Experience of a single center with congenital hepatic fibrosis:A review of the literature |
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Authors: | Ali Shorbagi Yusuf Bayraktar |
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Affiliation: | Ali Shorbagi, Yusuf Bayraktar, Faculty of Medicine, Department of Internal Medicine, Gastroenterology Clinic, Hacettepe University, 06100 Sihhiye, Ankara, Turkey |
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Abstract: | 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 ran... |
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Keywords: | Congenital hepatic fibrosis Fibropolycystic disorders Portal hypertension Bardet Biedl syndrome |
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