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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(r_s=-0.07,P=0.952)、与小胆管增生程度(r_s=-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。
Objective To investigate the pathological features of liver in patients with biliary atresia (BA) and to establish a comprehensive evaluation method to reflect the extent of liver pathological changes. Methods The clinical data, routine histology, immunohistochemistry and intraoperative frozen pathological examination of 81 cases of biliary atresia admitted to Children’s Hospital of Jiangxi Province from September 2010 to August 2013 were retrospectively analyzed. Of the 81 children, 41 were males and 40 were females, ranging in age from 35 to 150 days. Results Serum total bilirubin (TBIL), direct bilirubin (DBIL), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) in children were increased. There were 1 case (1.23%), 11 cases (13.58%), 51 cases (62.96%), 18 cases (22.22%), abnormal fibrosis and fibrosis (B1 ~ B3): 21 cases (25.93%), 41 cases (50.62%) and 19 cases (23.46%); The degree of inflammation was 40 cases , 41 cases (50.60%) and 0 cases (0%). The degree of inflammation had no correlation with hepatic fibrosis (r_s = -0.07, P = 0.952) and the degree of small bile duct hyperplasia (r_s = -0.07, P = 0.533). In 81 cases (100%), hepatocytes and cholesthesia were found in 75 cases, of which 72 cases (88.90%) had gallbladder embolism, of which 45 cases were mild (62.50%), 26 1 case (1.39%). 64 cases (79.00%) had multinucleated giant hepatocytes, and 11 cases (13.60%) showed steatosis. Intraoperative frozen section pathological diagnosis and pathological diagnosis of paraffin section in line with the rate of 97.50%. Intraoperative freezing and paraffin sections in liver fibrosis, small bile duct hyperplasia, the degree of inflammation and gallbladder consistency is better. Conclusion Intraoperative frozen liver biopsy has obvious advantages in the diagnosis of BA and the degree of lesion. Establishing a pathological comprehensive evaluation system of BA lesion can help to improve the accuracy of pathological diagnosis and assess the prognosis of postoperative and postoperative diseases.