odds of death.
Conclusions: Candidemia was strongly associated with use of CVCs, TPN,
and systemic antibiotics. Mortality from candidemia was higher in older adults,
those with underlying diseases or on systemic antibiotics, and infection with C.
krusei or C. tropicalis. Multivariable analysis will be conducted to determine
independent risk factors for mortality associated with candidemia.
420
PREVALENCE OF STREPTOCOCCAL SKIN INFECTIONS IN
AMERICAN SAMOAN CHILDREN WITH RHEUMATIC
FEVER AND RHEUMATIC HEART DISEASE
Broge T1, Gorman S1, Marrone J2, Burns JJ1. 1Florida State University Pediatric Residency Program and Sacred Heart Hospital, Pensacola, FL and
2
Lyndon B. Johnson Tropical Medical Center, Pago Pago, American Samoa.
Purpose of Study: Our study sought to identify a link between cutaneous
GAS infections and acute rheumatic fever (ARF) in American Samoa.
ARF and rheumatic heart disease (RHD) are considered endemic in the
South Pacific, particularly among indigenous populations. RHD is a known
sequelae of untreated group A streptococcal (GAS) pharyngitis, however,
there is increasing evidence implicating GAS skin infections in the pathogenesis of ARF and RHD in impetigo-endemic populations.
Methods Used: Electronic medical records of 115 American Samoan children with a history of ARF were analyzed retrospectively. Records were analyzed for history of a clinic visit in which the patients were noted to have
impetigo or sore throat prior to diagnosis with ARF. The proportion of children with ARF with history of skin infections were compared to the proportion of children with ARF with history of sore throat using McNemar’s test.
Summary of Results: 24.3% of patients were found to have had a clinic visit
for sore throat prior to being diagnosed with ARF. 47.8% of patients were
noted to have a clinic visit for impetigo prior to being diagnosed with
ARF. These were statistically different: McNemar test p value = 0.000.
Conclusions: Our findings suggest that GAS skin infections should be considered in the pathogenesis of ARF. Our data suggests that a history of symptomatic GAS pharyngitis is less common than cutaneous infections in
children with ARF. A defined link between GAS skin infections and rheumatic heart disease would have potentially significant implications for the
treatment and prevention of this disease in endemic populations.
421
ENGAGEMENT IN HIV PRIMARY CARE, USE OF
ANTIRETROVIRAL THERAPY, AND UNPROTECTED SEX
AMONG HOSPITALIZED HIV-INFECTED PATIENTS
models to identify factors associated with currently or previously having an
HIV care provider, currently taking ART, and having unprotected sex with an
HIV-negative or status unknown partner.
Summary of Results: Among 3,121 HIV-infected screener interviews, 75%
reported having ever used drugs, and 58% of them reported using non-injection
drugs in last 6 months and/or injection drugs in the last 12 months. Currently
having an HIV care provider and being on ART were associated with older
age, higher income, stable housing, lack of recent incarceration, absence of alcohol and recent drug use, and having been in substance abuse treatment. Having unprotected sex with an HIV-negative or status unknown partner was
associated with female gender, recent crack cocaine use, and recent diagnosis
of a STI. Among recent crack cocaine users, having ever been in substance
abuse treatment was associated with currently having an HIV care provider
(AOR 3.03, CI 1.80-5.09), currently taking ART (AOR 2.18, CI 1.31-3.64),
but also with having unprotected sex with an HIV-negative or status unknown
partner (AOR 1.83, CI 1.01-3.31) after adjusting for gender, age, income, education, housing, and incarceration.
Conclusions: These data suggest that substance abuse treatment is important
for engagement in HIV care and use of ART but does not appear to decrease
high-risk sexual behavior among HIV-positive drug users.
422
IS AZITHROMYCIN MODULATING INFLAMMATION VIA
TOLL LIKE RECEPTOR (TLR) PATHWAYS IN GROUP B
STREPTOCOCCUS (GBS) SEPSIS ?
Upadhyay K1, Meals E1, English B2, Talati AJ1. 1CFRI, UTHSC, Memphis,
TN and 2Michigan State University, Grand Rapids, MI.
Purpose of Study: Combination of ampicillin(AMP) and azithromycin(AZM)
improved clinical signs, mortality and cytokine levels in our mouse model of
GBS sepsis. We proposed to study if the effect of AZM in modulation of inflammation is secondary to bacterial factors[using AZM resistant strain(AZR)
of GBS] or host factors[using MyD88-/-, TLR9-/-, TLR2-/-mice].
Methods Used: Swiss Webster mice (wt 18g, age 3 wks) were injected
intraperitoneally (IP) with AZR GBS Ia (log 7-8 cfu/ml) and MyD88-/-,
TLR9-/- mice (wt 15 gm, age 3 wks) were injected with GBS Ia (log6 cfu/ml).
Mice were divided in 4 groups(grp). Grp 1 -no antibiotics, grp 2- treated with
AMP(100mg/kg/d; MIC ≤0.25 mcg/ml), grp 3- treated with AZM (10 or
50mg/kg/d; MIC 16ug/ml for AZR GBS and MIC ≤0.125ug/ml for AZM sensitive strain ) and grp 4- treated with AMP+AZM. Antibiotics were administered q24h IP. Mice were monitored using “clinical sepsis score (CSS)”. Mice
were sacrificed at 120h and blood was obtained for later cytokine assay.
Summary of Results: For AZR GBS experiment; case fatality rate was found
to be 100% in grp 1 and grp 3; 50% in grp 2 and 0% in grp 4. Mean serum TNFα was lower in grp IV(246±67 pg/ml) compared to grp 1 (6952±701 pg/ml),
grp 2 (7209±1826 pg/ml) and grp 3 (5980±626 pg/ml) (p≤0.05) The CSS
was significantly lower in grp 4 compared to other grps (fig). Also the case fatality rate was 100% in grp 1; 50% in grp 2 and 0% in grp 3 and 4 using
MyD88-/- mice. However, no difference was seen in mortality among
TLR9-/- mice. Similarly, the CSS was significantly lower in grp 3 and grp 4
compared to grp 1 and grp 2 in MyD88-/- mice but no significant difference
was seen in TLR9-/- mice.
Conclusions: The effect of AZM in modulation of inflammation may be related to TLR9 and is seen even in mice infected with AZR GBS.
Chang AG1, Bednarczyk R2, Cardenas G3, Metsch L4, del Rio C1,2. 1Emory
University School of Medicine, Atlanta, GA; 2Emory University Rollins
School of Public Health, Atlanta, GA; 3University of Miami Miller School
of Medicine, Miami, FL and 4Columbia University Mailman School of Public Health, New York, NY.
Purpose of Study: HIV-infected drug users are less likely to be engaged in
HIV primary care and be on antiretroviral therapy (ART). They are also more
likely to have high-risk sexual behaviors. We examined factors associated
with engagement in care, use of ART, and unprotected sex among hospitalized HIV-infected patients in two inner-city hospitals.
Methods Used: We performed cross-sectional analysis on screening interviews
from Project HOPE, a randomized controlled trial testing a behavioral intervention among hospitalized HIV-infected crack cocaine users in Atlanta, GA and
Miami, FL. We used bivariate analyses and multivariate logistic regression
532
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
Medical Education, Medical Ethics, and Advocacy
Concurrent Session
2:00 PM
Friday, February 21, 2014
423
INTEGRATED HANDS-ON COOKING AND CLINICAL
NUTRITION CURRICULUM IMPROVES PEDIATRIC
RESIDENTS’ COMPETENCIES AND ATTITUDES TOWARD
NUTRITION EDUCATION FOR PATIENTS
Urday P, Monlezun DJ, Harlan T. Tulane University School of Medicine, New
Orleans, LA.
Purpose of Study: Recent studies demonstrate how multiple competing education objectives within residency programs and medical schools often
complicate sufficient training in nutrition education as a hallmark of preventative medicine competencies. As a response, Tulane University School of
Medicine created The Goldring Center for Culinary Medicine. We sought
to adopt this curriculum, originally designed for medical students, for pediatric residents.
Methods Used: The Goldring Center for Culinary Medicine launched the
first two modules in its seven-module series spanning a 21-hour multidisciplinary curriculum over 3 years with integrated nutrition education and
hands-on cooking classes, guided by a professional chef and physician.
Residents’ survey responses (n=28) after the first year of curriculum participation including the first two modules were compared to baseline responses
prior to the first module. Mean scores for competencies and attitudes
according to dietary habits were analyzed using chi-square tests and nonparametric McNemar’s tests, followed by logistic regression models.
Summary of Results: Compared to responses prior to curriculum participation, more residents reported strong agreement that nutritional education
should be routine patient care (41.4% vs. 36.7%) and that physicians can influence patients’ dietary habits (41.4% vs. 37.5%). Fewer residents reported
less than suboptimal competencies in patient education on the health effects
of a low fat diet (82.1% vs. 93.9%) and assessing total calories and saturated
fat by food labels (57.1% vs. 63.3%) after their first two modules. With adequate personal daily intake of vegetables such as carrots and squash, they
were 85% less likely (versus 76% pre-curriculum) to report deficits in daily
hydration education for patients (OR=0.15, CI 0.0254-0.9517, p=0.044).
Conclusions: Our first year results indicate that The Goldring Center for Culinary Medicine’s integrated nutrition curriculum can improve resident competencies and attitudes in providing patients nutrition education. Ongoing
monitoring is underway to determine the longitudinal impact of the curriculum on residents.
424
COMPARISON OF LGBT CULTURAL SENSITIVITY
TEACHING METHODS USING THE VIRTUAL REALITY
WORLD OF SECOND LIFE VERSUS A TRADITIONAL
WORKSHOP FORMAT
Jones VF1, Rowland ML2, Brueckner-Collins J3, Mack A3, Gault S3,
Richardson A4, Hazard M5. 1University of Louisville, Louisville, KY; 2University of Louisville, Louisville, KY; 3University of Louisville, Louisville,
KY; 4University of Kentucky, Lexington, KY and 5University of Kentucky,
Lexington, KY.
Purpose of Study: The researchers sought to determine if the use of technology in the form of the virtual world of Second Life (SL) enhanced learners’
perceptions of knowledge and attitudes of Lesbian, Gay, Bisexual and Transgender (LGBT) healthcare issues.
Methods Used: Sixty-five health professional students attended a one hour
presentation designed to provide fundamental knowledge and skills in working
with LGBT youths. These students were randomly allocated to receive either a
traditional workshop or a workshop using a standardized patient encounter in
Southern Regional Meeting Abstracts
the virtual world of SL. A post-workshop survey gauged students’ interest in
and satisfaction with the traditional versus SL workshop formats.
Summary of Results: The SL group’s clinical scenarios were ranked as the
most effective component of that workshop, whereas the traditional control
group rated their paper-based scenarios as the third most effective component. The SL group felt more strongly that the session enhanced knowledge
of LGBTQ identities and community (rating of 1.9/5, with 1 as excellent and
5 as poor) than control (2.1/5). The SL group (1.6) was more convinced than
control (2.2) that the session increased their awareness of disparate health
problems within this population. Not only did Second Life enhance students’
self-perceived knowledge of barriers to healthcare specific to this population
(1.67), as compared to control (1.9), but it also increased their perceived sensitivity and compassion for LGBTQ patients (1.3), as compared to control (1.9).
Conclusions: This study provided preliminary evidence that use of virtual
world technology enhances student interest in and satisfaction with LGBT
instructional programming.
425
PRE-CLINICAL MEDICAL SCHOOL ELECTIVE TEACHING
END-OF-LIFE COMPETENCIES IMPROVES STUDENT
PREPAREDNESS IN CLINICAL ROTATIONS
Schwab Keeport ML, Cruz B. Tulane University School of Medicine, New
Orleans, LA.
Purpose of Study: Medical education in the U.S. inadequately addresses
end-of-life (EOL) and palliative care (1). We previously reported that a
pre-clinical elective increased students’ self-perception of readiness to discuss EOL-related issues at the end of the class (2). This study aims to determine if their preparedness to address these topics persists beyond completion
of the course and following a year of clinical education.
Methods Used: We designed an elective taken by twenty-five first-year medical students for ten total course hours. We have implemented it for three
consecutive years starting with the Class of 2014, in their MS-1 year. This
cohort has now completed its first and main clinical year of medical school.
A survey assessing self-perception of EOL competencies was administered
to students in the class of 2014. Results from those who completed the elective were compared with control students who had routine pre-clinical medical education. Survey data was collected using online survey instruments
and analyzed using chi-square tests via SPSS statistical software.
Summary of Results: Medical students who completed the elective in their
MS-1 year said they felt more prepared to speak to patients and/or patient
families about EOL issues than their counterparts when assessed shortly after
completion of the course (78% of elective students responding “Prepared”
compared to only 32% of controls; p < 0.001). These responses persisted
two years later into the clinical training years, where 70% of MS-3 students
who had taken the elective responded “Prepared” compared to only 36% of
students who had not taken the elective(p < 0.05).
Conclusions: The increased comfort and ability to speak about EOL-related
issues to patients and their families that students achieved following their
participation in the MS-1 elective has persisted into their clinical years. This
student-initiated elective exemplifies the usefulness and impact of teaching
EOL core competencies early in the pre-clinical curriculum.
(1) Hesselink, B. et al. (2010). Education on end-of-life care in the medical curriculum: Students’ opinions and knowledge. Journal of Palliative Medicine, 13(4), 381-387.
(2) Azevedo BM, Keeport MS, Anwar DM. “Teaching End of Life Care
to 1st Year Medical Students,” AAHPM Poster March 2013.
426
A NOVEL TEACHING MECHANISM IN NEPHROLOGY
OF THE DANGERS OF HYPOCALCEMIA IN CHRONIC
RENAL FAILURE
Sims N1,2, Dietiker K1,2, Feig D1, White M1,2, Peterson D2, Parks D2,
Youngblood A2, Zinkan L2, Tofil N1,2. 1UAB, Birmingham, AL and 2COA,
Birmingham, AL.
Purpose of Study: To standardize exposure and enhance learning.
Methods Used: We designed a simulation case to illustrate the interaction of
NaHCO3 and iCa levels in a hyperkalemic patient. Monthly simulations
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
533
Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
were conducted with residents on the renal rotation. Residents completed a
pre and post test. Scenario: 6 y/o with FSGS presents with vomiting, HA,
and abdominal pain. HR 92, BP 192/135, RR 26, O2 95% on RA. Physical
exam shows edema. Significant labs: Na130, K5.9 without hemolysis, HCO3
15, BUN 83, Cr 3.5, Ca 5.5, Phos 7.8, Alb 2.1. VBG: 7.23/35/45/15. iCa 0.8.
Peaked T waves on EKG.
Summary of Results: Twenty-one residents participated in the simulation.
Mean percentage of correct responses increased from pretest (66% +/−
24%) to posttest (97% +/− 7%), p <0.0001.Comparisons between pretest performance and year in residency did not demonstrate a significant difference
(62% +/− 22% vs. 70% +/− 26%, p=0.4). Comparison between pretest performance and number of times on nephrology service also failed to reveal
a significant difference (64% +/− 24% vs. 70% +/− 24%, p=0.5).
Conclusions: Administration of NaHCO3 and removal of hydrogen ions
from albumin causes calcium to rapidly bind to albumin resulting in abrupt
hypocalcemia leading to cardiac arrest. Residents are unaware of this physiologic mechanism regardless of year in residency or number of times on the
renal service. 40% of teams who were aware that NaHCO3 should not be
given to hypocalcemic patients gave NaHCO3 during the simulation indicating they did not understand why NaHCO3 was dangerous. This simulation
taught the physiologic interaction between NaHCO3 administration and
hypocalcemia.
modification and decreased oral cavity size. The mentum was retracted, along
with atlantoaxial instability through neck rivet alterations. Vocal cord anatomy
was retained to provide the intubation participant with a standard point of completion for securing the tracheal airway. The second phase of our project
involves putting the airway to test with direct and videolaryngoscopic
intubating techniques and is currently underway.
FIGURE 1. Difficult Pediatric Airway Modified Model
428
SHOULD PHYSICIANS TREAT THEIR RELATIVES OR
THEMSELVES? DECHANT RD, GOEBEL LJ, MUFSON MA.
MARSHALL UNIVERSITY JOAN C. EDWARDS SCHOOL OF
MEDICINE, HUNTINGTON, WV
427
DIFFICULT PEDIATRIC AIRWAY SIMULATION MODEL
DEVELOPMENT FOR STUDY EXAMINING EFFICACY
OF VIDEOLARYNGOSCOPY
Burhop J, Poirier MP, Clingenpeel J. Eastern Virginia Medical Center,
Children’s Hospital of the King’s Daughters, Norfolk, VA.
Case Report: It has been established that the videolaryngoscope is a valuable tool in securing the successful tracheal intubation of difficult airways
in adult patients. However, there is minimal available evidence to gauge its
efficacy within the infant, child and adolescent populations with anatomically difficult airways. Furthermore, the literature is virtually devoid of simulation studies in individuals with specific syndromes such as trisomy 21.
The purpose of our study is two-fold; first to develop an anatomically challenging model followed by comparison of video vs. direct laryngoscopic
techniques to secure a tracheal intubation. Additionally our study looks to
enhance safety and improve patient outcome through simulation of difficult
pediatric airway scenarios with an emphasis on laryngoscopic technology,
curriculum development and education.Anthropometric data regarding anatomical features and measurements of children with trisomy 21 was
employed during the model modification process. The starting point was a
standard newborn airway model. Macroglossia was achieved with tongue
534
DeChant R, Goebel L, Mufson M, Joan C. Edwards School of Medicine at
Marshall University, Huntington, WV.
Purpose of Study: We hypothesized that some physicians treat their
relatives for minor illnesses even though this practice is discouraged. The
AMA agrees that treatment of relatives has the potential for compromised
objectivity, incomplete evaluation and interpersonal problems in the face of
bad outcomes. To assess this issue at our university-based practice we
conducted a survey of faculty and resident physicians regarding their
attitudes and practices of treating relatives and themselves.
Methods Used: We surveyed physicians who attended one Grand Rounds in
Internal Medicine, Family Practice, OB/GYN or Pediatrics during the summer of 2013. Data was entered into an Excel spreadsheet for analysis and statistical tests applied as appropriate using the website Vassarstats.
Summary of Results: he study group comprised 30 faculty physicians and
51 residents and fellows. The response rate was 93%. Sixty-nine percent of
respondents were men, and the overall median age of respondents was 3039 years old and the median age of faculty was older, 50-59 years. One-half
of physicians felt it was “okay” to treat relatives at least sometimes. Women
physicians less often than men physicians agreed that they should treat their
relatives (p=0.004). Seventy-five percent of respondents had treated a relative for a minor illness, while 6% had treated a relative for a serious illness
(p<0.0001). Over one-third prescribed medications for family members without examining them. More than one-half treated themselves for minor
illnesses, with 21% prescribing their own medications. Two-thirds of
respondents had a primary care physician; significantly more women than
men had a primary care physician (88% vs 56%, p=0.01).
Conclusions: Our faculty and residents treated relatives and themselves often. Respondents to our survey often treated their relatives without an examination, risking all the pitfalls this lack of care incurs. Although many
physicians in our study had their own physicians, they treated themselves,
which was contrary to their best interest. More education is needed to discourage physicians from treating relatives and themselves, as optimal care
derives from your own impartial physician.
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
429
AN INVESTIGATION OF THE STATISTICAL METHODS
USED IN THE CURRENT MEDICAL LITERATURE
Narayanan R1, Nugent R2, Nugent K1. 1Texas Tech University Health Science
Center, Lubbock, TX and 2Carnegie Mellon University, Pittsburgh, PA.
Purpose of Study: Current ACGME guidelines require internal medicine
residents to develop skills in the interpretation of medical literature and to
understand principles of research. The statistical foundation and education
necessary to understand the current medical literature are uncertain.
Methods Used: We reviewed the statistical methods and terms used in 49
articles discussed at our Internal Medicine Journal Club between 1/1/2013
and 6/30/2013. We collected information on study type and on the statistical
methods used for summarizing and comparing samples, determining the
relationships between independent variables and dependent variables, and estimating models.
Summary of Results: Fourteen articles came from JAMA-IM, 11 from the
NEJM, 6 from The Annals of IM, 5 from JAMA, and 13 from other journals.
Twenty reported randomized controlled trials. Summary statistics included
mean values (39 articles), category counts (38), and medians (28). Group
comparisons were based on t-tests (14 articles), χ2 tests (21), and nonparametric ranking tests (10). The relationships between dependent and independent variables were analyzed with simple regression (6 articles), multivariate
regression (11), and logistic regression (8). Fourteen studies used sensitivity
analysis. Nine studies reported odds ratios with 95% confidence intervals,
and seven analyzed test performance using sensitivity and specificity
calculations. These papers used 128 statistical terms and context-defined
concepts, including some from epidemiology-biostatistics (31), data collection (12), data analysis (56), modeling (24), and meta-analysis (5). Based
on usual undergraduate and graduate statistics curricula, 64.8% of the
concepts and methods used in these papers required at least a master’s level
statistics education. Ten software programs were used in these articles.
Conclusions: The interpretation of the current medical literature requires a
significant background in statistical methods, and the methods used exceed
the education provided to students and residents. Given the complexity of
medical education, these deficiencies will be hard to fix, but this project
provides the basis for a curriculum in study design and statistical methods
needed by physicians.
430
INTERNAL MEDICINE RESIDENT CASE REPORT
PUBLICATIONS AND RELATED CITATIONS
Argueta E1, Nugent K2, Buscemi D1. 1Texas Tech University HSC, Lubbock,
TX and 2Texas Tech University HSC, Lubbock, TX.
Purpose of Study: Current guidelines require internal medicine residents to
participate in scholarly activity. Case report publications often provide
residents entry into the medical literature. However, journals limit the number of case reports, in part, because the citation frequency is low, and this
reduces journal impact factors. We tried to determine if this was a valid concern by reviewing publications by our residents.
Methods Used: We searched for all case report publications by internal medicine residents from Texas Tech HSC in Lubbock from 2008 through 2013.
We determined the journal impact factor using Journal Citations Reports.
We then identified all citations of articles published through 2014 using
Google Scholar and Scopus and the impact factors of the citing journal.
We also collected information on resident attitudes about case report publications using an anonymous questionnaire.
Summary of Results: This study involved 61 residents who published 56 case
reports/letters. Thirty-nine journals (69.6%) had impact factors; the median was
1.87 with an interquartile range (25%-75%) of 1.32-4.38. Twenty-five articles
had 86 citations. The types of articles citing the case reports included 36 case
reports (41.8%), 24 review articles (27.9%), 10 letters (11.6%), 9 clinical series
(10.5%), 3 research studies (3.5%), 2 book chapters (2.3%), 1 image (1.2%),
and 1 editorial (1.2%). Fifty citing journals had impact factors; the median
was 1.97 with an interquartile range of 1.29-3.90. There was no difference in
impact factors between the publishing journals and the citing journals based
on a ranking test (P= 0.40). Thirty-one residents responded to a questionnaire
Southern Regional Meeting Abstracts
on case reports. Twenty-five (80.6%) thought that the skills involved in writing
a case report would improve their core competencies. Six residents thought
completing a case report should be a mandatory requirement, 17 were neutral,
and 9 disagreed/strongly disagreed.
Conclusions: Many residents can successfully publish case reports, and
these reports are frequently cited. Case reports provide the basis for clinical
reviews for uncommon medical problems and can stimulate clinical studies.
Residents provide a critical work force for this activity; journals do benefit
through citations and increases in impact factors.
431
PHYSICIAN SPECIALISTS AND THE NRMP MATCHES
Ruiz MA. Louisiana State University Health Sciences Center, New Orleans, LA.
Case Report: Introduction The need for physician specialists is rising faster
than the modest increases in production. Supply is not keeping pace with demand, a situation already recognized in primary care. The same situation
applies to medical subespecialities.
Methods Used: This study uses data obtained from the 2010-12 GME Census of the AAMC and AMA. To illustrate the changing dynamics of the
growth of US medical schools on the access to GME positions, we
conducted a trend analysis of the data using SPSS.
Results: The results show a modest, steady increase of both medical students
and GME. The total medical school increase (‘04-‘11) is about 1005 per year
on average (1.5%/year). The total GME positions increase in the same period
is 1583 per year on average (1.7%/year). The match gap between PGY-1
positions and the medical school senior class size is approximately 6000 to
8000 per year for IMGs and others. The number of fellows matched in the
U.S. in 2012 is 6138 of 6799, about 90%2 filled. There is a relatively brief
“scramble” for the unfilled slots, and most are utilized each year. Most of
these positions are in Internal Medicine and Pediatric specialties; there are
a number of others in specialty training that do not use the fellowship title
or recognition.
Discussion: Thus, while increased numbers of at least some specialty GME
programs are underway and will help keep up with demand, further expansion is essentially prohibited by a congressional act in 1997. The supply
therefore has been “plateaued”. As reform is implemented, ever more
patients will need physicians, not only in primary care assuredly, but also a
variety of specialists.
Conclusions: While the results show that the number of medical students
are increasing, these trends indicate that an increase in GME positions
is required to accommodate the expanding medical school class size. For
example in 2004, there were 1.53 PGY-1 positions per active US Senior
compared to 1.45 positions in 2012. The current unfilled positions after
the annual NRMP match are mostly in primary care. Since the physician
shortage is and will be both in specialty and primary care, more GME
positions will be necessary.
432
THE CONTEXT OF ADOLESCENT HOPE
Bennett A1, Wood DL1,2, Goldhagen J1, Butterfield RC2, Kraemer D2. 1University of Florida-Jacksonville, Jacksonville, FL and 2UF Health at
Jacksonville, Jacksonville, FL.
Purpose of Study: Hope, reflected in one’s beliefs about the future, motivates goal-directed behavior and facilitates positive youth development.
Adolescents’ future expectations of life expectancy and educational attainment predict risk-taking behaviors, educational achievements, and health
outcomes. Previous studies have used these proxy measurements of hope
to characterize high-risk youth and their hopeless environments. The objective of this study was to use a human capital investment framework to investigate the individual and contextual assets of hopeful adolescents.
Methods Used: The public-use data (n=6,504) from Wave I of the National
Longitudinal Study of Adolescent Health was used for this analysis.
Adolescents made predictions about their chances of living to age 35 and attending college. Their predictions were assigned a numerical value and
summed to create a Hope Scale from 1-10. Those who were “almost certain”
of both were considered to have high hope. Multivariable logistic regression
compared the most hopeful, or those with a score of 10 on the Hope Scale, to
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Journal of Investigative Medicine • Volume 62, Number 2, February 2014
all others and identified variables that were significantly associated with
higher hope. The odds ratios for the unique contributions of each independent variable was determined by controlling for all other variables.
Summary of Results: Statistically significant (p<0.0001) relationships were
found between the highest sense of hope and social capital (family, neighborhood, school, and general connectedness), financial capital (household income and neighborhood poverty concentration), educational capital (parent
education), and environmental capital (connectedness scales, breastfeeding,
gender). Hope had stronger associations with social and educational capital
measurements than with financial capital measurements. Race and family
structure failed to remain significant when controlling for the other variables.
Conclusions: Hope, as a form of personal capital serves as a priceless asset
in the face of adversity. Identifying the human capital assets that serve as major determinants of health is crucial in order to guide the design of policy and
social interventions to optimize child health and well-being.
Neurology and Neurobiology
Concurrent Session
2:00 PM
Friday, February 21, 2014
433
SHAKE AND BAKE
Yu M1, Tadin D1, Engel LS1, Lopez FA1, Conrad EJ2. 1LSU Health Sciences
Center, New Orleans, LA and 2LSU Health Sciences Center, New Orleans, LA.
Case Report: Neuroleptic malignant syndrome (NMS) was first described
by the symptoms of pallor, hyperthermia, and respiratory and psychomotor
abnormalities in association with haloperidol.
Case: A 48-year-old male mental health resident with a history of schizoaffective disorder was noted by facility staff to have a stooped posture, a mild
temperature elevation and abdominal rigidity on exam. The patient was given
two doses of benztropine prior to transfer to our hospital. His mediations included clonazepam, divalproex sodium, olanzapine, and risperidone. At the
time of admission his rectal temperature was 103.1οF, heart rate 108 beats/
min, blood pressure 134/99, respiratory rate 43, and oxygen saturation
100% on room air. He appeared distressed and tremulous. He was alert
and oriented to person, place, and time but required frequent re-direction.
He was tachypneic with decreased breath sounds at the bases but without
crackles or wheezes. Bowel sounds were absent and his abdominal
muscles were visibly contracting. He had increased muscle tone and was
hyperreflexic throughout. His laboratory findings revealed a white blood
cell count of 10,800/ul with bandemia of 19% and mild thrombocytopenia
of 112,000/ul. He had rhabdomyolysis with creatinine kinase level of
103,275 u/l causing acute kidney injury and myoglobinuria. He also had
transaminitis (AST 1660 u/l and ALT 356u/l). All of his medications were
stopped. Cooling blankets, fans, and ice packs were used for his hyperthermia. He was given aggressive IV fluid repletion, placed on bromocriptine, valium as needed for agitation, and given one dose of dantrolene. Per
psychiatry, he was restarted on his home dose of divalproex sodium. His
muscle rigidity improved.
Discussion: NMS is classically associated with high potency first generation
antipsychotics. However newer atypical agents, as well as other classes of
anti-dopaminergic drugs are recognized causes. As soon as NMS is
suspected, the offending agent must be discontinued. Respiratory and circulatory systems should be monitored closely while intravenous fluids and
treatment of fever are undertaken. Treatment consists of conservative therapy, benzodiapines, dantrolene, and dopamine agonists, with electroconvulsive therapy used for refractory cases.
434
DILATED CARDIOMYOPATHY SECONDARY TO
MITOCHONDRIAL ENCEPHALOPATHY WITH LACTIC
ACIDOSIS AND STROKE-LIKE EPISODES
Graebert A, Engel LS, Lo B. LSU Health Sciences Center, New Orleans, LA.
536
Case Report: Mitochondrial Encephalopathy with Lactic Acidosis and
Stroke-like Episodes (MELAS) is a maternally inherited mitochondrial syndrome typically diagnosed in childhood or early teenage years. The strokelike episodes typically present in a relapsing-remitting manner with gradual
neurological decline leading to dementia.
Case: A 32 year old African American male with a diagnosis of MELAS,
confirmed by muscle biopsy 8 years prior, presented with a 2 month history
of shortness of breath, dyspnea on exertion, and lower extremity edema. The
patient was afebrile with a blood pressure of 108/90, heart rate of 103, respiratory rate of 20, and a room air oxygen saturation of 100%. Cardiac exam
revealed a III/VI holosystolic murmur heard best at the apex and an S3. Lung
exam was unremarkable. He had bilateral lower extremity pitting edema
extending to his hips. Laboratory studies showed a WBC count of 5.7 K/uL
with 71% neutrophils. His complete metabolic profile revealed a HCO3
18 mmol/L, BUN 38 mg/dL, creatinine 1.67 mg/dL, bilirubin 3 mg/dL,
AST 92 U/L, ALP 213 U/L, and ALT 100 U/L. BNP was greatly elevated
at >5000 pg/mL. Initial troponin was elevated at 0.14 ng/mL and peaked
at 0.17 ng/mL. Lactic acid was also elevated at 3.9 mmol/L. Urinalysis did
not contain any protein. Urine electrolytes revealed a fractional excretion of sodium of 6.7%; no urine eosinophils were present. EKG demonstrted normal sinus rhythm with evidence of left atrial enlargement, and chest x-ray revealed
mildly increased pulmonary vasculature. The patient was found to have 4 chamber enlargement, moderate to severe tricuspid regurgitation, and an ejection
fraction < 20% by echocardiogram. Diuresis with IV furosemide provided improvement in his respiratory status and edema; however, he continued to be oxygen dependent at the time of discharge.
Discussion: MELAS is a rare mitochondrial disorder that not only affects
the nervous system, but can also have cardiac, renal, and endocrine manifestations, as were seen in this patient with a dilated cardiomyopathy, renal
insufficiency, and diabetes mellitus. A hypertrophic cardiomyopathy is more
commonly associated with MELAS, but a dilated cardiomyopathy has also
been described.
435
ISCHEMIC BRAIN INJURY SECONDARY TO SEVERE
SYSTEMIC LOXOSCELISM
Soape M, Swaminath D, Whealy M, Desai V, Nugent K. Texas Tech University Health Sciences Center, Lubbock, TX.
Case Report: Rhabdomyolysis (RM), acute renal failure (ARF), and disseminated intravascular coagulation (DIC) have been frequently associated with
systemic loxoscelism. The case presented herein demonstrates these findings
plus bilateral globus pallidus lesions.
A 47 year old man was found unresponsive in a hotel and intubated at the
scene. His labs showed RM (CK 57,125 IU/L), ARF (BUN 37 mg/dL; Cr 4.1
mg/dL), hepatitis (ALT 5,721; AST 12,831) and coagulopathy (PT 20.9;
INR 2.02; PTT 35.5). He also had a high LDH (5,372) and developed a mild
anemia which would be consistent with hemolysis. A necrotic lesion formed
on his head with a raised edge and surrounding erythema. A dermatology
consult concluded the lesion was most likely secondary to a spider bite. After
successful extubation, he had weakness of his right upper extremity. A MRI
demonstrated ischemia in the bilateral globus pallidi. His labs and symptoms
improved over his remaining hospital stay with supportive care. He was
discharged home on day 12 of hospitalization.
Systemic loxoscelism is a rare complication of the bite from the spider
Loxoceles recluse or Brown Recluse. Diagnosed clinically, the lesion was
consistent with a brown recluse bite, endemic to the area of the incident.
With only speculation linking the lesion to a bite, a literature review found
only 2 confirmed bites. Understanding this diagnosis is one of exclusion, a
differential diagnoses was evaluated. Causes for RM such as illicit drug
and statin use were disproved with a negative urine drug screen and statin
therapy only started a few days prior to the event. DIC was investigated,
but blood cultures taken on admission showed no growth. This case also
demonstrates CNS injury likely due to loxosceles envenomization. The injury could be secondary to hypoxia; however, the CNS involvement would
be more diffuse. It is possible the event was secondary to thrombosis formation and DIC. A likely mechanism is a microvascular thrombosis induced by
the loxosceles venom sphingomyelinase D (SD). Also, it has been shown that
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
loxosceles venom has cytotoxic effects on the endothelial cells. This case
gives a unique presentation of brain involvement due to possible, but well
documented, pathological processes.
436
SEVERE HEADACHE AFTER A PAIN INJECTION IN
THE BACK - PNEUMOCEPHALUS AFTER EPIDURAL
STEROID INJECTION
Panikkath R, Aly M, Umezurike IC, Nugent K, Panikkath D. Texas Tech
University Health Sciences Center, Lubbock, Lubbock, TX.
Case Report:
Introduction: Epidural steroid injections are commonly performed for pain
relief. However, they can uncommonly cause significant complications, such
as pneumocephalus.
Methods: This is a case analysis and review of literature.
Results: A 78-year-old woman with chronic back pain, arthritis, and chronic
kidney disease was scheduled for an epidural steroid injection for severe
back pain. She received a steroid injection in her lower back and another
one in her neck from a pain specialist. However, as soon as she sat up after
the procedure, she developed excruciating headache. The headache was bifrontal, throbbing, 8/10 in intensity and was associated with vomiting. She
noted slight relief with lying down. There were no neurological deficits. A
CT scan of her head showed a rounded shaped hypodensities in her both lateral ventricles consistent with free air. She was admitted to the hospital, and
neurosurgery was consulted. They advised conservative management and assumption of supine position for 48 hours. She was treated with narcotics due
to the intensity of the headache. Her headache gradually resolved in 2 days.
A CT head performed after 2 days showed significant reduction in the
amount of air in the lateral ventricles. She was discharged home with advice
to report to the emergency room in the event of clinical deterioration. She did
not have any events on follow up.
Conclusion: Pneumocephalus, although is a very rare complication after epidural steroid injection, can manifest as severe excruciating headache. In contrast to the post dural puncture headache, this manifests immediately and
becomes aggravated by motion with little relief on supine position. Accidental puncture of the dura with introduction of air into the subdural space during epidural injection is believed to be the reason for this complication. The
mechanical effect of the air in the ventricle is believed to be the reason for the
headache. As little as 2ml of air can result in headache. Pneumocephalus can
often be managed with conservative management.
437
CARDIOVASCULAR RISK FACTORS PREDICT THE
SPATIAL DISTRIBUTION OF BRAIN WHITE MATTER
HYPERINTENSITIES
Banerjee S, King K, McColl R, Peshock R. *UT Southwestern Medical Center, Dallas, TX.
Purpose of Study: Increased volume of white matter hyperintensities
(WMH) are associated with cardiovascular risk factors; however, WMH are
also prevalent with normal aging. Recent studies have suggested that some
brain regions are more strongly associated with specific risk factors. The purpose of this study was to create a map of every individual brain voxel that
was significantly associated with a risk factor (hypertension, diabetes, hypercholesterolemia) as compared to the control population. Furthermore, the
study aims to create a predictive model to diagnose and prognosticate brain
injury secondary to these risk factors, based on a patient’s WMH distribution.
Methods Used: The 2066 study participants were sampled from the Dallas
Heart Study, a population based study. The participants’ WMH distributions
were generated from their MRIs using an automated algorithm developed by
Hulsey et al. (2012). For each risk factor, the subjects were divided into a
case group and a control group. Every voxel of WMH was compared between the two groups using a two tailed nonparametric permutation test.
For each risk factor, an algorithm was created that calculated the Bayesian odds ratio of having risk factor associated damage at each voxel. For
Southern Regional Meeting Abstracts
every patient, the average Bayesian odds ratio was determined and this odds
ratio approximates the likelihood of having risk factor associated damage
given a patient’s WMH distribution.
Summary of Results: Of the total of 431891 voxels that comprise the distribution of WMH over the entire population, 64998 voxels (15%) were significantly more prevalent in hypertensives compared to the controls. Similarly,
59592 voxels (14%) were more prevalent in diabetics, and 34201 voxels
(8%) were more prevalent in participants with hypercholesterolemia.
Conclusions: For hypertension, diabetes, and hypercholesterolemia, certain
voxels were significantly associated with a risk factor, and maps of these voxels
were created. Knowing the WMH distribution significantly associated with
each risk factor will improve the specificity for evaluating patients for risk factor
associated white matter injury. Additionally, a Bayesian predictive model was
developed for each risk factor that assigns an odds ratio which evaluates an individual patient’s probability of having risk factor associated injury.
438
CEREBRAL VASCULAR CYTOPROTECTIVE EFFECTS
OF HEAD COOLING DURING SEIZURES IN NEWBORN
PIGLETS: ROLE OF HEME OXYGENASE PATHWAY
Harsono M1, Parfenova H2, Leffler CW2,1, Pourcyrous M1,2. 1University of
Tennessee HSC, Memphis, TN and 2University of Tennessee HSC, Memphis, TN.
Purpose of Study: Cerebral vascular function is damaged in postictal period. Activation of Heme Oxygenase (HO) pathway during seizures protects
postictal cerebral vascular function. Selective head cooling in neonatal hypoxic ischemic encephalopathy has promising neuro cytoprotective effects.
However the role of HO pathway during head cooling is unclear. We hypothesized that cerebral vascular cytoprotective effects of head cooling in epileptic seizures are via HO pathway.
Methods Used: Cerebrovascular reactivity (CVR) was tested in: 1) Intact control (no seizures, no head cooling, no SnPP/CrMP); 2) Normothermic seizures
(only seizures induction); 3) Head cooling (only head cooling); 4) Head
cooling-Seizures (no SnPP/CrMP); 5) SnPP-Seizures (no head cooling); 6)
SnPP-Head cooling-Seizures; and 7) CrMP-Head cooling-Seizures. Newborn
piglets were anesthetized with ketamine+xylazine. Intubated, ventilated and
paralyzed with pancuronium bromide. Bicuculline was used for seizures induction. Tin protoporphyrin (SnPP) or chromium mesoporphyrin (CrMP) were
used to block HO activity. In head cooling groups, ice pads were placed around
the head 30 mins prior to seizures induction and kept during seizures. When
fully conscious, piglets were extubated and placed at animal care facility. 48h
later, CVR to physiologically relevant vasodilators was tested using closed cranial window methodology. CVR was tested using endothelium-dependent (bradykinin; hemin), astrocyte-dependent (glutamate) and smooth-muscle-dependent
(sodium nitroprusside; isoproterenol) vasodilators.
Summary of Results: In normothermic seizures group (37-38C), postictal
CVR to endothelium- and astrocyte-dependent vasodilators were reduced
compared to intact control group. In head cooling seizures group (34-35C),
postictal CVR to endothelium- and astrocyte-dependent vasodilators were
completely preserved. HO inhibitors, SnPP or CrMP, attenuated or blocked protective effects of head cooling on postictal CVR to endothelium- and astrocytedependent vasodilators.
Conclusions: Selective head cooling during seizures has protective effects
on postictal endothelium-dependent cerebral blood flow regulation. HO pathway is involved in cerebral vasoprotection of head cooling during seizures.
439
ISCHEMIC STROKE FOLLOWING SPONTANEOUS
INTERNAL CAROTID DISSECTION IN A YOUNG
PATIENT WITH HISTORY OF MIGRAINES
Rivas AM, Sotello D, Copeland S, Orellana-Barrios M, Suarez J. Texas Tech
University Health Science Center, Lubbock, TX.
Case Report: Spontaneous dissection of the carotid or vertebral arteries is a
recognized cause of cerebral ischemia and stroke. An association between
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
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Southern Regional Meeting Abstracts
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
spontaneous dissections and migraine has been suggested but not proven. We
present the case of a young, healthy patient with history of migraines who
developed a right middle cerebral artery ischemic stroke and was found to
have a dissection of the internal carotid artery (ICA).
A 31-year-old male patient with past medical history of migraines
presented to the emergency room with left-sided weakness preceded by
“the worse headache of my life”. Head CT scan and MRI showed acute ischemic injury involving the right middle cerebral artery distribution. Neck
and head CT angiogram showed total occlusion of the mid and proximal right
internal carotid artery up to C1 level with reconstitution of flow above C1
likely due to patent anterior communicating artery with low flow to the
ICA and low flow to the right MCA. Arterial Doppler of the extracranial carotid arteries showed mild plaque in the right common, internal and external
carotid arteries without stenosis but with high resistive flow noted in the right
internal carotid artery. Transthoracic echocardiogram was unremarkable as
well as workup for hypercoagulable conditions. LDL cholesterol was found
to be 103mg/dL. The patient had significant left-sided weakness, which resolved almost completely with 3 months of physical therapy.
Most ischemic events related to internal carotid artery dissection arise
from thromboembolic events due to narrowing of the arterial lumen. The exact mechanism by which spontaneous carotid artery dissection develop is not
clear yet and literature on this subject is mostly limited to case reports. Inflammatory conditions affecting the vessels have been proposed as risk
factors, more specifically altered vascular reactivity during migraine has
been thought to contribute to the development of cervical artery dissection
in isolated cases. Interestingly our patient’s symptoms of stroke were preceded by a severe headache which he thought was a migraine attack, in fact
past medical history of migraine was the only risk factor we found on this patient for arterial dissection.
440
FIRST USE OF K2, FIRST SEIZURE
Moti D, Ahmed M. Texas Tech Univ Health Sciences Center, Amarillo, TX.
Case Report: “K2”, “Spice”, Incense” is one of the synthetic, legal, intoxicating drugs (SLIDS) which is sold over the counter as bath salts and incense with
a label “not for human consumption” simply to avoid regulation by the FDA.
A 17 yr old male with no PMH was brought to the hospital by his family
after he presented with seizure-like activity. Pt was at a party when he suddenly started to have tonic clonic movement of the upper and lower extremities with rolling of the eyes, and drooling of saliva and being incontinent to
urine. The event lasted for about 2 minutes and thereafter patient was confused for about 20 minutes. Prior to this event, pt was not having any headache, nor blurring of vision, nor fever, nor chills. There is no family history
of seizure and patient, himself, had no prior history of seizure. Pt claims he
has never smoked or consumed alcohol in the past. Urine tox test was neg for
any illegal substance. Upon repeated questioning, he admitted that he used
K2 in the party for the first time. SLIDS are increasingly used in USA by
young generation. A survey of youths at a rave party indicated that 21%
had used one of them on at least one occasion. These drugs are relatively
cheap and are not detected on a standard urine screen. It can produce a sympathomimetic syndrome with features that range from pure adrenergic stimulation to a mixed picture of stimulant and hallucinogenic effects. K2
contains a synthetic cannabinoids which has full agonist mechanism of action with variable concentration and potency. Physicians need to be aware
of SLIDS in order to recognize and manage the intoxication syndrome associated with these substances when encountered in clinical practice.
441
A HAND NOT UNDER CONTROL OF THE
MIND - THE ALIEN LIMB
Panikkath R, Nugent K, Yik Lim S, Panikkath D. Texas Tech University
Health Sciences Center, Lubbock, Lubbock, TX.
Case Report: Introduction: Alien limb is a phenomenon in which a limb is
not under control of the mind. The person loses control of the limb, and it
acts as if it had a mind of its own.
Methods: This is a case analysis and literature review.
Case Report: A 77-year-old woman with chronic atrial fibrillation had her
anticoagulation stopped temporarily for spine surgery. No bridging with
low molecular weight heparin was done. Two days later while watching television, she noted an arm flinging across her visual field. She got terrified
when she realized that this was her left hand. She did not have any control
of the left arm for almost 30 minutes, and it continued to move doing purposeful movements. She later noted that her left arm was numb and slightly
weak after she regained control over it. Her husband helped her to the car to
take her to the hospital and noted that she was dragging he left lower limb
while walking. In the hospital, a CT and MRI of the brain, showed an acute
infarct in the right parietal lobe. Transthoracic and transesophageal echocardiograms did not show any evidence of clots. She gradually regained normal
power on the left side. A diagnosis of transient ischemic attack possibly due
to cardioembolic stroke was made. She was discharged home with instructions to not to stop anticoagulation without bridging with low molecular
weight heparin.
Conclusion: Alien limb syndrome or Dr Strangelove syndrome is an interesting situation in which a person loses control of his limb which starts to
act as if it had a mind of its own. It has been reported after brain surgery,
brain tumor, aneurysms, degenerative conditions of the brain, and uncommonly after stroke. Alien limb as a manifestation of cardioembolic TIA is extremely rare with only few cases reported in the literature. Alien limb persists
for variable duration of time depending on the etiology of causation. Although self-injurious behaviors like slapping and choking have been reported
in patients with alien limb syndrome, it rarely leads to significant injury.
There is no well-established drug therapy for this syndrome, although in
one case report clonazepam was been reported to be beneficial.
Perinatal Medicine I
Concurrent Session
2:00 PM
Friday, February 21, 2014
442
GENETIC ASSOCIATION OF FOLATE RECEPTOR GENES
(FOLR2, FOLR3) AND MYELOMENINGOCELE
Tenpenny JC1, Kit Sing Au P2, Northrup H2. 1University of Texas-Houston,
Houston, TX and 2University of Texas-Houston, Houston, TX.
Purpose of Study: To identify single nucleotide polymorphisms (SNPs) and
mutations within coding regions of genes involved in folate transport,
538
© 2014 The American Federation for Medical Research
Copyright © 2014 American Federation for Medical Research. Unauthorized reproduction of this article is prohibited.
Journal of Investigative Medicine • Volume 62, Number 2, February 2014
specifically FOLR2 and FOLR3 genes, and determine whether these SNPs
are associated with increased risk for myelomeningocele (MM).
Methods Used: A total of 96 patients with MM (48 Hispanic, 48 Caucasian)
were selected from a biobank of subjects with MM. Subjects were primarily
recruited from three sites (Houston, TX, Toronto, Canada, and Los Angeles,
CA). Their DNA was sequenced to identify variants across ten exons of the
FOLR2 and FOLR3 genes. Forward and reverse primers were designed for
PCR amplification of each of the five exons identified on the FOLR2 gene,
as well as for the five exons on the FOLR3 gene. These were downloaded
from NCBI website. Gel electrophoresis was used to verify exon size for
each pair of primers. Sequences of amplified products were obtained by the
Sanger sequencing method and compared to the published reference sequence. Sequences that differed from the reference sequences were considered variants. SNPs are variants previously reported in dbSNP databases,
while novel mutations are those not previously reported. Frequencies of
SNPs in MM subjects were compared to ethnically matched controls in
dbSNP. We used 2-sided Fisher’s exact method to test for association.
Summary of Results: We identified three novel and six previously described
SNPs. Of these novel variations, we found a single, large insertion variant of
173 bp in the 5′-untranslated region of FOLR2. We also identified two novel
variants in FOLR3 not previously described. Two of the six previously described SNPs, both in FOLR2, demonstrated significant association with MM.
Conclusions: We identified three previously undescribed variations including one insertion variant involving exon 1 of FOLR2 and two substitutions
near exon 2 of FOLR3 that may be associated with MM. We also demonstrated association of two SNPs in FOLR2 gene with MM. One of the
variants we described appears to involve important splicing regions of the
FOLR3 gene that may alter the transport of folate and thus contribute to
the development of MM.
443
GENETIC ASSOCIATION OF THE GLUT2 GENE AND
MYELOMENINGOCELE (MM)
Ruggiero J1,2, Northrup H2, Kit Sing Au P2. 1University of Texas - Houston,
Houston, TX and 2University of Texas - Houston, Houston, TX.
Purpose of Study: Single nucleotide polymorphisms (SNPs) within coding
regions of genes involved in glucose metabolism, specifically the GLUT2
gene, are associated with increased risk for MM.
Methods Used: We selected 96 patients (48 Caucasian, 48 Hispanic) with
MM from a cohort of subjects recruited primarily from three sites (Houston,
TX, Toronto, Canada, and Los Angeles, CA). Patient DNA was amplified
with Genomiphi and PCR reactions were completed following primer design
for each of the 11 exons of GLUT2 (RefSeq NM_000340 downloaded from
UCSC Genome Browser). Exon products of PCR reactions were verified by
gel electrophoresis for predicted sizes. Sequences of PCR products generated
by the Sanger sequencing method were compared to the GLUT2 RefSeq.
Variants not reported in the public single nucleotide polymorphisms database
(dbSNP) are considered novel variants. Novel variants will be verified by reverse sequencing. Parents’ DNA will be sequenced to determine whether the
variant is inherited or de novo. Statistical analysis with Fisher’s exact test has
been performed to compare allele frequencies between MM subjects and ethnically matched reference populations found in public databases (dbSNPs,
1000Genomes, NHLBI ESP) to evaluate association with MM.
Summary of Results: Analysis of sequencing data revealed a single base
pair deletion within the coding region of exon 11 of the GLUT2 gene in
one Hispanic study patient. A total of 8 known SNPs within the coding
and non-coding regions of GLUT2 were also identified and allele frequencies are significant for 4 SNPs in our study population.
Conclusions: We found a novel single base pair deletion that results in truncation of the GLUT2 protein at the C-terminus. The observed deletion alters
the coding frame and is predicted to alter the function of the GLUT2 protein.
We also found four SNPs that are significantly associated with risk for MM.
These variants may disrupt glucose metabolism and influence susceptibility
to MM. Glucose homeostasis is critical to embryogenesis, suggesting that altered pathways may be associated with MM. The anticipated results of this
Southern Regional Meeting Abstracts
study will warrant further investigation of the GLUT2 gene and its association with MM.
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HYPOGLYCEMIA AND/OR HYPERGLYCEMIA IN
INFANTS WITH HYPOXIC ISCHEMIC ENCEPHALOPATHY
IN THE COOLCAP TRIAL WERE ASSOCIATED WITH
POOR OUTCOME
Basu SK1, Kaiser JR1, Gunn AJ2. 1Baylor College of Medicine, Houston, TX
and 2Auckland University, Auckland, New Zealand.
Purpose of Study: Deranged glucose homeostasis is common in infants with
hypoxic ischemic encephalopathy (HIE). Our study investigates whether hypoglycemia and/or hyperglycemia during the first 48 hrs after randomization
in infants with HIE in the CoolCap Trial was associated with poor outcome.
Methods Used: We performe