Clinical and pathological features of patients with congenital hepatic fibrosis： An analysis of 26 cases
- VernacularTitle:26例先天性肝纤维化患者的临床及病理特点分析
- Author:
Shixuan LIU
1
;
Yujiao ZHANG
2
;
Ying ZHENG
3
;
Aiping SONG
1
;
Tailing WANG
1
;
Xiuhong WANG
1
Author Information
- Publication Type:Journal Article
- Keywords: Congenital Hepatic Fibrosis; Pathological Conditions， Signs and Symptoms; Portal Hypertension
- From: Journal of Clinical Hepatology 2025;41(11):2317-2322
- CountryChina
- Language:Chinese
- Abstract: ObjectivesTo summarize the clinical and pathological features of patients with congenital liver fibrosis （CHF）， and to investigate the differences in clinical and pathological features between patients in different age groups. MethodsA retrospective analysis was performed for the clinicopathological data of 26 patients with pathologically confirmed CHF in China-Japan Friendship Hospital from August 2005 to June 2023， and the patients were stratified by age to investigate the clinical and pathological features of patients in different age groups. ResultsAmong the 26 patients， there were 12 male patients and 14 female patients， with an age of onset of 4 — 61 years. There were 19 patients with portal hypertension type （73.08%）， 2 patients with cholangitis type （7.69%）， 4 patients with mixed type （15.38%）， and 1 patient with occult type （3.85%）. Of all 26 patients， 4 had unknown clinical symptoms， and among the 22 patients with clear clinical symptoms， 10 （45.45%） attended the hospital due to upper gastrointestinal bleeding caused by portal hypertension. Pathological manifestations included roughly normal liver parenchyma separated by fibrous septa in the portal area， with the presence of abnormal reactive bile duct hyperplasia. Denser fibrous septa and a lack of portal veins with the corresponding caliber were observed in 4 pediatric patients with disease onset before the age of 10 years， with a significant reduction or even disappearance of compensatory thin-walled blood vessels. ConclusionPortal hypertension-type CHF is the most common type in clinical practice. Patients with an early age of onset have certain histopathological features of the liver， with the presence of serious complications associated with portal hypertension. Therefore， liver biopsy should be performed for patients suspected of CHF in clinical practice， and genetic testing should be performed when necessary. Early identification and diagnosis are of great importance for improving the prognosis of patients.
