| Autor: |
Irene Y. Chen, Christa L. Whitney-Miller, Xiaoyan Liao |
| Jazyk: |
angličtina |
| Rok vydání: |
2021 |
| Předmět: |
|
| Zdroj: |
Diagnostic Pathology, Vol 16, Iss 1, Pp 1-10 (2021) |
| Druh dokumentu: |
article |
| ISSN: |
1746-1596 |
| DOI: |
10.1186/s13000-021-01142-y |
| Popis: |
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 CHF group was further sub-classified into those with clinical suspicion (CHF-c, n=8) and those as incidental histology findings (CHF-i, n=5). Patients of CHF-i were much older than CHF-c or mimics (P |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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