胆道闭锁患者肝脏组织病理改变的综合评价

摘要

Objetive The process of disease in biliary atresia (BA)has its unique pathological charac-teristics,but the degree of pathological damage in BA has no consensus.The purpose of this article is to inves-tigate the clinicopathological characteristics of BA and establish a new comprehensive evaluation method which could be reflected the degree of process in BA. Methods Clinical data,routine histology,immunohistochemis-try,and intraoperative frozen pathology sections of 81 patients with biliary atresia(diagonosed and treaded in our hospital from Sep.2010 to Aug.2013)were analyzed.Patient age ranged from 35 to 150 days.The group included 41 males and 40 females. Results Laboatory review revealed elevated serum total bilirubin(TBIL), direct bilirubin(DBIL),alanine aminotransferase(ALT),and gamma-glutamyl transpeptidase (GGT).The morphological changes observed in the periportal small bile ducts include ductal hyperplasia,deformity,and at-rophy with fibrous tissue formation.Degree of liver fibrosis were F1(1 case),F2(11 cases),F3(51 cases),F4 (18 cases).The degree of small bile duct hyperplasia ranged from B1 to B3,with the distribution as follows:B1:21 cases,B2:41 cases,B3:19 cases.Liver inflammation severity level:G1 40 cases (49.40%),G2 41 cases (50.60%),G3 0 case.There was no correlation between extent of inflammation and degree of liver fi-brosis(rs =-0.07,P=0.952)and no correlation between extent of inflammation and degree of small bile duct proliferation(rs =-0.07,P=0.533).There was a varying degree of liver cell and bile canaliculi cholestasis in 100% of cases of BA.72 of 81 (88.90%)cases exhibited small bile ducts in the portal area with visible bile plugs:mild in 45 cases(62.50%),moderate in 26 cases(36.10%),severe in 1 case(1.39%).64 cases n (79.00%)had multinucleated giant liver cells and 11 cases (13.60%)in fatty degeneration of liver cell.The concordance rate of diagnosis of BA by either intraoperative frozen section versus postoperative paraffin section was 97.50%.There was good consistency between intraoperative frozen sections and paraffin sections when comparing degree of small bile duct hyperplasia,liver fibrosis staging,extent of inflammation and bile plugs. Conclusion Intraoperative frozen liver tissue biopsy in the diagnosis of BA and judgment of lesion degree has obvious advantages.To establish a comprehensive pathologic grading system in BA might be useful for judge-ment of BA pathological diagnosis and prognosis.%目的探讨胆道闭锁（Biliary atresia，BA）患者肝脏病理形态学特征，初步建立反映肝脏病理改变程度的综合性评价方法。方法收集2010年9月至2013年8月江西省儿童医院收治的81例胆道闭锁患儿临床资料、常规组织学、免疫组化以及术中冰冻病理检查资料，进行回顾性分析。81例患儿中，男性41例，女性40例，年龄35～150 d。结果患儿血清总胆红素（TBIL）、直接胆红素（DBIL）、谷丙转氨酶（ALT）、谷草转氨酶（AST）均增高。汇管区细小胆管增生、畸形、萎缩，伴纤维组织增生，肝纤维化程度（F1～F4）：1例（1．23％）、11例（13．58％）、51例（62．96％）、18例（22．22％）；小胆管增生程度（B1～B3）：21例（25．93％）、41例（50．62％）、19例（23．46％）；BA 炎症程度分级（G1～G3）：40例（49．40％）、41例（50．60％）、0例（0％）。炎症程度与肝纤维化（rs ＝－0．07，P＝0．952）、与小胆管增生程度（rs ＝－0．07，P＝0．533）无相关性。81例（100％）均出现不同程度肝细胞、毛细胆管淤胆，72例（88．90％）汇管区可见胆栓，其中轻度45例（62．50％），中度26例（36.10％），重度 1例（1．39％）。64例（79．00％）有多核巨肝细胞，11例（13．60％）可见脂肪变性。术中冰冻切片病理诊断与术后石蜡切片病理诊断符合率为97．50％。术中冰冻与石蜡切片在肝纤维化、小胆管增生、炎症程度与胆栓方面一致性较好。结论术中冰冻肝活检对BA的诊断和病变程度的判断具有明显优势，建立BA病变程度的病理综合评价系统，有助于提高病理诊断的准确率和术中、术后对疾病预后的评估。