eISSN: 2449-8238
ISSN: 2392-1099
Clinical and Experimental Hepatology
Current issue Archive Manuscripts accepted About the journal Editorial board Subscription Contact Instructions for authors Ethical standards and procedures
Editorial System
Submit your Manuscript
SCImago Journal & Country Rank
Search
Editorial Policies
Sarajevo Declaration on Integrity and Visibility of Scholarly Publications
3/2017
vol. 3
 
Share:
Send email
Share:
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
DOI: https://doi.org/10.5114/ceh.2017.70299
Online publish date: 2017/09/25
View full text Get citation
 
PlumX metrics:
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
Termedia
About us Contact
Copyright notice Privacy policy Advertising policy Contact us
Quick links
Journals Books eBooks Events
© 2024 Termedia Sp. z o.o.
Developed by Bentus.