Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution.

ABSTRACT:

Background

Congenital hepatic fibrosis (CHF) is a rare inherited form of ductal plate malformation associated with polycystic kidney disease. The diagnosis requires histopathologic confirmation, but can be challenging to distinguish from other undefined fibrocystic liver diseases. We aimed to describe the clinicopathologic features of congenital hepatic fibrosis (CHF), with comparisons to other entities that may clinically and/or histologically mimic CHF.

Methods

Nineteen cases that carried a clinical and/or histologic impression of CHF were identified at our institution, of which the histology was reassessed and reappraised into two categories: CHF (n=13) and mimics (n=6). The clinicopathologic features between the two groups were analyzed and compared.

Results

The C

SUBMITTER: Chen IY 

PROVIDER: S-EPMC8406726 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature