Sickle Cell Disease and Trait in White Populations

Armand P. Gelpi, MD; Richard P. Perrine, MD
JAMA. 1973;224(5):605-608. doi:10.1001/jama.1973.03220180029007.
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The sickle cell gene is widely distributed among populations of southern Europe, the Middle East, and India, reflecting diffusion of African genetic material during a millennium or more of the east African slave trade. Mild homozygous sickle cell disease, reported among Eti-Turks and Arabs from Kuwait and Saudi Arabia, may in part represent favorable interaction between sickle cell disease and α-thalassemia or hemoglobin H disease. Favorable interaction between glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and sickle cell disease, in populations where high frequencies of the enzyme defect and the sickle cell trait coexist, has been postulated but certainly not established. A better understanding of sickle cell disease may be afforded by further studies of the problem in populations with high frequencies of the thalassemia traits and G-6-PD deficiency occurring in association with sickle cell disease.


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