MORNING REPORT TUESDAY, AUGUST 9 T H , 2011

39 Days Smarter!!

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 rod-like 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

Acute Chest Syndrome

2nd leading cause of admissions after VOC

More common in children but more severe in adults

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 IgMTx: Supportive care (transfusion)

Osteomyelitis

Salmonella*, S. aureusClinical 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

75% ischemic or thrombotic Additional 22% with silent strokes

Neuropsychometric defecits learning and cognitive problems

Peak incidence 2-10 yoLarge 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 HTNRenal diseaseAvascular necrosisLeg ulcers PriapismCholelithiasisChronic pain syndromes

NOON CONFERENCE: CARDIAC SURGICAL REPAIR, DR. JASON TURNER

Thanks for your attention!