Congenital hepatic fibrosis with novel mutations in PKD1 gene masquerading as early cryptogenic cirrhosis: a rare case report

Congenital hepatic fibrosis with novel mutations in PKD1 gene masquerading as early cryptogenic cirrhosis: a rare case report

Abstract Background Congenital hepatic fibrosis (CHF) is a rare disorder of the porto-biliary system occurring due to the defective remodeling of ductal architecture leading to progressive fibrosis of the portal tract. Though classically, CHF has been reported to be associated with autosomal recessi...

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Bibliographic Details
Main Authors: Souradeep Dutta, Ankit Jain, Reddy Abhinaya, Bheemanathi Hanuman Srinivas, Vishnu Prasad Nelamangala Ramakrishnaiah
Format: Article
Language:English
Published: SpringerOpen 2021-07-01
Series:Egyptian Liver Journal
Subjects:
Congenital hepatic fibrosis
Portal hypertension
Polycystic kidney disease
Case report
Online Access:https://doi.org/10.1186/s43066-021-00132-5

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