Liver Failure in Neonates With G6PD Deficiency

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.