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

Brief Reports1 January 1979Improved Erythrocyte Survival with High-Dose Vitamin E in Chronic Hemolyzing G6PD and Glutathione Synthetase DeficienciesS. P. SPIELBERG, M.D., Ph.D., L. A. BOXER, M.D., L. M. CORASH, M.D., J. D. SCHULMAN, M.D.S. P. SPIELBERG, M.D., Ph.D., L. A. BOXER, M.D., L. M. CORASH, M.D., J. D. SCHULMAN, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-90-1-53 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptGlutathione (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...References1. TAPPEL A: Selenium, glutathione peroxidase, and vitamin E. Am J Clin Nutr 27:960-965, 1974 CrossrefMedlineGoogle Scholar2. BEUTLER E: Disorders due to enzyme defects in the red blood cell. Adv Metab Disord 6:131-160, 1972 CrossrefGoogle Scholar3. BANNAITSUKEDAOKUMURA SHH: Effects of antioxidants on cultured human diploid fibroblasts exposed to cystine-free medium. Biochem Biophys Res Comm 74:1582-1588, 1977 CrossrefMedlineGoogle Scholar4. HAFEMANHOEKSTRA DW: Protection against carbon tetrachloride-induced lipid peroxidation in the rat by dietary vitamin E, selenium, and methionine as measured by ethane evolution. J Nutr 107:656-665, 1975 CrossrefGoogle Scholar5. SPIELBERGKRAMERGOODMANBUTLERTIETZEQUINNSCHULMAN SLSJFPJ: 5-oxoprolinuria: biochemical observations and case report. J Pediatr 91:237-241, 1977 CrossrefMedlineGoogle Scholar6. FARRELLBIERIFRATANTONIWOODDI SANT' AGNESE PJJRP: The occurrence of effects of human vitamin E deficiency. A study in patients with cystic fibrosis. J Clin Invest 60:233-241, 1977 CrossrefMedlineGoogle Scholar7. : Recommended methods for radioisotope red-cell survival studies. Br J Haematol 21:241-250, 1971 CrossrefMedlineGoogle Scholar8. BIERIPRIVAL JE: Serum vitamin E determined by thin-layer chromatography. Proc Soc Exp Biol Med 120:554-557, 1965 CrossrefMedlineGoogle Scholar9. ALLENJOHNSONCADMANKAPLAN DGSM: Membrane polypeptide aggregates in glucose-6-phosphate dehydrogenase deficient and in vitro aged red blood cells. J Lab Clin Med 91:321-327, 1978 MedlineGoogle Scholar10. TUDHOPEHOPKINS GJ: Lipid peroxidation in human erythrocytes in tocopherol deficiency. Acta Haematol 53:98-104, 1975 CrossrefMedlineGoogle Scholar11. MENZELSLAUGHTERBRYANTJAUREGUI DRAH: Prevention of ozonide-induced Heinz bodies in human erythrocytes by vitamin E. Arch Environ Health 30:234-236, 1975 CrossrefMedlineGoogle Scholar12. DIPALMARITCHIE JD: Vitamin toxicity. Ann Rev Pharmacol Toxicol 17:133-148, 1977 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. 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SCHULMAN, M.D., S. HARVEY MUDD, M.D., JERRY A. SCHNEIDER, M.D., STEPHEN P. SPIELBERG, M.D., Ph.D., LAURENCE BOXER, M.D., JANET OLIVER, Ph.D., LAURENCE CORASH, M.D., MICHAEL SHEETZ, Ph.D.Therapeutic Uses of Vitamin E in MedicineTREATMENT OF GLUTATHIONE PEROXIDASE DEFICIENCY WITH VITAMIN EProtection of Granulocytes by Vitamin E in Glutathione Synthetase DeficiencyTreatment of glutathione synthetase deficient fibroblasts by inhibiting γ-glutamyl transpeptidase activity with serine and borate 1 January 1979Volume 90, Issue 1Page: 53-54KeywordsCellsGlutathioneHemolytic anemiaInborn errors of metabolismLigasesMedical servicesOxidative damageOxidative stressTissue culturesVitamin E Issue Published: 1 January 1979 PDF downloadLoading ...