Best Practice topics for July

Extract from BMJ Best Practice, looking this month, at Sickle Cell Anaemia

Sickle cell anaemia is a disease of red blood cells caused by an autosomal-recessive single gene defect in the beta chain of haemoglobin.  The characteristic crescent-shaped haemoglobin can disrupt blood flow and break. There is a predisposition for obstruction of small blood capillaries, causing painful crises, organ damage, and increased vulnerability to severe infections. Infants are screened, with findings confirmed by haemoglobin electrophoresis, FBC, reticulocyte count, and peripheral blood smear. Treatment goals include fluid replacement therapy, pain management, and symptom control.

and related conditions

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