Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management
- Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD more...
Most individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency do not need treatment. However, they should be taught to avoid drugs and chemicals that can cause oxidant stress. Patients should also avoid broad beans (ie, fava beans). Favism occurs primarily in the Mediterranean variety of G6PD deficiency.
Identification and discontinuation of the precipitating agent is critical to manage hemolysis in patients with G6PD deficiency. Anemia should be treated with appropriate measures, recognizing that hemolysis is self-limited and often resolves in 8 to 14 days. Transfusions are rarely indicated. Splenectomy is usually ineffective.
Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy with a bili light (see Neonatal Jaundice). Exchange transfusion may be necessary in cases of severe neonatal jaundice or hemolytic anemia caused by favism. However, Samanta and colleagues found that in neonates with idiopathic hyperbilirubinemia, transfusion with G6PD-deficient blood was significantly less effective than transfusion with G6PD-normal blood.
Patients with chronic hemolysis or non-spherocytic anemia should be placed on daily folic acid supplements. Consultations with a hematologist and a geneticist should be sought.
Patients with G6PD deficiency should avoid the following:
Oxidant drugs, such as the antimalarial drugs primaquine, chloroquine, pamaquine, and pentaquine
Nalidixic acid, ciprofloxacin, niridazole, norfloxacin, methylene blue, chloramphenicol, phenazopyridine, and vitamin K analogues
Sulfonamides, such as sulfanilamide, sulfamethoxypyridazine, sulfacetamide, sulfadimidine, sulfapyridine, sulfamerazine, and sulfamethoxazole
Nonsteroidal anti-inflammatory drugs (NSAIDs),nitrofurantoin, and phenazopyridine
Isobutyl nitrite, naphthalene (moth balls), phenylhydrazine, and acetanilide
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