Case History
Family History: Sickle Cell Trait in mother
HPI: 2 month old female with perinatal HIV exposure presented to CHoR
Emergency Department August 9, 2014 for fever, vomiting, diarrhea and
fussiness that began the day prior to admission. Fever noted as high 104 ⁰ F
at home, not responsive to over the counter antipyretics (acetaminophen).
Emesis was described as non-bloody, non-bilious and was also not posttussive. Diarrhea was non-bloody and without mucous. She had decreased
intake but no change in her urine output. No cough, congestion, respiratory
distress, diarrhea or rashes noted at admission.
She was initially admitted to General Pediatrics. Blood, urine and CSF
cultures were obtained. Inadequate CSF volume was obtained for sending
cell counts or HSV. She was started empirically on vancomycin and
ceftriaxone for rule-out sepsis. Macular rash subsequently developed over
her trunk on August 10, day off illness #2 with perioral dermatitis, cracked
lips, and desquamating diaper dermatitis. Abdominal distension developed
on August 11, day off illness #3 and nasogastric sump placed. On August 14,
day of illness #5, she developed worsening tachycardia, tachypnea, a new
oxygen requirement, generalized edema, oliguria, diarrhea, worsening
anemia and thrombocytopenia. She was transferred to the PICU where
central access was obtained, she was intubated and doxycycline was added
to her antibiotic regimen. She required platelet and packed red blood cell
transfusions. She continued to spike fevers and remained intubated without
an identified etiology of her illness. She was given an empiric course of IVIG.
The patient continued to spike fevers and have intermittent bouts of
diarrhea until Aug 22, day of illness #14.
PMH:
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Full term – mom HIV +, undetectable viral load. Other labs benign
Sickle Cell Trait
Perinatal HIV exposure – completed 6 weeks AZT with negative PCR
testing at birth, 4 weeks, 8 weeks of age
PSH: None
Social History:
 Lives with mom and dad
 Family from Sierra Leona (have been in US for 5 years)
Allergies: No known allergies
Medications: Tylenol as needed
Physical Exam – at time of PICU Transfer
Vital Signs: T 38.0 C, BP 97/74, HR 182, RR 54, SpO2 96% on 2L NC, Wt 6.8 kg
(up 800 g since admission)
General: crying in mom’s arms, not easily consolable, generalized edema
HEENT: Anterior fontanel soft and flat, no scleral icterus, + conjunctival
injection with clear drainage and desquamation of lids, erythematous and
cracked lips with erythematous tongue, no intraoral lesions
Neck: supple
Resp: head bobbing, nasal flaring; good aeration bilaterally without
wheezes, rales or rhonchi
CV: tachycardic, no murmurs appreciated. Dorsalis pedis pulse 2+, diffuse
non-pitting edema of extremities and face noted.
Abd: soft, distended, normal bowel sounds present. Palpable liver edge 2 cm
below costal margin, palpable spleen tip, reducible umbilical hernia
MSK: transverse brown lines on fingernails
Neuro/Psych: awake, alert, tracking, no focal deficits, spontaneously moving
all extremities
Integument: erythematous diaper dermatitis with desquamation; macular
erythematous rash across trunk
Laboratory and Imaging
Admission Labs (8/10):
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WBC – 20.8 x 109/L (N 64%, L 28%, M 6%, E 2%), Hemoglobin – 10.4 g/dL,
Hematocrit – 31.6%, Platelets – 275 x 109/L
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Na – 135 mmol/L, K – 4.6 mmol/L, Cl – 105 mmol/L, HCO3 – 23 mmol/L,
BUN – 7 mg/dL, Cr – 0.22 mg/dL, Glu – 135 mg/dL, Ca – 9.6 mg/dL
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AST – 50 U/L, ALT – 32 U/L, Alk Phos – 206 U/L, Bilirubin – 0.8 mg/dL,
Protein – 5.4 g/dL, Albumin – 3.5 g/dL
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CRP – 9.6 mg/dL
Additional Work-Up:
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Blood culture 8/10: Negative
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Urine Culture 8/10: Negative
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CSF Culture 8/10: Negative
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CSF Enterovirus 8/11: Negative
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Respiratory Pathogen Panel: Negative
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West Nile Virus: Negative
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Enterovirus (Stool): Negative
Echo (8/14): trivial mitral regurgitation, normal left ventricle and size, normal
coronary arteries, trivial pericardial effusion, small ASD vs PFO
CXR (8/10): No focal consolidation, effusion or pneumothorax
Abd US (8/14): Mild hepatomegaly, small intraperitoneal free fluid
Department of Pediatrics
Clinicopathologic Conference
February 3, 2015
Bone Marrow Biopsy (8/15): Normocellular
Admission Medications:
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Vancomycin
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Ceftriaxone
Continued Laboratory Studies 8/14/14:
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WBC – 14.0 x 109/L (N 86%, L 8%, M 3% E 2%); Hemoglobin – 8.4 g/dL,
Hematocrit – 25.8%, Platelets – 30 x 109/L
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CRP – 15.5 mg/dL
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PT – 19.4 sec, INR – 1.7, PTT – 50 sec, Fibrinogen – 265 mg/dL, D-Dimer –
3.04 mcg/mL FEU
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AST – 32 U/L, ALT – 24 U/L, Alk Phos – 100 U/L, Bili – 0.6 mg/dL, Protein –
3.0 g/dL, Albumin – 1.8 g/dL
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Zinc: < 25 ug/dL, Copper – 81 ug/dL
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Triglyceride – 198 mg/dL
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Ferritin – 813 ng/mL
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Anti-ds DNA Ab: < 12.3 IU/mL
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Haptoglobin – 142 mg/dL, Lactate Dehydrogenase – 335 U/L
A confirmatory laboratory test was sent
2 month old female with fever, diarrhea and rash