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KMID : 1038820160190020147
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Pediatric Gastroenterology, Hepatology & Nutrition
2016 Volume.19 No. 2 p.147 ~ p.151
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Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis
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Choi Shin-Jie
Choi Jong-Sub
Chun Peter
Yoo Jung-Kyung
Moon Jin-Soo
Ko Jae-Sung
Kim Woo-Sun
Kang Gyeong-Hoon
Yi Nam-Joon
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Abstract
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Neonatal hemochromatosis (NH) is a severe neonatal liver injury that is confirmed by extra-hepatic iron accumulation. Although a recent study described treating NH with exchange transfusions and intravenous immunoglobulin, liver transplantation should be considered for patients with severe liver failure that does not respond to other medical treatment. Herein, we report the case of a two-month-old female infant who presented with persistent ascites and hyperbilirubinemia. Her laboratory findings demonstrated severe coagulopathy, high indirect and direct bilirubin levels, and high ferritin levels. Abdominal magnetic resonance imaging presented low signal intensity in the liver on T2-weighted images, suggesting iron deposition. The infant was diagnosed with NH as a result of the clinical findings and after congenital infection and metabolic diseases were excluded. The infant was successfully treated with a living-donor liver transplantation. Living related liver transplantation should be considered as a treatment option for NH in infants.
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KEYWORD
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Neonatal hemochromatosis, Gestational alloimmune disease, Liver failure, Liver transplantation
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