单中心儿童血友病A抑制物累积发生率及危险因素  

Status and risk factors of inhibitor occurrence in single - center children with hemophilia

作　　者：魏琪琪[1] 唐凌[2] 陈振萍[1] 李刚[1] 甄英姿[1] 吴心怡[1] 张宁宁[3] 王岩[4] 张纪水[4] 于国霞[5] 张颖[1] 张维娜[1] 吴润晖[1] 成小玲[6] 

机构地区：[1]首都医科大学附属北京儿童医院血液肿瘤中心,100045 [2]首都医科大学附属北京儿童医院,100045 [3]首都医科大学附属北京儿童医院影像中心,100045 [4]首都医科大学附属北京儿童医院神经康复中心,100045 [5]首都医科大学附属北京儿童医院口腔科,100045 [6]首都医科大学附属北京儿童医院药剂科,100045

出　　处：《中华实用儿科临床杂志》2017年第3期212-215,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的了解北京儿童医院单中心血友病A患儿抑制物累积发生率及其相关危险因素，为制定有中国特色的儿童血友病A抑制物防治策略提供依据。 方法顺序收集2012年11月至2013年12月在北京儿童医院血友病专业门诊就诊≤20暴露日（exposure day，ED）的血友病A患儿资料，并自2012年11月起至2015年4月止对上述患儿进行连续性抑制物检测，了解其抑制物累积发生率及相关危险因素。 结果共收集到48例患儿，入组时中位月龄为30（14～71）个月、中位ED为7（1～20） d；轻型3例（6.3%），中间型17例（35.4%），重型28例（58.3%）。抑制物累积发生率：经过中位24（15～29）个月、20（5～150）ED的随访和172次抑制物连续检测，抑制物累积发生率为27.1%（13/48例），其中低滴度占30.8%（4/13例），高滴度69.2%（9/13例）。抑制物发生的危险因素包括首次暴露原因为抢救危及生命出血[OR＝6.67，95%CI（1.31～33.94），P〈0.05]和20 ED之内[OR＝5.19，95%CI（1.02～26.94），P〈0.05]；而高滴度抑制物形成的相关危险因素仅为20 ED之内[OR＝8.53，95%CI（1.46～50.05），P〈0.05]。 结论该单中心儿童血友病A抑制物累积发生率达27.1%、高滴度占2/3，危重症抢救首次接触因子及20 ED之内是抑制物及高滴度抑制物产生的危险因素。Objective To investigate the cumulative incidence and risk factors of inhibitor occurrence in Chinese children with hemophilia in Beijing Children＇s Hospital（BCH） , and provide the basis for developing control strate- gies for Chinese children with hemophilia inhibitor. Methods To know the cumulative incidence of inhibitor, outcome and associated risk factors of hemophilia by collecting patients＇ data from November 2012 to December 2013 at BCH hemophilia professional clinic, ~〈20 exposure days （ED）, continuity detecting inhibitor at fixed point time as much as possible from November 2012 to April 2015. Results Forty - eight cases were enrolled, and the median duration was 30（ 14 -71 ） months and the median exposure time was 7（ 1 -20） days;these patients consisted of 3 （6.3%） mild ca- ses, 17 （35.4%） moderate cases and 28 （58.3%） sever cases. The cumulative incidence of inhibitors was 27.1% （13/48 cases） with 172 times of detecting in which the median time of follow -up was 24（ 15 -29） months and 20 （5- 150）ED. The occurrence of low titer inhibitor was 30.8% （4/13 cases）, and for high titer inhibitors it was 69.2% （9/13 cases）. To rescue life- threatening bleeding was the reason for the first time exposure [ OR = 6.67, 95%CI （1.31 -33.94） ,P 〈0.05] and ED within the 20 ED[OR =5.19,95% CI （1.02 -26.94） ,P 〈0.05] were the related risk factors of inhibitors,but exposure days within the 20 ED [ OR = 8.53,95% CI （ 1.46 - 50.05 ） ,P 〈 0.05 ] was associated with the formation of high titer inhibitors. Conclusions The inhibitor cumulative incidence of hemophilia A was 27.1% ,in which high titer inhibitors accounted for 2/3. To rescue life - threatening bleeding as the reason for the first time exposure and ED within the 20 ED are the related risk factors of inhibitors.

关 键 词：儿童 血友病A 抑制物 

分 类 号：R725.5[医药卫生—儿科]