Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017 Sep;3(3):176-179.
doi: 10.5114/ceh.2017.70299. Epub 2017 Sep 25.

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

Kamil Janowski  1 , Maria Goliszek  1 , Joanna Cielecka-Kuszyk  1 , Irena Jankowska  1 , Joanna Pawłowska  1
Affiliations
Case Reports

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

Kamil Janowski et al. Clin Exp Hepatol. 2017 Sep.

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.

Keywords: congenital hepatic fibrosis; ductal plate malformation; esophageal varices; portal hypertension.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Periportal fibrosis and sclerosis, proliferation of bile ducts lined by cuboidal epithelial cells. Two central veins remain intact, without signs of central fibrosis. H&E, 200x
See this image and copyright information in PMC

References

    1. Dobbs RH. Congenital hepatic fibrosis with portal hypertension. Proc R Soc Med. 1960;53:327–328. - PubMed
    1. Kerr DN, Harrison CV, Sherloc S, et al. Congental hepatic fibrosis. Q J Med. 1961;30:91–117. - PubMed
    1. Kerr DN, Okonkwo S, Choa RG. Congenital hepatic fibrosis: the long-term prognosis. Gut. 1978;19:514–520. - PMC - PubMed
    1. Murray-Lyon IM, Ockenden BG, Williams R. Congenital hepatic fibrosis: is it a single clinical entity? Gastroenterology. 1973;64:653–656. - PubMed
    1. Rawat D, Kelly DA, Milford DV, et al. Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis. J Pediatr Gastroenterol Nutr. 2013;57:161–166. - PubMed

Publication types

LinkOut - more resources