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39 Days Smarter!!. Morning report Tuesday, August 9 th , 2011. CXR:. ~Superior segment RLL PNA ~Early LLL . Complications of Sickle Cell Disease. Quick Note on Pathogenesis of SCD…. - PowerPoint PPT Presentation
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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 RBCSeverity 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 VOCMore 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 daysDx: 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!