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April 30, 1973

Sickle Cell Disease and Trait in White Populations

Author Affiliations

From the Palo Alto Medical Research Foundation and the Palo Alto Medical Clinic, Calif (Dr. Gelpi), and the Medical Department, Arabian American Oil Company, Dharan, Saudi Arabia (Dr. Perrine).

JAMA. 1973;224(5):605-608. doi:10.1001/jama.1973.03220180029007

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.