空肠间置胆囊结肠吻合术治疗PFIC和Alagille综合征13例分析  

Short-term outcomes and prognostic factors of cholecystojejunocolonic anastomosis for PFIC and Alagille syndrome

作　　者：沈桢[1] 郑珊[1] 董岿然[1] 陈功[1] 王晓红[2] 俞蕙[2] 王建设[2] 

机构地区：[1]复旦大学附属儿科医院普外科,上海201102 [2]复旦大学附属儿科医院肝病科,上海201102

出　　处：《中华小儿外科杂志》2013年第9期658-661,共4页Chinese Journal of Pediatric Surgery

摘　　要：目的观察空肠间置胆囊结肠吻合术治疗1、2型进行性家族性肝内胆汁淤积症（PFIC）和Alagille综合征的短期疗效，并研究潜在的预后相关因素。方法11例PFIC和2例Alagille综合征患儿接受空肠间置胆囊结肠吻合术，随访时间2～26个月。术前和术后1周、1个月、6个月和1年定期查肝功能，观察患儿术后血清总胆汁酸、总胆红素、瘙痒分级以及HeightZ值的变化。结果术后1个月，5例患儿总胆汁酸（TBA）下降至术前50％以下，此5例患儿（A组）术后1个月、6个月和1年的TBA分别为术前的（27．0±6．4）%、（24．4±10．2）％和（40．4±17．2）％，与术前TBA浓度比较，差异有统计学意义（P〈0．05）；术后1个月、6个月和1年的总胆红素（TB）分别为（51．6±31．9）％，（37．8±36．5）％和（49．6±21．5）％；术后6个月瘙痒分级较术前下降（2．5±1．3）级，术后1年时50％（2／4）出现瘙痒分级的反跳，75％（3／4）患儿出现追赶性生长。其余8例（B组）术后6个月TBA、TB均未显著下降，瘙痒分级无改善。单因素分析显示两组患儿年龄、性别、术前TB、TBA和术前瘙痒分级差异无统计学意义，但B组患儿术前均存在持续性黄疸，与A组差异有统计学意义（P=0．01）。结论空肠间置胆囊结肠吻合术可缓解部分Alagille综合征和PFIC患儿的瘙痒和黄疸。术前持续性黄疸以及术后1个月胆汁酸下降〈50％是预后不良的危险因素。胆汁内转流术的应用价值仍有待探讨。Objective To observe the short-term outcomes of cholecystojejunocolonic anastomosis for patients with progressive familiar intrahepatic cholestasis （PFIC） or Alagille syndrome and to determine the possible prognostic factors. Methods Cholecystojejunocolonie anastomosis was performed on 11 patients with PFIC and 2 patients with Alagille syndrome from August 2010 to August 2012. Total bile acids （TBA） and total bilirubin （TB） were measured preoperatively, 1 week, 1, 6 and 12 months postoperatively. Pre- and postoperative TBA, TB, pruritus scale and height Z value were compared. Results TBA decreased by ）50% 1 month after operation in 5 patients （group A）.In group A, TBA decreased to （27.0± 6.4）%, （24.4± 10.2）% and （40.4± 17.2）% and TB decreased to （51.6 ± 31.9）%, （37. 8 ± 36. 5）% and （49. 6± 21.5）% of the preoperative concentrations at 1, 6 and 12 months after operation. Pruritus scale decreased by （2. 4± 1.3） and （1.5 ± 1.2） points at 6 and 12 months after operation. Height Z increased in 3 patients. In the rest 8 patients （group B）, TBA, TB, pruritus scale and height Z did not improve after operation. Univariate analysis showed age, sex, pre-operative TB and TBA had no differences in group A and B, but only 1 patient in group A had persistent preoperative jaundice which was significantly different from group B. Conelusions PIBD can relieve pruritus and jaundice for some patients with PIFC/AGS. Persistent preoperative jaundice and a decrease of 〈50% 1 month after operation are unfavorable prognostic factors.

关 键 词：胆汁淤积 肝内 ALAGILLE综合征 吻合术 外科 预后 

分 类 号：R726.5[医药卫生—儿科]