Sickle Cell Disease and Glucose-6-Phosphate Dehydrogenase

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Abstract
The incidence of G-6-PD defect was 3 times as high in sickle cell disease as in the general population of Ghana. It was higher in females than males, in sickle cell anemia than in [sickle cell] disease, and in the Akan-Fanti patients as compared with the GaKrobo cases. The level of activity tended to be high at the time of crisis and low after promazine therapy. There was good agreement between the Brewer and quantitative tests although the former was more sensitive, in cases proven by genetic study. Red cell survival was slightly longer in cases with the enzyme defect, which seems to have a favorable effect on the course of sickle cell disease.