scholarly journals Glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia: frequency and features of the association in an African community

Blood ◽  
1975 ◽  
Vol 46 (4) ◽  
pp. 591-597
Author(s):  
U Bienzle ◽  
O Sodeinde ◽  
CE Effiong ◽  
L Luzzatto
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dehydrogenase Deficiency ◽  
Clinical Course ◽  
Normal Subjects ◽  
Starch Gel Electrophoresis ◽  
African Community ◽  
Starch Gel ◽  
Male Patients ◽  
Glucose 6 Phosphate Dehydrogenase

The glucose 6-phosphate dehydrogenase (G6PD) genotype was determined in 100 male patients with homozygous sickle cell anemia (SS) by a combination of quantitative assay, cytochemical testing, and starch-gel electrophoresis. Of the 100 patients tested, 16 were found to be G6PD deficient (GdA-), AND 84 G6PD normal (22GsA and 62 GdB). This distribution of G6PD genotypes did not differ significantly from that observed in the general population. The level of G6PD activity in GdA- SS patients was nearly always higher than in G6PD-deficient subjects who did not have an associated hemolytic state, but it was nearly always lower than in G6PD-normal subjects. The clinical course of sickle cell disease, including the degree of anemia, was not milder in GdA- than in G6PD-normal patients but could not be proved to be significantly more severe. It was concluded that in this community the incidence of G6PD deficiency in sickle cell anemia was not greater than would be expected by chance, and there was no evidence that the coexistence of the GdA- gene in SS patients ameliorated their disease.

scholarly journals Glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia: frequency and features of the association in an African community

Blood ◽  
1975 ◽  
Vol 46 (4) ◽  
pp. 591-597 ◽  
Author(s):  
U Bienzle ◽  
O Sodeinde ◽  
CE Effiong ◽  
L Luzzatto
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dehydrogenase Deficiency ◽  
Clinical Course ◽  
Normal Subjects ◽  
Starch Gel Electrophoresis ◽  
African Community ◽  
Starch Gel ◽  
Male Patients ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract The glucose 6-phosphate dehydrogenase (G6PD) genotype was determined in 100 male patients with homozygous sickle cell anemia (SS) by a combination of quantitative assay, cytochemical testing, and starch-gel electrophoresis. Of the 100 patients tested, 16 were found to be G6PD deficient (GdA-), AND 84 G6PD normal (22GsA and 62 GdB). This distribution of G6PD genotypes did not differ significantly from that observed in the general population. The level of G6PD activity in GdA- SS patients was nearly always higher than in G6PD-deficient subjects who did not have an associated hemolytic state, but it was nearly always lower than in G6PD-normal subjects. The clinical course of sickle cell disease, including the degree of anemia, was not milder in GdA- than in G6PD-normal patients but could not be proved to be significantly more severe. It was concluded that in this community the incidence of G6PD deficiency in sickle cell anemia was not greater than would be expected by chance, and there was no evidence that the coexistence of the GdA- gene in SS patients ameliorated their disease.

scholarly journals Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia

Blood ◽  
1988 ◽  
Vol 71 (3) ◽  
pp. 748-752 ◽  
Author(s):  
MH Steinberg ◽  
MS West ◽  
D Gallagher ◽  
W Mentzer
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
G6pd Deficiency ◽  
Electrophoretic Pattern ◽  
Hemoglobin Concentration ◽  
Wild Type ◽  
Standardized Protocol ◽  
Cellulose Acetate Membranes ◽  
Male Patients ◽  
Glucose 6 Phosphate Dehydrogenase

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.

Enhanced Erythrocyte Apoptosis in Sickle Cell Anemia, Thalassemia and Glucose-6-Phosphate Dehydrogenase Deficiency

2002 ◽  
Vol 12 (5-6) ◽  
pp. 365-372 ◽  
Author(s):  
Karl Lang ◽  
Benjamin Roll ◽  
Svetlana Myssina ◽  
Markus Schittenhelm ◽  
Hans-Gerhard Scheel-Walter ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dehydrogenase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase

CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIA ASSOCIATED WITH ERYTHROCYTE GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY IN A NEGRO FAMILY

PEDIATRICS ◽  
1966 ◽  
Vol 37 (4) ◽  
pp. 624-629
Author(s):  
Aaron Grossman ◽  
K. Ramanathan ◽  
Parvin Justice ◽  
Joan Gordon ◽  
Nasrollah T. Shahidi ◽  
...  
Keyword(s):  
Enzyme Activity ◽  
Hemolytic Anemia ◽  
Gel Electrophoresis ◽  
Dehydrogenase Deficiency ◽  
Red Cells ◽  
Starch Gel Electrophoresis ◽  
Negro Family ◽  
Starch Gel ◽  
Normal Enzyme ◽  
Glucose 6 Phosphate Dehydrogenase

This paper describes a Negro family with congenital nonspherocytic hemolytic anemia associated with glucose-6-phosphate dehydrogenase deficiency. All four affected males in this family showed a hemolytic anemia characterized by low hemoglobin, reticulocytosis, and jaundice. There was no detectable G-6-P D in the red cells and about a tenth of normal enzyme activity in the white cells. By starch gel electrophoresis, the G-6-P D was present as a single band which migrated at the rate of 104% of normal. Physico-chemical studies revealed a marked increase of the Michaelis constant for both G-6-P and TPN, a marked lability of the enzyme upon heating at 40°C and 48°C, and a single narrow peak at pH 9.0. Most of these features were similar to those seen in the Oklahoma I variant. Four of the females in the family were studied (two sisters, the mother, and a maternal aunt of the propositus); all showed a lesser degree of anemia and reticulocytosis but no jaundice, except in the mother. There was a decrease of haptoglobins and both the mother and one sister showed a decrease of erythrocyte survival time as measured by chromium-51. The female members had between 11 and 26% of normal G-6-P D activity in the red cells and between 35 and 63% of normal enzyme activity in the white cells. Starch gel electrophoresis in the mother and aunt showed a single band which migrated at 110% of normal with a trail at the position of the affected males. The presence of a milder degree of hemolysis in the heterozygous carriers of the gene for G-6-P D deficiency associated with congenital nonspherocytic hemolytic anemia provides further support for the Lyon hypothesis in man.

scholarly journals Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia

Blood ◽  
1988 ◽  
Vol 71 (3) ◽  
pp. 748-752 ◽  
Author(s):  
MH Steinberg ◽  
MS West ◽  
D Gallagher ◽  
W Mentzer
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
G6pd Deficiency ◽  
Electrophoretic Pattern ◽  
Hemoglobin Concentration ◽  
Wild Type ◽  
Standardized Protocol ◽  
Cellulose Acetate Membranes ◽  
Male Patients ◽  
Glucose 6 Phosphate Dehydrogenase

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.

Clinical and Biochemical Interactions of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle-Cell Anemia

1972 ◽  
Vol 287 (5) ◽  
pp. 213-217 ◽  
Author(s):  
Sergio Piomelli ◽  
Carl A. Reindorf ◽  
Mohamed T. Arzanian ◽  
Laurence M. Corash
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dehydrogenase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase

Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia

Blood ◽  
2009 ◽  
Vol 114 (3) ◽  
pp. 742-743 ◽  
Author(s):  
David C. Rees ◽  
Christopher Lambert ◽  
Elaine Cooper ◽  
Jack Bartram ◽  
David Goss ◽  
...  
Keyword(s):  
Cerebrovascular Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dehydrogenase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase
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