Glucose‐6‐Phosphate Dehydrogenase Deficiency and Homozygous Sickle Cell Disease in Jamaica

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Abstract
The relationship between D-glucose-6-phosphate:NADP oxido-reductase (glucose-6-phosphate dehydrogenase; G6PD) deficiency and homozygous sickle cell (SS) disease was examined in 120 patients. The proportion of hemizygotes (22.6%) was slightly more than that observed, and the combined proportions of heterozygotes and homozygotes (28.3%) were slightly less than would be expected in the general population, but the differences were not significant. The proportion of patients of abnormal G6PD status in the 10-19 yr age group was 41.7%, significantly more than that found in the 20-29 yr age group (0.02 < P < 0.05) or expected in the general population (P = 0.05). Possible reasons for this were discussed. Difference in G6PD status did not affect the total Hb concentration, reticulocyte count, unconjugated serum bilirubin or Hb F concentration, irreversibly sickled cell counts or plasma Hb concentration, and there was no demonstrable correlation between clinical severity or leg ulceration and abnormal G6PD status.