Abstract
Glutathione is present in high concentration in erythrocytes. The main part is kept in the reduce from. It is well known that a decrease of reduce glutation may be linked with hyperhemolysis, but the excat mechanism is unknown. We have investigated three families with nonspherocytic hemolytic anemia in which diminished reduced glutation was a constant finding. The three propositi (two females, one male) had signs of hyperhemolysis from early infancy, one of them even needed two exchange-transfusion. In two of them on one or several occasions stomatocytosis was found, and in one of them high sodium and low potassium in the erythrocytes was observed. Family studies revealed several apparently healthy members with the same deficiency and presenting evidence of mild to moderate hyperhemolysis. Genetic transmission was dominant-autosomal. The phenotypic expression seems to differ greatly. In one case who presented with gallstones at the age of 19 years splenectomy beneficially influenced the intensity of the hemolysis process.
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Lo, S., Httzig, W. & Marti, H. 60. Hemolytic anemia associated with reduced glutathione deficiency. Pediatr Res 5, 95–96 (1971). https://doi.org/10.1203/00006450-197102000-00065
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DOI: https://doi.org/10.1203/00006450-197102000-00065