eISSN: 2449-8238
ISSN: 2392-1099
Clinical and Experimental Hepatology
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SCImago Journal & Country Rank
3/2017
vol. 3
 
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abstract:
Case report

Congenital hepatic fibrosis in a 9-year-old female patient – a case report

Kamil Janowski
,
Maria Goliszek
,
Joanna Cielecka-Kuszyk
,
Irena Jankowska
,
Joanna Pawłowska

Clin Exp HEPATOL 2017; 3, 3: 176-179
Online publish date: 2017/09/25
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Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive disorder, clinically characterized by hepatic fibrosis and portal hypertension. CHF results from ductal plate malformation (DPM) of the intrahepatic bile ducts. Four clinical forms can be observed: portal hypertensive, cholangitic, mixed and latent. CHF is one of the “fibropolycystic diseases” which also include several conditions with a variety of intrahepatic bile duct dilatation and associated periportal fibrosis such as Caroli disease, autosomal recessive and dominant polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune, Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes. Most of them are accompanied by progressive cystic degeneration of the kidneys. We present the case of a 9-year-old female patient with CHF with nonspecific clinical manifestation and a review of the literature.
keywords:

congenital hepatic fibrosis, portal hypertension, ductal plate malformation, esophageal varices

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