摘要目的 探讨肝脏病理标准化评分系统在先天性胆道闭锁诊断中的作用及其与预后的关系.方法 选择复旦大学附属儿科医院2017年1月至2018年6月期间入院的经手术证实的119例先天性胆道闭锁和53例胆汁淤积症患者,由2名病理科医师基于8个病理特征(汇管区胆小管增生、胆管反应、汇管区胆小管内胆栓形成、肝纤维化、汇管区水肿、胆汁淤积、汇管区炎性细胞浸润、胆管板发育异常),总分为21分的半定量肝病理评分系统进行病理诊断及预后分析.结果 胆道闭锁的肝组织病理改变主要是不同程度的肝细胞胆汁淤积、汇管区水肿、胆小管增生、纤维化及炎性细胞浸润,其中汇管区水肿、胆小管增生及纤维化程度与非先天性胆道闭锁的婴儿胆汁淤积症组相比差异有统计学意义(P<0.01).另外,97.5%(116/119)病例存在特征性的胆小管内胆栓形成,9.2%(11/119)病例存在胆管板发育异常改变,与非先天性胆道闭锁的婴儿胆汁淤积症组比较差异有统计学意义(P<0.05).该评分系统诊断先天性胆道闭锁的灵敏度及特异度分别为94.1%、94.3%,总体诊断的准确性94.2%.当分值≥11分时支持诊断先天性胆道闭锁可能大,当分值<11分时支持诊断胆汁淤积症可能大.同时,肝纤维化程度、胆管板发育异常与预后相关.结论 肝病理评分系统(8个特征21分制)对于胆道闭锁的鉴别诊断具有较高的准确性,可能指导临床病理诊断.该肝组织病理评分系统对患者预后评估标准具有可行性.

abstractsObjective 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.