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Published on: June 5, 2014
Liver Failure in Neonates With G6PD Deficiency
Milaan Shah1, Vani Gopalareddy2
1University of South Carolina School of Medicine, Columbia, SC.
Insights
Glucose-6-phosphate dehydrogenase (G6PD) deficiency can cause liver issues in newborns. This enzyme defect may lead to progressive liver failure, even requiring transplantation in severe cases.
Area of Science:
- Biochemistry
- Pediatrics
- Hepatology
Background:
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common inherited red blood cell disorder.
- While associated with hemolysis and jaundice, its role in severe liver dysfunction, particularly liver failure, is not well-established.
Observation:
- Two neonates with G6PD deficiency presented with progressive liver failure.
- The liver failure was unresponsive to ursodiol treatment.
Findings:
- G6PD deficiency was linked to severe, treatment-resistant liver failure in these neonates.
- Both infants ultimately required liver transplantation due to the severity of their condition.
Implications:
- Highlights the critical need for vigilant liver function monitoring in jaundiced neonates with G6PD deficiency.
- Suggests that G6PD deficiency can precipitate or exacerbate severe liver disease, potentially leading to liver failure.
Abstract:
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a commonly inherited enzyme defect that can present with hemolysis, hyperbilirubinemia, and jaundice and may cause kidney and liver dysfunction. G6PD deficiency may serve as a cofactor for chronic liver disease; however, an association with liver failure is not well described. We present the cases of 2 neonates with G6PD deficiency and progressive liver failure resistant to treatment with ursodiol that eventually required liver transplantation. Our cases underscore the importance of monitoring liver function in jaundiced neonates with underlying G6PD deficiency and demonstrate the potential precipitation of liver disease by G6PD deficiency.
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