儿童血友病A抑制物产生相关因素横断面分析  

A cross-sectional survey of coagulation factor Ⅷ inhibitor in children with hemophilia A

作　　者：魏琪琪[1] 李刚[1] 唐凌[1] 陈振萍[1] 甄英姿[1] 吴心怡[1] 张宁宁[2] 张纪水[3] 于国霞[4] 吴润晖[1] 

机构地区：[1]首都医科大学附属北京儿童医院血液病肿瘤中心,100045 [2]首都医科大学附属北京儿童医院影像中心,100045 [3]首都医科大学附属北京儿童医院心理科,100045 [4]首都医科大学附属北京儿童医院口腔科,100045

出　　处：《中华儿科杂志》2014年第2期99-102,共4页Chinese Journal of Pediatrics

基　　金：国家自然科学基金（81341018）;北京市卫生系统高层次卫生技术人才培养计划

摘　　要：目的 了解儿童血友病A抑制物发生情况,探讨抑制物发生的相关因素,为制定防控策略提供依据.方法 收集2012年11月至2013年5月就诊于北京儿童医院血友病门j诊的血友病A患儿的临床资料,并进行抑制物筛查,分析抑制物发生的相关因素.结果 共纳入血友病A患儿102例,其中轻型5例,中间型32例,重型65例;筛查时的中位年龄为55.5（3.0 ～200.0）个月;抑制物阳性19例（18.6％）：低滴度9例（8.8％）,高滴度10例（9.8％）.因首次出血即为危及生命的大出血而进行凝血因子Ⅷ替代治疗是抑制物形成的相关因素（P=0.03）,OR值为4.10（95％CI：1.17 ～14.32）;20个暴露日以内为产生抑制物的高风险期（P=0.04）,OR值为3.32（95％ CI：1.02～10.86）.短期内接受高强度因子暴露是导致高滴度抑制物发生的相关因素（P=0.01）,OR值为5.25（95％ CI：1.45 ～21.92）.结论 本组患儿血友病A抑制物总体发生率及高、低滴度抑制物发生率与国外报道相似,危重症抢救所接受的高强度因子治疗是抑制物以及高滴度抑制物产生的相关因素.Objective To study the current situation of coagulation factor Ⅷ （F Ⅷ） inhibitor development in children with hemophilia A （HA） through a cross-sectional survey,and to explore the risk factors of inhibitor development in order to provide evidence for further prevention and management strategies.Method The clinical data of outpatients with hemophilia A in Beijing Children＇s Hospital seen from November 2012 to May 2013 were collected,FⅧ inhibitor was screened and analyzed its risk factors.Result A total of 102 HA children were enrolled,5 were mild cases,32 were moderate,and 65 were severe cases ; the median age on enrollment was 55.5 （3.0-200.0） months：19 （18.6%） of patients had inhibitors and 9 （8.8%） had low-titer inhibitors,10 （9.8%） had high-titer inhibitors.Receiving FⅧ treatment for life-threatening bleeding（P =0.03）,OR 4.10 （95% Cl：1.17-14.32） was a risk factor for inhibitor generation and patients within 20 exposure days have more chances of inhibitor development （P =0.04）,OR 3.32（95%CI：1.02-10.86）.High and intense FⅧ exposure within short term was the risk factor for high titer inhibitor development （P =0.01）,OR 5.25 （95% Cl：1.45-21.92）.Conclusion Intense FⅧ exposure for severe hemorrhage was the risk factor of inhibitors development especially of high titer inhibitors.

关 键 词：儿童 血友病A 抑制物 

分 类 号：R725.5[医药卫生—儿科]