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Sickle Cell
Disease
HbSC disease
Compound heterozygosity for HbS and HbC genes
HbSE disease
Compound heterozygosity for HbS and HbE genes
HbS-β-thalassemia disease
Compound heterozygosity for HbS and a β0- or β+-
thalassemia gene
HbSO Arabia
Compound heterozygosity for HbS and HbO Arabia
HbSD Los Angeles (Punjab)
Compound heterozygosity for HbS and HbD
Rare complications
Leg ulcers, priapism, nephropathy+renal failure, severe anemia
Infections
Major cause of death in children and adults
Hydroxyurea
Only FDA-approved drug for primary Rx
Begin early, before irreversible vasculopathy and organ
damage develop
Reduces of acute painful events and acute chest syndrome
Reduces mortality 40% and reduces hemolysis
Fewer hospitalizations, reduced medical costs
Improved physical capacity
Hypertension
Cause of ventricular hypertrophy and HF
May contribute to sickle nephropathy and renal failure
Chest pain
Very common but MI unusual