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Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant

INTRODUCTION: Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular...

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Autores principales: Yangın-Ergon, Ezgi, Ermis, Nebahat, Colak, Ruya, Polat, Burak, Alkan-Ozdemir, Senem, Yıldız, Meral, Kulalı, Ferit, Omur-Ecevit, Cigden, Ergun, Orkan, Calkavur, Sebnem
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Jaypee Brothers Medical Publishers 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395480/
https://www.ncbi.nlm.nih.gov/pubmed/30828559
http://dx.doi.org/10.5005/jp-journals-10018-1283
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author Yangın-Ergon, Ezgi
Ermis, Nebahat
Colak, Ruya
Polat, Burak
Alkan-Ozdemir, Senem
Yıldız, Meral
Kulalı, Ferit
Omur-Ecevit, Cigden
Ergun, Orkan
Calkavur, Sebnem
author_facet Yangın-Ergon, Ezgi
Ermis, Nebahat
Colak, Ruya
Polat, Burak
Alkan-Ozdemir, Senem
Yıldız, Meral
Kulalı, Ferit
Omur-Ecevit, Cigden
Ergun, Orkan
Calkavur, Sebnem
author_sort Yangın-Ergon, Ezgi
collection PubMed
description INTRODUCTION: Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. CASE REPORT: The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). CONCLUSION: Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases. How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166.
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spelling pubmed-63954802019-03-01 Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant Yangın-Ergon, Ezgi Ermis, Nebahat Colak, Ruya Polat, Burak Alkan-Ozdemir, Senem Yıldız, Meral Kulalı, Ferit Omur-Ecevit, Cigden Ergun, Orkan Calkavur, Sebnem Euroasian J Hepatogastroenterol Case Report INTRODUCTION: Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. CASE REPORT: The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). CONCLUSION: Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases. How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166. Jaypee Brothers Medical Publishers 2018 2019-02-01 /pmc/articles/PMC6395480/ /pubmed/30828559 http://dx.doi.org/10.5005/jp-journals-10018-1283 Text en Copyright © 2018; Jaypee Brothers Medical Publishers (P) Ltd. This work is licensed under a Creative Commons Attribution 3.0 Unported License. To view a copy of this license, visit http://creativecommons.org/licenses/by/3.0/
spellingShingle Case Report
Yangın-Ergon, Ezgi
Ermis, Nebahat
Colak, Ruya
Polat, Burak
Alkan-Ozdemir, Senem
Yıldız, Meral
Kulalı, Ferit
Omur-Ecevit, Cigden
Ergun, Orkan
Calkavur, Sebnem
Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title_full Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title_fullStr Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title_full_unstemmed Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title_short Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant
title_sort abernethy malformation type 2 and biliary atresia coexistence: a rare cause of infantile liver transplant
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395480/
https://www.ncbi.nlm.nih.gov/pubmed/30828559
http://dx.doi.org/10.5005/jp-journals-10018-1283
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