胆道闭锁多中心综合诊断治疗方案研究

A multicenter study on the diagnosis and treatment of biliary atresia in children

目的探讨胆道闭锁综合诊疗方案应用的有效性和安全性。方法根据具有地域代表性的四家儿童医院专家确定的胆道闭锁诊疗方案，2007年1月开始对胆道闭锁进行诊断及治疗。收集2004年1月至2006年12月四家医院经确诊为胆道闭锁并进行根治手术的病例作为对照组；2007年1月至2009年12月病例作为观察组。使用标准化量表统计首次就诊年龄、手术年龄、术前及术后3个月，6个月和1年黄疸清除率，术后胆管炎发生率，2年自体肝生存率。结果符合诊断标准498例，实施Kasai术456例，其中对照组184例，手术168例；观察组314例，手术288例。二组首次就诊年龄无明显差异E（33±27）d比（34±26）d，P〉0．05]，使用标准化诊断方案后平均手术年龄由（64±24）d下降为（74±31）d，P〈0．05；观察组术后3个月、6个月及1年退黄率（53．9％、56．9％、58．0％）较对照组（38．9％、39．9％、43．4％）明显提高（P〈0．05）；其术后胆管炎发生率亦显著降低（31．8％比47．8％，P〈0．05）；对照组2年自体肝生存率39．3％，观察组56．1％（P〈0．05）。结论标准化胆道闭锁综合诊断治疗方案可降低手术年龄、提高术后黄疸清除率、减少术后反流性胆管炎，显著提高术后2年自体肝生存率。

Objective To evaluate the efficacy and safety of a standardized protocol for the diagnosis and treatment of biliary atresia （BA） in children. Methods This study enrolled 498 patients from 4 hospitals diagnosed with BA from January 2004 to December 2009. The BA patients of the standard- ized protocol group, who enrolled in this study after January 2007, were diagnosed and treated using the standardized protocol. The rest of the patients were taken as controls. The patients were followed up for 2 years. The age of the first diagnosis, the age at operation, jaundice clearance, the occurrence of cholangitis and two year survival rates with native liver were retrospectively reviewed and compared between the 2 groups. Results Among the 498 patients, 456 patients underwent Kasai procedure. Age of the first diagnosis between the two groups showed no difference （33 ± 27 vs 34 ± 26 days, P〉 0. 05）, but the patients of the standardized protocol group received surgeries at earlier ages （64 ± 24 vs 74 ± 31 days, P〈0. 05）. The jaundice clearance rates of 3, 6 and 12 months after operation in the patients of the standardize protocol group were higher than those of controls （53. 9%, 56. 9 %, 58.0% vs 38.9%, 39. 9%, 43.4%, P〈0. 05）. Patients of the standard protocol group had lower occurrence of cholangitis （31.8% vs 47. 8%, P〈0. 05）, and better two years survival rate （56. 1 % vs 39. 3%, P〈 0. 05）. Conclusions The standardized protocol for the diagnoses and treatment of BA improves the clinical outcomes of the children with BA.