Gestational alloimmune liver disease and neonatal hemochromatosis

Neonatal hemochromatosis(NH) is a clinical syndrome of liver disease and pathologic siderosis of various extrahepatic tissues.NH is a form of secondary hemochromatosis in which severe fetal liver injury causes iron overload due to poor regulation of maternofetal iron flux.Gestational alloimmune liver disease (GALD) has been established as the cause of fetal liver injury resulting in nearly all cases of NH.GALD may produce subacute and chronic fetal liver injury (congenital cirrhosis) typical of NH.It may also produce acute injury and acute liver failure of the fetus and newborn.Demonstration of extrahepatic-siderosis, which can be get by oral mucosa tissue biopsy or by magnetic resonance imaging of pancreas clinically, is necessary to make the diagnosis of NH.Diagnosis of GALD depends on immunohistochemistry for C5B-9 of a liver biopsy.Therapeutic strategy of NH, which based on its etiology of immune injury, is combination of exchange transfusion and administration of high-dose intravenous immunoglobulin.Prognosis of severe NH is poor and liver transplant often is necessary in babies of severe NH.Recurrence of sever NH can be prevented by treatment during gestation. Key words: Gestational alloimmune liver disease; Neonatal hemochromatosis; Neonatal acute liver failure