Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.

ABSTRACT:

Background

Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults.

Methods

We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients'

SUBMITTER: Zhu B 

PROVIDER: S-EPMC7191434 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature