Gestational alloimmune liver disease and neonatal hemochromatosis / 国际儿科学杂志

ABSTRACT

Neonatal hemochromatosis(NH) is a clinical syndrome of liver disease and pathologic sider osis 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 matemofetal 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 faihure of the ferus 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.