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Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis
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 fai...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942314/ https://www.ncbi.nlm.nih.gov/pubmed/27437193 http://dx.doi.org/10.5223/pghn.2016.19.2.147 |
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author | 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 |
author_facet | 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 |
author_sort | Choi, Shin Jie |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4942314 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition |
record_format | MEDLINE/PubMed |
spelling | pubmed-49423142016-07-19 Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis 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 Pediatr Gastroenterol Hepatol Nutr Case Report 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. The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition 2016-06 2016-06-28 /pmc/articles/PMC4942314/ /pubmed/27437193 http://dx.doi.org/10.5223/pghn.2016.19.2.147 Text en Copyright © 2016 by The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report 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 Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title | Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title_full | Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title_fullStr | Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title_full_unstemmed | Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title_short | Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis |
title_sort | living related liver transplantation in an infant with neonatal hemochromatosis |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942314/ https://www.ncbi.nlm.nih.gov/pubmed/27437193 http://dx.doi.org/10.5223/pghn.2016.19.2.147 |
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