Sickle Cell Disease and Glucose-6-Phosphate Dehydrogenase

Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease in Brazil

“…The results in the group of SC patients suggest higher frequency of defi ciency than among SS or S/B-thal patients al though the number of studied SC patients was small and there is no statistical significance. These data cannot be easily explained, but in dicate that a larger group of SC patients should be studied, since the few reports on G-6-PD deficiency in SC present lower fre quencies than those on SS patients [3,4]. In the group of patients studied here, we found no difference for the hemoglobin con centration and reticulocyte counts between the G-6-PD-and G-6-PD+ male patients.…”

“…Our re sults are in agreement with those reported by Bienzle et al [ 1 ] in Nigeria and Steinberg et al [7] in a large US study. In contrast, the higher frequency of G-6-PD deficiency among SS pa tients was described in Jamaica [2], New York [6], Ghana [3] and Saudi Arabia [8], These dis crepancies may be the result of several factors, including the study of a small number of pa- tients, the use of methods for detection of G-6-PD that are unreliable in patients with sickle cell disease and the study of populations with different genetic backgrounds [7], The racial background of the Brazilian population with sickle cell disease is different from those of the American, Jamaican and African pop ulations, where the largest investigations were performed. Although the HbS gene is of Afri can origin, there is a more significant admix ture with Europeans, as indicated by a large proportion of patients classified as 'light mullatoes' [23], In this population, the frequencies of G-6-PD A -and G-6-PD A among SS pa tients were both slightly lower than those re ported in Nigeria [1], Jamaica [2] and some US populations [7,12].…”

“…Several reports have suggested that the incidence of G-6-PD defi ciency is higher in SS patients than in the general population [1,3,8,10,11], although others do not confirm this association [1,7,12]. In addition, the possible influence of G-6-PD deficiency upon the clinical manifes tation of SS is also the subject of contradic tory reports among different populations [1,7,10,13].…”

Interaction of Sickle Cell Trait and Glucose-6-Phosphate Dehydrogenase Deficiency in Cameroon

“…Since then, numerous studies have verified the finding that G6PD deficiency is more common not only in individuals with sickle cell anemia, but also in those with sickle cell trait [8,14,15,17,18,28]. To account for the increased prevalence of G6PD deficien cy with Hb S, Lewis and Hathorn [16] sug gested that G6PD deficiency exerts a benefi cial effect on the course of sickle cell ane mia.…”

Glucose-6-Phosphate Dehydrogenase Electrophoresis in Ghanaians with AA and SS Haemoglobin

“…The average figure for all surveys in Ghana is 17% (table II). In Ghana, a higher than expected incidence of G -6-PD deficiency has been reported in neonatal jaundice [5], infectious hepatitis [8,9], in ty phoid fever [10J and in sickle cell disease [11]. These findings arc sum marized in table III.…”