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What is G6BD deficiency?

Updated: 11/5/2022
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Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection. G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency

G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase, which helps red blood cells work properly. Too little G6PD leads to the destruction of red blood cells. This process is called hemolysis. When this process is actively occurring, it is called a hemolytic episode. The episodes are usually brief, because the body continues to produce new red blood cells, which have normal activity. Red blood cell destruction can be triggered by infections, severe stress, certain foods (such as fava beans), and certain drugs, including: * Antimalarial drugs * Aspirin * Nitrofurantoin * Nonsteroidal anti-inflammatory drugs (NSAIDs) * Quinidine * Quinine * Sulfonamides Other chemicals, such as those in mothballs, can also trigger an episode. Persons with this condition do not display any signs of the disease until their red blood cells are exposed to certain chemicals in food or medicine, or to stress. Symptoms are more common in men and may include: * Dark urine * Enlarged spleen * Fatigue * Paleness * Rapid heart rate * Shortness of breath * Yellow skin color (jaundice) Treatment may involve: * Medicines to treat an infection, if present * Stopping any drugs that are causing red blood cell destruction * Transfusions, in some cases

Possible Complications Rarely, kidney failure or death may occur following a severe hemolytic event.

Outlook (Prognosis) Spontaneous recovery from hemolytic crises is the usual outcome.

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