胆道闭锁肝胆病理改变与预后的关系研究

Correlation between the pathologic changes of liver and fibrous portal tissue and prognosis of biUary atresia

目的通过研究BA患儿术中肝活检和肝门纤维块的病理表现，探讨肝脏和纤维块病理表现与BA预后的关系。方法2009年4月至2010年9月，61例BA患儿在我院行Kasai手术治疗，术中取肝活检和肝门纤维块。，病理切片观察肝小叶结构、纤维化情况，将所有BA患儿根据肝病理诊断是否为胆汁性肝硬化分为肝硬化组和非肝硬化组。根据镜下观察到纤维块中小胆管增生的程度分为少许小胆管增生组和较多小胆管增生组。术后随访3～17个月。结果病理诊断为肝硬化者术时年龄较大、术中门脉测压较高、术后肝功能恢复情况较差。肝硬化组术后3个月黄疽消退率明显较非肝硬化组低（X^2=4．020，P=0．045），术后1年病死率明显较非肝硬化组高（P=0．046）。肝硬化者纤维块内小胆管增生程度较重（X^2=4．244，P=0．039）；纤维块内小胆管增生越严重，术后3个月黄疸消退率越低（P=0．045）。结论肝病理检查是较早的评估BA患儿预后的可靠方法，肝硬化是影响BA预后的重要因素；纤维块小胆管增生程度与BA患儿的短期预后有相关关系，但其是否与BA的远期预后有关系还需要进一步的研究。

Objective To investigate the correlation between the pathologic changes of liver and fibrous portal tissue and prognosis of biliary atresia （BA）. Methods Between April 2009 and September 2010, the Kasai＇s procedures were carried out on 61 patients with BA. Specimens of the liver tissue and fibrous portal tissue were collected in operation. The pathology of the liver tissue and fibrous portal tissue was studied. Based on the severity of hepatic cirrhosis, the patients were grouped into two groups., cirrhosis group and no cirrhosis group. According to whether or not there was obvious bile ductular proliferation in fibrous portal tissue, the hepatic cirrhosis patients were further grouped into 2 groups. All of patients with BA were followed up for 3 to 17 months. Results The patients of hepatic cirrhosis group were older at operation, and had higher portal pressure and poorer prognosis. The 3 months post-operative jaundice clearance rate of cirrhosis patients was significantly lower than that of no cirrhosis patients （39. 5% vs 83.3%, X^2 = 4. 020, P = 0. 045）. The 1-year post-operadve mortality of the cirrhosis patients was significantly higher than that of no cirrhosis patients （53.3%vs 0, P = 0. 046）. The patients of the cirrhosis group had more bile ductular proliferation （60% vs 0, ）X^2 = 4. 244, P = 0. 039）. The more severe bile ductal proliferation in the cirrhosis patients was, the lower jaundice clearance rate they would have （76. 5% vs 38. 5% ,P = 0. 045）. Conclusions The pathologic findings of liver can be used to predict the prognosis of the BA patients after Kasai＇s procedure. The patients with severe hepatic cirrhosis and bile ductal proliferation have Door prognosis.