G6PD Deficiency Treatment & Management
- Author: Lawrence C Wolfe, MD; Chief Editor: George T Griffing, MD more...
Most individuals with G6PD deficiency do not require any treatment.
The acute hemolytic anemia in G6PD-deficient patients is largely preventable by avoiding exposure to fava beans, drugs, and chemicals that can cause oxidant stress. Identification and discontinuation of the precipitating agent is critical in management of hemolysis in patients with G6PD deficiency.
Anemia secondary to mild-to-moderate hemolysis in G6PD deficient patients is usually self-limited and often resolves in 8-14 days. Transfusion is rarely needed in cases of severe anemia.
Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy. Exchange transfusion may be necessary in cases of severe neonatal jaundice or hemolytic anemia caused by favism.
Systematic assessment for the risk of severe hyperbilirubinemia should be performed before discharge in neonates in whom G6PD deficiency is suspected to provide early and focused follow-up to prevent bilirubin encephalopathy.[11, 13, 5]
Persons with chronic hemolysis or nonspherocytic anemia should be placed on daily folic acid supplements. Consultations with a hematologist are ideal for long-term follow up.
Vaccination against hepatitis A and B is recommended in communities with high prevalence of G6PD deficiency.
Transcriptional upregulation of G6PD enzyme in response to HDACi (histone deacetylase inhibitors) in in-vitro experiments on human B cells and erythroid precursor cells has been reported by Makarona K et al, which opens new areas of potential treatment in future.
There is no evidence of selective red cell destruction in the spleen; hence splenectomy is usually ineffective and not recommended.
Consultations with a hematologist are ideal for long-term follow up of those with chronic hemolysis or nonspherocytic anemia.
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