title: The potential use of xylitol in glucose-6-phosphate dehydrogenase deficiency anemia.
abstract: NADP-linked xylitol dehydrogenase has been found to be present in human red blood cells. This enzyme activity is normal in most glucose-6-phosphate dehydrogenase (G6PD)-deficient red cells. Xylitol was explored as a potential agent for treatment of hemolysis in patients with G6PD-deficiency. Intracellular GSH (glutathione, reduced) was first converted to its oxidized form by incubation of the erythrocytes with acetylphenylhydrazine or by pretreatment with methyl phenyldiazenecarboxylate. The addition of 0.15 M xylitol was shown to be more effective than 0.15 M glucose in maintaining the levels of GSH in G6PD-deficient red cells during such oxidative challenge. Rabbit erythrocytes contain less activity of G6PD and glutathione reductase compared with the normal human adult values, but have an active xylitol dehydrogenase. The rabbit erythrocyte is sensitive to acetylphenylhydrazine and primaquine phosphate. In both in vivo and in vitro experiments, xylitol was found to partially prevent acetylphenylhydrazine induced acute hemolysis of the rabbit red cell and GSH content was found to be preserved. The intravenous injection of xylitol (0.5 g/kg body weight per 6 hr) for 6 days, seemed to be nontoxic to the animal. The results suggest that xylitol should be further investigated as an agent for the treatment of G6PD-deficient patients during acute hemolytic episodes.
mesh_terms: D000429:Alcohol Oxidoreductases / Q000378:metabolism; D000438:Alcohols / Q000627:therapeutic use*; D000818:Animals; D004912:Erythrocytes / Q000187:drug effects / Q000201:enzymology; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000188:drug therapy* / Q000201:enzymology; D005980:Glutathione Reductase / Q000378:metabolism; D006461:Hemolysis / Q000187:drug effects; D006801:Humans; D008297:Male; D009249:NADP / Q000378:metabolism; D010659:Phenylhydrazines / Q000494:pharmacology; D011319:Primaquine / Q000494:pharmacology; D011817:Rabbits; D014993:Xylitol / Q000627:therapeutic use
chemical_list: D000438:Alcohols; D010659:Phenylhydrazines; D009249:NADP; D000429:Alcohol Oxidoreductases; D005980:Glutathione Reductase; D011319:Primaquine; D014993:Xylitol