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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  2 nd 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 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  75% ischemic or thrombotic  Additional 22% with silent strokes  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

NOON CONFERENCE: CARDIAC SURGICAL REPAIR, DR. JASON TURNER Thanks for your attention!