Improved Erythrocyte Survival with High-Dose Vitamin E in Chronic Hemolyzing G6PD and Glutathione Synthetase Deficiencies

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Abstract

Glutathione (GSH) and vitamin E both play vital roles in the protection of cells from oxidative stress (1). Decreased reduced erythrocyte GSH resulting from inborn errors of metabolism is associated with hemolytic anemia (2). Hemolysis may be caused by endogenous or drug-induced oxidative damage to erythrocytes. Pharmacologic levels of vitamin E partially protect cells experimentally rendered GSH deficient in tissue culture and in vivo (3, 4). Therefore, we have examined the effects of chronic administration of high doses of vitamin E on erythrocyte survival in two patients with inherited disorders of GSH metabolism. Both patients have chronic hemolytic anemia, one

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