Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature

Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2...

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Autores principales: Saha, Hinglaj, Tapanjyoti, Ghosh, Biswas, Somak, Mishra, Prafulla Kumar, Basu, Kalyani Saha, Chatterjee, Uttara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322173/
https://www.ncbi.nlm.nih.gov/pubmed/30686894
http://dx.doi.org/10.4103/jiaps.JIAPS_235_17
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author Saha, Hinglaj
Tapanjyoti, Ghosh
Biswas, Somak
Mishra, Prafulla Kumar
Basu, Kalyani Saha
Chatterjee, Uttara
author_facet Saha, Hinglaj
Tapanjyoti, Ghosh
Biswas, Somak
Mishra, Prafulla Kumar
Basu, Kalyani Saha
Chatterjee, Uttara
author_sort Saha, Hinglaj
collection PubMed
description Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.
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spelling pubmed-63221732019-01-25 Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature Saha, Hinglaj Tapanjyoti, Ghosh Biswas, Somak Mishra, Prafulla Kumar Basu, Kalyani Saha Chatterjee, Uttara J Indian Assoc Pediatr Surg Case Report Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC. Medknow Publications & Media Pvt Ltd 2019 /pmc/articles/PMC6322173/ /pubmed/30686894 http://dx.doi.org/10.4103/jiaps.JIAPS_235_17 Text en Copyright: © 2018 Journal of Indian Association of Pediatric Surgeons http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Saha, Hinglaj
Tapanjyoti, Ghosh
Biswas, Somak
Mishra, Prafulla Kumar
Basu, Kalyani Saha
Chatterjee, Uttara
Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title_full Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title_fullStr Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title_full_unstemmed Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title_short Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature
title_sort two cases of progressive familial intrahepatic cholestasis type 2: role of surgery with brief review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322173/
https://www.ncbi.nlm.nih.gov/pubmed/30686894
http://dx.doi.org/10.4103/jiaps.JIAPS_235_17
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