[Prevalence and morbidity of G6PD deficiency in sickle cell disease in the homozygote].

Abstract

Sickle cell disease and G6PD deficiency have similar prevalence of 8 to 10% in Senegalese population. Our objectives were to determine the prevalence of G6PD deficiency in Hb S carriers and normal subjects, and to assess the interaction of G6PD deficiency on clinical severity of sickle cell disease. G6PD activity was measured in 319 sickle cell patients and in 318 subjects without HbS. Clinical severity was compared in male homozygous sickle cell patients (11 with G6PD deficiency and 19 without deficit). In homozygous sickle cell patients, the G6PD status was assessed after correction of reticulocyte count following the micro-centrifugation method of Herz. We found that prevalence of G6PD deficiency was higher in sickle cell disease patients (21.6 %) than in normal subjects (12.3 %) (p = 0.001). No difference was found in the two groups of male sickle cell disease patients concerning number of vaso-occlusive crisis, number of transfusion, frequency of infectious episodes, number of chronic complications, disturbances on patient's activity and total index severity.