glucose-6-phosphate dehydrogenase deficiency

glucose-6-phosphate dehydrogenase deficiency

      hereditary metabolic defect characterized by an increased tendency of the red blood cells to break and release their hemoglobin (hemolysis), especially after the intake of certain drugs. The condition is caused, as the name indicates, by the markedly reduced activity in the red blood cells of a particular organic catalyst, or enzyme, called glucose-6-phosphate dehydrogenase. This low enzyme activity is associated with a decrease in the formation of certain substances that normally help to prevent the oxidative destruction of the red blood cell membrane. Under normal conditions, the affected red blood cells are only slightly more fragile than usual, but more than 40 drugs, including chloramphenicol and sulfonamides, all of which are converted in the body to oxidant compounds, have been shown to produce hemolysis in susceptible persons. There seem to be several variants of the disorder, all of which appear to be sex-linked and fully expressed in males only. The most common form is found chiefly in persons whose ancestors inhabited either Africa or the Eastern Mediterranean basin. A possible protective effect of this metabolic abnormality against malaria has been suggested.

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Universalium. 2010.

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  • glucose-6-phosphate dehydrogenase deficiency — n a hereditary metabolic disorder affecting red blood cells that is controlled by a variable gene on the X chromosome, that is characterized by a deficiency of glucose 6 phosphate dehydrogenase conferring marked susceptibility to hemolytic anemia …   Medical dictionary

  • glucose-6-phosphate dehydrogenase deficiency — glucose 6 phosphate dehydrogenase deficiency. См. дефицит глюкозо 6 фосфатдегидрогеназы. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • Glucose-6-phosphate dehydrogenase deficiency — ] The discovery of G6PD deficiency relieved heavily upon the testing of prisoner volunteers at Illinois State Penitentiary, although today such studies cannot be performed. When some prisoners were given the drug primaquine, some developed… …   Wikipedia

  • glucose-6-phosphate dehydrogenase deficiency — a hereditary (X linked) condition in which the absence of the enzyme glucose 6 phosphate dehydrogenase (G6PD), which functions in carbohydrate metabolism, results in the breakdown of the red blood cells (haemolysis), usually after exposure to… …   Medical dictionary

  • glucose-6-phosphate dehydrogenase deficiency — a hereditary (X linked) condition in which the absence of the enzyme glucose 6 phosphate dehydrogenase (G6PD), which functions in carbohydrate metabolism, results in the breakdown of the red blood cells (haemolysis), usually after exposure to… …   The new mediacal dictionary

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