ArticleClinical and experimental hepatology2017
Congenital hepatic fibrosis in a 9-year-old female patient - a case report.
Article in Clinical and experimental hepatology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 9 citations in OpenAlex.
- Elderly onset congenital hepatic fibrosis with portal hypertension diagnosed after recurrent cholangitis: a case report.Clinical journal of gastroenterology · 2022Article
- Congenital hepatic fibrosis: case report and review of literature.The Pan African medical journal · 2021Review
- Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.Gastroenterology research and practice · 2020Article
- Congenital hepatic fibrosis and coexistent retinal macular degeneration: A case report.Medicine · 2019Article
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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.
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