Role of a liver pathology standardized scoring system in the diagnosis of congenital biliary atresia and its relationship with prognosis
10.3760/cma.j.issn.0529?5807.2019.10.002
- VernacularTitle:肝脏病理标准化评分在先天性胆道闭锁诊断中的作用及其与预后的关系
- Author:
Jiayan FENG
1
;
Lian CHEN
;
Yangyang MA
;
Jing ZHAO
;
Di DING
Author Information
1. 复旦大学附属儿科医院病理科
- Keywords:
Bile duct diseases;
Bile ducts,extrahepatic;
Propensity score;
Diagnostic techniques and procedures;
Prognosis
- From:
Chinese Journal of Pathology
2019;48(10):755-761
- CountryChina
- Language:Chinese
-
Abstract:
Objective To evaluate the diagnostic value of a histologic scoring system in congenital biliary atresia and its prognostic relevance. Methods From January 2017 to June 2018 at Children′s Hospital of Fudan University, 172 wedge liver biopsy specimens were obtained from infants with neonatal cholestasis [119 patients with congenital biliary atresia (CBA) and 53 patients with non?obstructive cholestasis as control]. A pathologist, single?blinded to the final diagnosis, made the histological diagnosis individually based on an 8?feature (portal ductal proliferation, bile duct reaction, bile plugs in portal ductules, liver fibrosis, edema in portal region, cholestasis, inflammatory cells infiltration in portal region, and ductal plate malformation), 21?point scoring system. Results The main pathologic changes of biliary atresia were hepatocyte cholestasis, hyperplasia of bile ducts, fibrosis and infiltration of inflammatory cells in the portal area. There were significant difference in the degree of portal edema, bile duct hyperplasia and fibrosis between two groups (P<0.01). In addition, there were characteristic bile duct thrombosis in 97.5%(116/119) of the cases and abnormal development of bile duct plate in 9.2%(11/119) of the cases. Compared with non?CBA infant cholestasis group, the difference was statistically significant (P<0.05). The scoring system has high sensitivity, specificity(both 94.1%) and accuracy(94.3%) in the diagnosis of CBA. A score equal to or more than 11 points supported a diagnosis of CBA; whereas a score less than 11 points might suggest cholestasis. The degree of hepatic fibrosis and ductal plate malformation were related to prognosis. Conclusions The liver pathology scoring system (8?feature, 21?point) is more accurate in diagnosing CBA than previous methods, which may guide the clinicopathological diagnosis. This histological scoring system also helps to assess the prognosis of CBA.
