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

作     者：Ali Shorbagi Yusuf Bayraktar 

作者机构：Faculty of MedicineDepartment of Internal MedicineGastroenterology ClinicHacettepe University 

出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)

年 卷 期：2010年第16卷第6期

页      面：683-690页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：Congenital hepatic fibrosis Fibropolycystic disorders Portal hypertension Bardet Biedl syndrome 

摘      要：Congenital hepatic fibrosis(CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile *** 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 *** 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 *** 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 *** 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 *** with Caroli syndrome was the most common diagnosis,with all such patients presenting with symptoms of recurrentcholangitis 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 *** all patients with CHF portal vein morphology should be evaluated by all means since portal vein involvement results in more severe and complicated portal *** associations include the Joubert and Bardet-Biedl syndromes.