肝病理评分在胆道闭锁诊断中的作用  

Usefulness of a histological scoring system for assisting in the diagnosis of biliary atresia

作　　者：薛萍[1] 陈功[1] 郑珊[1] 陈莲[2] 马阳阳[2] 

机构地区：[1]复旦大学附属儿科医院外科,201102 [2]复旦大学附属儿科医院病理科,201102

出　　处：《中华小儿外科杂志》2015年第12期898-903,共6页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨肝病理改变及标准化评分系统在胆道闭锁诊断中的价值。方法选择复旦大学附属儿科医院外科2012年6月至2013年7月期间入院的经手术证实的58例胆道闭锁和28例胆汁淤积症患儿，由2名病理科医师基于8个病理特征（肝纤维化、胆小管增生、汇管区胆栓形成、胆汁淤积、肝细胞病变、汇管区炎症细胞浸润、髓外造血、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肝内门脉区胆管反应及纤维化的形成，还有特征性的胆栓形成以及胆管板发育异常存在，该评分系统诊断胆道闭锁的敏感性、特异性和准确性分别为94．7%、86．2％和91．9％。当分值≥8分时支持诊断胆道闭锁可能性大。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）对于胆道闭锁鉴别诊断具有相对以往方法较高的准确性，可能指导临床病理诊断；该肝组织病理评分系统对患儿预后评估标准具备可行性。Objective To ascertain the usefulness of a histological scoring system for assisting the diagnosis of biliary atresia （BA）. Methods Liver biopsy specimens were collected from infants with neonatal cholestasis （58 patients with BA and 28 patients with non-obstructive cholestasis） from June 2012 to July 2013 were prospectively reviewed. Two pathologists, blinded to the final diagnosis, made the histological diagnosis based on a 8-feature （liver fibrosis, portal ductal proliferation, bile plugs in portal ductules, cholestasis, hepatocellular changes, an infiltration of inflammatory cells in portal region, extramedullary hematopoiesis ~ ductal plate malformation） 21-point （0 to 21） scoring system. Results Ductular reaction （bile ductular proliferation） and liver fibrosis in portal area, bile plugging and ductal plate malformation were all established histological features of BA. With this scoring system, a score of ~ 8 had the best diagnostic utility of differentiating BA histologically from other intrahepatic cholestases （sensitivity 94. 7%, specificity 86. 2%, accuracy 91.9%）. Liver fibrosis and ductal plate malformation were correlated with the prognosis. Conclusions A 8-feature, 21-point histological scoring system has excellent diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis. It is feasible for prognostic assessments.

关 键 词：胆道闭锁 病理过程 预后 

分 类 号：R726.5[医药卫生—儿科]