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Acute complications of sickle cell disease in children

Abstract

Sickle cell disease is a recessively inherited condition in which synthesis of haemoglobin is abnormal. The disease, which occurs mainly in people of African, African-Caribbean, Indian, Mediterranean and Middle Eastern descent, is characterised by chronic anaemia, susceptibility to infection, bouts of severe pain and organ dysfunction. While the life expectancy for patients has improved, from a median survival age of 14 years in the 1970s among those homozygous for the sickle haemoglobin gene to survival into the mid-40s, childhood remains a period of peak mortality and morbidity.1 Here, we discuss the acute complications of sickle cell disease in children, concentrating on the management of the acutely unwell child.

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