Prevalence of Glucose-6-Phosphate Dehydrogenase Deficiency in Sickle-Cell Disease

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Abstract
Glucose-6-phosphate dehydrogenase genotypes were determined in 24 male patients with sickle-cell disease from 21 families and from 24 male sibs, including at least one from each family. The frequency of A- genotype of glucose-6-phosphate dehydrogenase was somewhat higher in the patients with sickle-cell disease than has been reported in the general Afro-American population; however, the prevalence of that genotype in brothers of patients with sickle-cell disease was even higher. These findings are not consistent with the proposal that glucose-6-phosphate dehydrogenase deficiency confers protection upon patients with sickle-cell disease. They can be explained by the fact that the frequency of the deficiency will parallel the frequency of the sickle-cell gene as genes of African origin are diluted by other gene pools. (N Engl J Med 290:826–828, 1974)