Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

Mithat Gunaydin,1 Asudan Tugce Bozkurter Cil2 1Avicenna Hospital, Department of Pediatric Surgery, Istanbul, Turkey; 2Medicana International Samsun Hospital, Department of Pediatric Surgery, Samsun, Turkey Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessi...

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Autores principales:	Gunaydin M, Bozkurter Cil AT
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2018
Materias:	Intrahepatic cholestasis jaundice biliary diversion. Diseases of the digestive system. Gastroenterology RC799-869
Acceso en línea:	https://doaj.org/article/c69ef86c202d497c9b6ded273c126690
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spelling	oai:doaj.org-article:c69ef86c202d497c9b6ded273c1266902021-12-02T00:12:33ZProgressive familial intrahepatic cholestasis: diagnosis, management, and treatment1179-1535https://doaj.org/article/c69ef86c202d497c9b6ded273c1266902018-09-01T00:00:00Zhttps://www.dovepress.com/progressive-familial-intrahepatic-cholestasis-diagnosis-management-and-peer-reviewed-article-HMERhttps://doaj.org/toc/1179-1535Mithat Gunaydin,1 Asudan Tugce Bozkurter Cil2 1Avicenna Hospital, Department of Pediatric Surgery, Istanbul, Turkey; 2Medicana International Samsun Hospital, Department of Pediatric Surgery, Samsun, Turkey Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options. Keywords: intrahepatic cholestasis, jaundice, biliary diversionGunaydin MBozkurter Cil ATDove Medical PressarticleIntrahepatic cholestasisjaundicebiliary diversion.Diseases of the digestive system. GastroenterologyRC799-869ENHepatic Medicine: Evidence and Research, Vol Volume 10, Pp 95-104 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Intrahepatic cholestasis jaundice biliary diversion. Diseases of the digestive system. Gastroenterology RC799-869
spellingShingle	Intrahepatic cholestasis jaundice biliary diversion. Diseases of the digestive system. Gastroenterology RC799-869 Gunaydin M Bozkurter Cil AT Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
description	Mithat Gunaydin,1 Asudan Tugce Bozkurter Cil2 1Avicenna Hospital, Department of Pediatric Surgery, Istanbul, Turkey; 2Medicana International Samsun Hospital, Department of Pediatric Surgery, Samsun, Turkey Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options. Keywords: intrahepatic cholestasis, jaundice, biliary diversion
format	article
author	Gunaydin M Bozkurter Cil AT
author_facet	Gunaydin M Bozkurter Cil AT
author_sort	Gunaydin M
title	Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
title_short	Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
title_full	Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
title_fullStr	Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
title_full_unstemmed	Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
title_sort	progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
publisher	Dove Medical Press
publishDate	2018
url	https://doaj.org/article/c69ef86c202d497c9b6ded273c126690
work_keys_str_mv	AT gunaydinm progressivefamilialintrahepaticcholestasisdiagnosismanagementandtreatment AT bozkurtercilat progressivefamilialintrahepaticcholestasisdiagnosismanagementandtreatment
_version_	1718403889724653568

Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

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