NEONATAL HEMOCHROMATOSIS: A RARE CASE REPORT WITH CONSANGUINITY

Abstract

Background: Neonatal hemochromatosis (NH) is a rare and severe liver disease of mainly intra-uterine onset, characterized by neonatal liver failure, hepatic and extrahepatic iron acquisition.NH is also called as  Gestational alloimmune liver disease (GALD).  This leads to an altered iron metabolism with resulting siderosis ,multi-organ failure and infants may be stillborn or present with advance, overwhelming liver disease. The disease represents the most common cause of liver failure in neonates and is also the most common indication for neonatal liver transplantation. We present an neonate who died at 15 days of age,and who was found to have massive iron overload in the liver. Initial treatment consisted of chelation therapy and antioxidants, but lack of laboratory and clinical improvement led to an exchange transfusion followed by  intravenous immunoglobulin (IVIG). Irrespective of all above treatment no improvement of general condition of the patient. The unfavourable course of the disease is described in this case report.