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

Abstract

The prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell trait was determined in 371 Cameroonian males and 668 male blood donors in Chicago. The number of males with both sickle cell trait and G6PD deficiency was significantly greater than expected (p < 0.05) in Cameroon. The number of males with both sickle cell trait and G6PD deficiency in the Chicago population also exceeded the expected number, although this was not statistically significant (p > 0.30). A young red cell population associated with the sickle cell gene leading to elevated G6PD levels in G6PD-deficient males suggests that sickle hemoglobin may exert a beneficial effect on G6PD deficiency, rather than the opposite, as had previously been proposed. These red cells may be better able to deal with oxidative stress, which can precipitate severe hemolytic disease in G6PD deficiency.

Hematological profile of twenty-nine tribal compound cases of hemoglobinopathies and G-6-PD deficiency in rural Orissa

“…It suggests that sickle cell disease patients with G-6-PD defi ciency are prone to increased hemolysis when exposed to drugs or oxidative stress. These fi ndings are contradictory to Bernstein et al [9] in that a young red cell population associated with the sickle cell gene leading to elevated G-6-PD levels in G-6-PD defi cient males suggests that sickle hemoglobin may exert a benefi cial effect on G-6-PD deficiency, rather than the reverse. These red cells may be better able to deal with oxidative stress, which can precipitate severe hemolytic disease in G-6-PD defi ciency.…”

“…sickle cell disease and G-6-PD defi ciency has been the subject of a number of studies in different populations. [7][8][9][10]12,14,15,17,26] The state of Orissa is hyper endemic for malaria and the high frequency of these disorders is related to selective advantage against malaria. [27] The present study reported 29 cases affl icted with double hereditary hemolytic disorders for the fi rst time in two tribal communities of Sundargarh district in northwestern Orissa.…”

“…[3,6] The reports regarding the relationship of G-6-PD deficiency with sickle cell disease are confl icting. In areas of prevalence of both these genes, their coincidence has been considered signifi cantly higher by some workers, [7][8][9][10] while others have not supported this observation. [11,12] Similarly, controversy has prevailed regarding the benefi cial, deleterious or neutral effects of G-6-PD deficiency on the clinical status of sickle cell disease.…”

“…[11,12] Similarly, controversy has prevailed regarding the benefi cial, deleterious or neutral effects of G-6-PD deficiency on the clinical status of sickle cell disease. [9,10,[12][13][14][15][16][17] Although both hemoglobinopathies and G-6-PD deficiency are prevalent in malaria endemic areas in India, to the best of our knowledge, no study has ever reported combined conditions in a single individual from India. The present study highlights 29 tribal cases with compound occurrence of hemoglobinopathies and G-6-PD defi ciency in a randomly conducted study in Sundargarh district of northwestern Orissa.…”

Influence of sickle heterozygous status and glucose-6-phosphate dehydrogenase deficiency on the clinico-haematolgoical profile of <i>Plasmodium falciparum</i>-infected children

“…Reports on the effect of G6PD deficiency on patients with sickle cell trait have been controversial. Bernstein et al [15] claimed that the combination was beneficial while Steinberg et al 16 found no beneficial or adverse relation in co-inheritance. In this study we investigate the contribution of sickle haemoglobin and G6PD deficiency gene in the clinico-haematological profile of falciparum malaria infected subjects with intent to throw more light into the role these genetic defects could play in the protection against malaria severity.…”