Successful long-term phenobarbital therapy of hyperbilirubinemia in congenital hemolytic anemia due to glucose phosphate isomerase deficiency.

Long-term phenobarbital treatment (2 mg/kg body weight/day) permanently reduced hyperbilirubinemia in a patient with congenital non-spherocytic hemolytic anemia due to erythrocyte glucose phosphate isomerase deficiency. Phenobarbital treatment is recommended in patients with congenital hemolytic anemia in whom hyperbilirubinemia is not improved by splenectomy or in whom splenectomy is not indicated. Early phenobarbital treatment may prevent the development of cholelithiasis in patients with chronic hemolysis.