Document Detail

Glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia: frequency and features of the association in an African community.
MedLine Citation:
PMID:  1174693     Owner:  NLM     Status:  MEDLINE    
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.
U Bienzle; O Sodeinde; C E Effiong; L Luzzatto
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Blood     Volume:  46     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  1975 Oct 
Date Detail:
Created Date:  1975-12-18     Completed Date:  1975-12-18     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  591-7     Citation Subset:  AIM; IM    
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MeSH Terms
Africa / ethnology
Anemia, Sickle Cell / complications*,  epidemiology
Electrophoresis, Starch Gel
Glucosephosphate Dehydrogenase Deficiency / blood,  complications*,  epidemiology,  genetics
Hemoglobin, Sickle
United States
Reg. No./Substance:
0/Hemoglobin, Sickle

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