Abstract

Hemolytic disease of the fetus and newborn, which normally presents with unconjugated hyperbilirubinemia, has been known to cause cholestatic jaundice, albeit in rare circumstances; seen especially in the setting of Rhesus (Rh) hemolytic disease or when the mother has received intrauterine transfusion(s). Infants presenting with cholestatic jaundice in the setting of hemolytic anemia pose a clinical dilemma with regard to investigative approach and management. There are no evidence-based guidelines as to whether such infants should be managed conservatively or investigated comprehensively for other causes of cholestasis. Furthermore, whether such babies should receive chelation therapy besides supportive treatment is not known. We describe one such case where a neonate with Rh hemolytic disease presented with cholestasis and acute liver failure.

Key words: Cholestasis, vitamin K, hemorrhagic disease of newborn, hemolytic disease of newborn