G6PD Deficiency in the Newborn Treatment & Management

Updated: Feb 04, 2022
  • Author: Lawrence C Wolfe, MD; Chief Editor: George T Griffing, MD  more...
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Treatment

Medical Care

Most individuals with glucose-6-phosphatase dehydrogenase (G6PD) deficiency do not require any treatment. However, infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy, and 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, so that early and focused follow-up can be provided to prevent kernicterus. [10, 11, 12]

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.

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Surgical Care

There is no evidence of selective red cell destruction in the spleen; hence, splenectomy is usually ineffective and not recommended.

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Consultations

Consultations with a hematologist are ideal for long-term follow-up of patients with chronic hemolysis or nonspherocytic anemia.

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Diet

Persons with chronic hemolysis or nonspherocytic anemia should be placed on daily folic acid supplements.

 

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Prevention

Acute hemolytic anemia in G6PD-deficient patients is largely preventable through avoidance of exposure to fava beans and drugs and chemicals that can cause oxidative stress. Identification and discontinuation of the precipitating agent is critical in the management of hemolysis in patients with G6PD deficiency.

Vaccination against hepatitis A and B is recommended in communities with a high prevalence of G6PD deficiency. [40]

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