Glucose-6-phosphate dehydrogenase deficiency medical therapy

{Glucose-6-phosphate dehydrogenase deficiency}} Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.D. [2]

Please help WikiDoc by adding content here. It's easy! Click here to learn about editing.

Overview

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive hereditary disease featuring abnormally low levels of the G6PD enzyme, which plays an important role in red blood cell function. Individuals with the disease may exhibit non-immune hemolytic anemia in response to a number of causes. It is closely linked to favism, a disorder characterized by a hemolytic reaction to consumption of broad beans, with a name derived from the Italian name of the broad bean (fava). Sometimes the name, favism, is alternatively used to refer to the enzyme deficiency as a whole.

Treatment

• In the acute phase of hemolysis, blood transfusions might be necessary. Blood transfusion is an important symptomatic measure, as the transfused red cells are generally not G6PD deficient.
• Dialysis in acute renal failure
• Some patients benefit from removal of the spleen (splenectomy), as this is an important site of red blood cell destruction. * Folic acid should be used in any disorder featuring a high red blood cell turnover.
• Although vitamin E and selenium have antioxidant properties, their use does not decrease the severity of G6PD deficiency.

Contraindicated medications

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is considered an absolute contraindication to the use of the following medications:

• Pegloticase

References

Template:WikiDoc Sources Need content