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What is All of the following statements are true about sickle cell disease EXCEPT: and explain in detail?
- Patients of Sickle cell anemia rarely presents before 6 months age as abundance of HbF inhibits polymerisation of HbS. CLINICAL FEATURES OF SICKLE CELL ANEMIA: Hand foot syndrome / dactylitis of bones of hands, feet Priapism & erectile dysfunction Stroke & retinopathy- loss of visual acuity & blindness Auto splenectomy d/t splenic INFARCTS (Spleen becomes non palpable) Chronic hemolysis - pt may require frequent blood transfusions Renal Involvement- papillary necrosis & hyposthenuria cardiomegaly Infections mainly by encapsulated bacteria . - Fever in a child with sickle cell anemia is a medical emergency, require prompt medical evaluation and delivery of antibiotics because of increased risk of bacterial infection and subsequent high moality rate. - Positive correlation is seen b/w concentration of HbS & polymerization of HbS