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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제19권 제2호
- 발행연도
- 2016.1
- 수록면
- 147 - 151 (5page)
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초록· 키워드
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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 lev-els, 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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등록된 정보가 없습니다.
참고문헌 (20)
참고문헌 신청
Whitington PF / 2006 / Fetal and infantile hemochromatosis / Hepatology 43:654~660
Lopriore E / 2013 / Neonatal hemochromatosis: management, outcome, and prevention / Prenat Diagn 33:1221~1225
Whitington PF / 2012 / Gestational alloimmune liver disease and neonatal hemochromatosis / Semin Liver Dis 32:325~332
Pan X / 2010 / Novel mechanism of fetal hepatocyte injury in congenital alloimmune hepatitis involves the terminal complement cascade / Hepatology 51:2061~2068
Whitington PF / 2008 / Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin / Pediatrics 121:e1615~e1621
Lopriore E / 2013 / Neonatal hemochromatosis: management, outcome, and prevention / Prenat Diagn 33:1221~1225
Whitington PF / 2012 / Gestational alloimmune liver disease and neonatal hemochromatosis / Semin Liver Dis 32:325~332
Pan X / 2010 / Novel mechanism of fetal hepatocyte injury in congenital alloimmune hepatitis involves the terminal complement cascade / Hepatology 51:2061~2068
Whitington PF / 2008 / Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin / Pediatrics 121:e1615~e1621
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