39 Days Smarter!!
MORNING REPORT
T U E S D A Y , A U G U S T 9 TH, 2 0 1 1
CXR:
~Superior segment RLL
PNA
~Early LLL
Complications of Sickle Cell
Disease
Quick Note on Pathogenesis of SCD…
 Single nucleotide substitution in the beta globin gene
on chromosome 11 substitution of valine for
glutamic acid polymerization of HgS on
deoxygenation multiple polymers bundle into rodlike structures that distort RBC into crescent shape
vascular occlusion, organ ischemia, end organ
damage
 AR in inheritance
Clinical Manifestations
 Appear during first postnatal year
 Due to decrease in HbF

Inhibits deoxy-HbS polymerization in RBC
 Severity based on Hb varient:
 SS (Hb 6-8)
 S-beta0-thalassemia (Hb 7-9)
 SC (Hb 10-14)
 S-beta+-thalassemia (Hb 9-12)
Clinical Complications of SCD
 Young children
 Bacterial infection
 Splenic sequestration
 Stroke
 Adolescents and adults
 Pulmonary HTN
 Renal disease
 Stroke
 Avascular necrosis
 Leg ulcers
 Chronic pain syndromes
2nd leading
cause of
admissions after
VOC
More common
in children but
more severe in
adults
Acute Chest Syndrome
Acute Chest Syndrome
 Definition:
 The radiologic appearance of new pulmonary infiltrate
involving at least one complete lung segment plus one of the
following
 Fever
>38.5
 Hypoxia
 Chest pain
 Signs of respiratory distress (tachypnea, wheezing, cough,
retractions)
Acute Chest Syndrome
 Triggers:



Infection (bacteria, viruses, Mycoplasma, Chlamydia)
VOC (fat embolism, hypoventilation)
Asthma
 Treatment:






Broad spectrum Abx (cephalosporin + macrolide)
Oxygen (Pox>92%)
Hydration
Incentive Spirometry
Early intervention with simple transfusion
Exchange transfusion

Reserved for patients with progressive, multilobe infiltrates and
hypoxia
Aplastic Crisis
 Most common cause of transient red cell aplasia
 Presentation:
 Fever
 URI Sx
 Fatigue
 Pallor (with absence of scleral icterus)
 Decrease in baseline Hb with reticulocytopenia

7-10 days
 Dx: Parvovirus B19 IgM
 Tx: Supportive care (transfusion)
Osteomyelitis
 Salmonella*, S. aureus
 Clinical presentation with significant overlap with
VOC
No definitive imaging modality can differentiate with certainty
 Diagnosis on clinical assessment
 Fever
 Leukocytosis
 ESR
 +BCx

Bacteremia/ Sepsis
 Functional asplenia
 At 1 yo 30%
 At 6 yo 90%
 Risk of S. pneumoniae bacteremia 400-fold higher in
SCD
 Prevention


PCN
Pneumococcal immunizations
 Parenteral Abx (ceftriaxone) still standard of care
with temp>101
Acute Splenic Sequestration
 Occurs in children<3 yo with HbSS and at any age
with the other HbS varients
 Clinical Characteristics




Sudden enlargement of spleen
2 g/dL drop in HgB from baseline
Reticulocytosis
+/- Thrombocytopenia
 Frequently results in
circulatory collapse!!
Cerebrovascular Disease
 Significant cause of morbidity in children with SCD
 Stroke in HbSS patients
 11% overt stroke by age 20


Additional 22% with silent strokes


75% ischemic or thrombotic
Neuropsychometric defecits learning and cognitive problems
Peak incidence 2-10 yo
 Large artery disease
 Internal carotid
 Anterior and middle cerebral arteries
Cerebrovascular Disease
 Prevention
 TCD
Screening recommended in all
children between 2-16y
 Detecting children at risk
 Flow velocity >200cm/sec


Chronic transfusion therapy
Goal: HbS <30%
 Prevents second stroke in 80%
 Reduces stroke risk 10 fold for
patients with at risk TCDs

Chronic Organ Damage
 Pulmonary HTN
 Renal disease
 Avascular necrosis
 Leg ulcers
 Priapism
 Cholelithiasis
 Chronic pain syndromes
Thanks for your attention!
NOON CONFERENCE: CARDIAC SURGICAL
REPAIR, DR. JASON TURNER