a Record control number WOS:000274459200004

a ISSN 1007-9327

a Original cataloging agency NEU GrandLibrary

a Language code of text/sound track or separate title eng

a Classification number WI 140

b Item number .E97 2010

a Personal name Shorbagi, Ali.

h Medium Electronic Document

a Title Experience of a single center with congenital hepatic fibrosis: A review of the literature

c Statement of responsibility, etc Ali Shorbagi, Yusuf Bayraktar.

b Name of publisher, distributor, etc W. J. G. Press,

c Date of publication, distribution, etc 2010.

a Summary, etc Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. It is one of the fibropolycystic diseases, which also include Caroli disease, autosomal dominant polycystic kidney disease, and autosomal recessive polycystic kidney disease. Clinically it is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. CHF is known to occur in association with a range of both inherited and non-inherited disorders, with multiorgan involvement, as a result of ductal plate malformation. Because of the similarities in the clinical picture, it is necessary to differentiate CHF from idiopathic portal hypertension and early liver cirrhosis, for which a liver biopsy is essential. Radiological tests are important for recognizing involvement of other organ systems. With regards to our experience at Hacettepe University, a total of 26 patients have been diagnosed and followed-up between 1974 and 2009 with a diagnosis of CHF. Presentation with Carob syndrome was the most common diagnosis, with all such patients presenting with symptoms of recurrent cholangitis and symptoms related to portal hypertension. Although portal fibrosis is known to contribute to the ensuing portal hypertension, it is our belief that portal vein cavernous transformation also plays an important role in its pathogenesis. In all patients with CHF portal vein morphology should be evaluated by all means since portal vein involvement results in more severe and complicated portal hypertension. Other associations include the Joubert and Bardet-Biedl syndromes. (C) 2010 Baishideng. All rights reserved.

a Topical term or geographic name as entry element Gastroenterology & Hepatology

a Topical term or geographic name as entry element Near East University Article

a Topical term or geographic name as entry element Yakın Doğu Üniversitesi Makale

g Relationship information FEB 14 2010, Vol.16 Issue 6, p683-690

t Title World journal of gastroenterology

x International Standard Serial Number 10079327

u Uniform Resource Identifier http://library.neu.edu.tr:2048/login?url=http://dx.doi.org/10.3748/wjg.v16.i6.683