Sickle cell anaemia:
• genetic disorder – defect in synthesis of beta chain of haemoglobin production of abnormal haemoglobin (Hb S)
• sickle cell increase blood viscosity, travel poorly through capillaries and tend to obstruct flow tissue infarct
• have sickling crises from thrombosis
• clinical features – retarded intellectual development; cardiopulmonary insufficiency; recurrent infections
• avascular necrosis (especially femur and humerus)
• management –
• prevention – genetic counselling; prenatal tests
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