Successful treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin in lower northern Thailand: Case report and review literature
Keywords:
Alloimmune, hemochromatosis, siderosis, liver failure, immunoglobulinAbstract
Background: Neonatal hemochromatosisis a rare disorder and clinically defined assevere neonatal hepatic disease in associated with extrahepatic siderosis. NH - GALD has a significant mortality and morbidity risk. Case report: A 14-day old male neonate who is a second child in a Thai family and born to mother without a previous history of NH-GALD. He developed severe liver failure since
birth.
Result: Diagnosis of NH-GALD is positive iron staining (Prussian blue) of minor salivary glands in lower lip biopsy and by magnetic resonance imaging (MRI). Additionally, serum ferritin level as well as alpha-fetoprotein is high and liver biopsy proves severe hepatocyte injury with iron overload. The patient successfully treated with combination of exchange transfusion, intravenous immunoglobulin, and chelation-antioxidant therapy.
Conclusion: The diagnosis of NH-GALD requires diagnosing extrahepatic siderosis by tissue analysis or MRI. Early treatment improves the outcome and decrease rate the liver transplantation
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