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MH Steinberg, MS West, D Gallagher and W Mentzer
Jackson VA Medical Center MS 39216.
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.
This article has been cited by other articles:
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| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
