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Glucose-6-phosphate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase deficiency

a deficiency of glucose-6-phosphate dehydrogenase, an enzyme important for maintaining cellular concentrations of reduced nucleotides.

deficiency of this enzyme is the commonest disease-causing enzyme defect in humans affecting an estimated 400 million people.

The gene for this enzyme is on the x chromosome and there are various polymorphic forms.

males with the enzyme deficiency develop haemolytic anaemia when red blood cells are exposed to oxidant drugs such as the antimalarial primaquine, the sulfonamide antibiotics or sulfones, naphthalene moth balls, or fava beans. It can also cause anaemia of the newborn, and chronic nonspherocytic haemolytic anaemia.

Inheritance: x-linked.


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