Before the prophylactic use of anti-D antibodies in pregnancy, hemolytic anemia of the newborn was the most common cause of hyperbilirubinemia. Nowadays, given the rarity of hemolytic anemia of the newborn, hepatobiliary abnormalities, perinatal infections, and metabolic disorders have become the most common conditions in the differential diagnosis of neonatal cholestasis. Here, we report 3 instances of cholestatic giant cell hepatitis in 3 infants who had Coombs' positive hemolysis due to ABO incompatibility in 1, Rh incompatibility in another, and combined ABO and Rh incompatibility in the third. Although rare, cholestatic neonatal giant cell hepatitis associated with hemolysis still needs to be considered in patients with neonatal cholestasis. A marked elevation of aspartate aminotransferase over alanine aminotransferase can be a helpful clue to an early diagnosis.

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