Neonatal Hemochromatosis

Abstract

Neonatal hemochromatosis (NH) is a rare disorder characterized by signs of hepatic insufficiency. The rapidly progressive course terminates in death in-utero or in the early neonatal period. Histopathologically, there is increased tissue iron deposition in many organs, particularly the liver, pancreas, heart and endocrine glands, but the extrahepatic reticuloendothelial system is relatively unaffected. Cases of NH are distributed in a pattern consistent with an error of metabolism inherited as an autosomal recessive trait. We present a premature infant who died 44 days postpartum of severe hepatic failure. Postmortem examination established the diagnosis of NH.