title: Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia.
abstract: We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS. A total of 801 male patients over age 2 had G6PD electrophoresis on cellulose acetate membranes. Assays of both G6PD activity and hexokinase activity were then done on all samples that had an electrophoretic pattern other than the normal wild type (GdB). The collection of clinical data used a standardized protocol. Using cluster analyses we classified 10.4% males to be G6PD deficient, while 18.4% had the functionally normal GdA+ enzyme. The prevalence of G6PD deficiency did not change significantly when age was stratified by decade, suggesting little survival advantage or disadvantage of the combination of G6PD deficiency and HbSS. Compared to patients who were not G6PD deficient, there were no significant differences in the hemoglobin concentration, mean corpuscular volume, reticulocyte count, bilirubin, or SGOT level in patients with HbSS who had G6PD deficiency. The incidence of painful episodes, sepsis, or acute anemic episodes was similar in both groups. Our results are consistent with recent studies of smaller numbers of patients that have found little influence of G6PD deficiency upon HbSS. Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS.
authors.affiliation: Jackson VA Medical Center MS 39216.
authors.forename: M H
authors.initials: MH
authors.lastname: Steinberg
authors.forename: M S
authors.initials: MS
authors.lastname: West
authors.forename: D
authors.initials: D
authors.lastname: Gallagher
authors.forename: W
authors.initials: W
authors.lastname: Mentzer
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D004588:Electrophoresis, Cellulose Acetate; D005838:Genotype; D005954:Glucosephosphate Dehydrogenase / Q000145:classification / Q000378:metabolism; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications* / Q000201:enzymology / Q000235:genetics / Q000503:physiopathology; D006593:Hexokinase / Q000378:metabolism; D006801:Humans; D008297:Male; D012805:Sickle Cell Trait / Q000150:complications* / Q000503:physiopathology
chemical_list: D005954:Glucosephosphate Dehydrogenase; D006593:Hexokinase