@Article{Janowski2017,
journal="Clinical and Experimental Hepatology",
issn="2392-1099",
volume="3",
number="3",
year="2017",
title="Congenital hepatic fibrosis in a 9-year-old female patient – a case report",
abstract="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.",
author="Janowski, Kamil
and Goliszek, Maria
and Cielecka-Kuszyk, Joanna
and Jankowska, Irena
and Pawłowska, Joanna",
pages="176--179",
doi="10.5114/ceh.2017.70299",
url="http://dx.doi.org/10.5114/ceh.2017.70299"
}