Abstract
Sickle-cell disease: a call to action
Highlights
The inherited disorders of haemoglobin are the commonest monogenic diseases
The hypothesis of five independent origins, four in sub-Saharan Africa and one in the Middle East and India (Arab-India) is almost universally accepted, both early and recent work supported the alternative hypothesis of a single origin.[6,7]
Significant differences in the level of foetal haemoglobin (HbF) between the sub-Saharan African haplotypes and the Asian might suggest that the sicklecell mutation has arisen at least twice
Summary
The inherited disorders of haemoglobin are the commonest monogenic diseases. It has been estimated that over 300 000 babies are born each year with one of these conditions, either sickle-cell disease or severe forms of thalassaemia.[1]. The hypothesis of five independent origins, four in sub-Saharan Africa (named Bantu, Benin, Cameroon and Senegal) and one in the Middle East and India (Arab-India) is almost universally accepted, both early and recent work supported the alternative hypothesis of a single origin.[6,7] Significant differences in the level of foetal haemoglobin (HbF) between the sub-Saharan African haplotypes and the Asian might suggest that the sicklecell mutation has arisen at least twice.
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