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Mortality in a Cohort of Men Expressing the Glucose-6-Phosphate
Dehydrogenase Deficiency
Pierluigi Cocco,
Pierfelice Todde,
Susanna Fornera,
Maria Bonaria
Manca,
Pierina Manca, and
Ana Rosa Sias
From the Istituto di Medicina del Lavoro, Università di
Cagliari, Cagliari, Italy; the Servizio di Anatomia Patologica,
Ospedale S. Michele, ASL 8, Cagliari, Italy; and the Servizio di Igiene
Pubblica, ASL n. 6, S. Gavino Monreale, Italy.
The objective of this study was to test the hypothesis
of a lower mortality from cancer and cardiovascular diseases among men
expressing glucose-6-phosphate dehydrogenase (G6PD) deficiency. We
designed a mortality study based on death certificates from January 1, 1982 through December 31, 1992 in a cohort of G6PD-deficient men.
Cohort members were 1,756 men, identified as expressing the G6PD-deficient phenotype during a 1981 population screening of the G6PD
polymorphism. The setting was the island of Sardinia, Italy. Outcome
measures were cause-specific standardized mortality ratios (SMRs),
which were computed as 100 times the observed/expected ratio, with the
general Sardinian male population as the reference. Deaths from all
causes were significantly less than expected due to decreased SMRs for
ischemic heart disease (SMR, 28; 95% confidence interval [CI], 10 to
62), cerebrovascular disease (SMR, 22; 95% CI, 6 to 55), and liver
cirrhosis (SMR, 12; 95% CI, 0 to 66), which explained 95.6% of the
deficit in total mortality. All cancer mortality was close to the
expectation, with a significant increase in the SMR for non-Hodgkin's
lymphoma (SMR, 545; 95% CI, 147 to 1,395). A decrease in mortality
from cardiovascular diseases was one of the study hypotheses, based on
an earlier human report and experimental evidence. However, selection
bias is also a likely explanation. Further analytic studies are
warranted to confirm whether subjects expressing the G6PD-deficient
phenotype are protected against ischemic heart disease and
cerebrovascular disease. This cohort study is consistent with more
recent case-control studies in rejecting the hypothesis of a decreased
cancer risk among G6PD-deficient subjects. The observed increase in
mortality from non-Hodgkin's lymphoma and decrease in mortality from
liver cirrhosis were not previously reported.
Blood, Vol. 91 No. 2 (January 15), 1998:
pp. 706-709
© 1998 by The American Society of Hematology.

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