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1. Sickle Cell Disease and Acute Chest Syndrome
Joehar Hamdan
2. Quick Review Autosomal recessive Single amino acid substitution in beta globin chain: Glu ? Val at 6th AA ? Hgb S Poorly soluble when deoxygenated ? polymerization ? elongated rope-like fibers ? RBC deformation Low Hgb with high Reticulocyte count