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Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings

Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We experienced 3 cases of congenital hepatic fibrosi...

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Autores principales: Kim, Myung Hwan, Ryu, Jin Sook, Yang, Suk Kyun, Lee, Sung Koo, Kim, Hae Ryun, Joung, Young Hwa, Lee, Young Sang, Min, Young
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Association of Internal Medicine 1990
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4535005/
https://www.ncbi.nlm.nih.gov/pubmed/2098093
http://dx.doi.org/10.3904/kjim.1990.5.2.101
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author Kim, Myung Hwan
Ryu, Jin Sook
Yang, Suk Kyun
Lee, Sung Koo
Kim, Hae Ryun
Joung, Young Hwa
Lee, Young Sang
Min, Young
author_facet Kim, Myung Hwan
Ryu, Jin Sook
Yang, Suk Kyun
Lee, Sung Koo
Kim, Hae Ryun
Joung, Young Hwa
Lee, Young Sang
Min, Young
author_sort Kim, Myung Hwan
collection PubMed
description Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We experienced 3 cases of congenital hepatic fibrosis with Caroli’s disease in 3 siblings, whose clinical manifestations were diverse, such as repeated cholangitis, variceal hemorrhage, or intrahepatic stones. All of them had multiple renal cysts, so we supposed that the clinical entities of these patients were in the spectrum of fibropolycystic disease of the liver and kidney.
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spelling pubmed-45350052015-10-02 Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings Kim, Myung Hwan Ryu, Jin Sook Yang, Suk Kyun Lee, Sung Koo Kim, Hae Ryun Joung, Young Hwa Lee, Young Sang Min, Young Korean J Intern Med Case Report Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We experienced 3 cases of congenital hepatic fibrosis with Caroli’s disease in 3 siblings, whose clinical manifestations were diverse, such as repeated cholangitis, variceal hemorrhage, or intrahepatic stones. All of them had multiple renal cysts, so we supposed that the clinical entities of these patients were in the spectrum of fibropolycystic disease of the liver and kidney. Korean Association of Internal Medicine 1990-07 /pmc/articles/PMC4535005/ /pubmed/2098093 http://dx.doi.org/10.3904/kjim.1990.5.2.101 Text en Copyright © 1990 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kim, Myung Hwan
Ryu, Jin Sook
Yang, Suk Kyun
Lee, Sung Koo
Kim, Hae Ryun
Joung, Young Hwa
Lee, Young Sang
Min, Young
Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title_full Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title_fullStr Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title_full_unstemmed Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title_short Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings
title_sort three cases of congenital hepatic fibrosis with caroli’s disease in three siblings
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4535005/
https://www.ncbi.nlm.nih.gov/pubmed/2098093
http://dx.doi.org/10.3904/kjim.1990.5.2.101
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