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Alternative names Return to top
G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiencyDefinition Return to top
G-6-PD deficiency is a hereditary, sex-linked enzyme defect that results in the breakdown of red blood cells when the person is exposed to the stress of infection or certain drugs.Causes, incidence, and risk factors Return to top
G-6-PD deficiency is an inheritable, X-linked recessive disorder whose primary effect is the reduction of the enzyme G-6-PD in red blood cells, causing destruction of the cells, called hemolysis. Ultimately, this hemolysis leads to anemia -- either acute hemolytic or a chronic spherocytic type.
In the United States, many more black than white people have the disorder. Approximately 10-14% of the black male population is affected. The disorder may occasionally affect a black women to a mild degree (depending on their genetic inheritance).
People with the disorder are not normally anemic and display no evidence of the disease until the red blood cells are exposed to an oxidant or stress.
Drugs that can bring on this reaction include:
Symptoms Return to top
Signs and tests Return to top
Treatment Return to top
If the cause is an infection, it should be treated. If the cause is a drug, the offending agent should be stopped. People with the Mediterranean form, or those in hemolytic crisis may occasionally require transfusions.Expectations (prognosis) Return to top
Spontaneous recovery from hemolytic crises is the usual outcome.Complications Return to top
Rarely, kidney failure or death may occur following a severe hemolytic event.Calling your health care provider Return to top
Call for an appointment with your health care provider if symptoms of hemolytic anemia due to G-6-PD deficiency develop.Prevention Return to top
People with G-6-PD must strictly avoid factors that can precipitate an episode, especially drugs known to cause oxidative reactions.
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Page last updated: 28 October 2004 |