河南省186例儿童血友病门诊患者临床治疗与现状分析

Analysis of clinical treatment and current condition of 186 Children outpatients with nemophilia in He'nan

目的通过对河南省红十字血液中心门诊部就诊的186例儿童血友病患者的临床治疗情况和现状分析,为进一步加强儿童血友病综合防治工作提供临床依据。方法回顾分析河南省红十字血液门诊部2012年7月～2013年12月186例儿童血友病患者的门诊治疗病历资料。结果一般情况：186例儿童血友病患者来自河南省18个市、地区,年龄5.5～18岁,平均12.6岁,其中城镇儿童患者24例（12.9%）,农村儿童患者162例（87.1%）。均为男性,血友病A患者162例,血友病B患者19例,血友病C患者5例。血友病甲患者首次出血年龄（1.2±2.1）岁,初次确诊年龄（3.2±1.3）岁。临床情况：（1）农村血友病儿童患者所占比例较高,因为经济,交通等问题普遍存在治疗不及时,治疗量不足的情况。（2）儿童患者以关节出血居多,其次为肌肉出血、黏膜、皮下出血;血尿、消化道出血、脑出血情况较少见。（3）关节、肌肉出血后,运动能力明显受障,致残率高。（4）治疗及时疗效较好。结论血友病儿童患者一旦出现症状就要及时治疗,越早治疗,治疗效果越好。本研究建议采取家庭医院结合治疗的办法。

Objective To analyze the clinical treatment and current condition of 186 children hemophilic outpatients from outpatient department of Henan Red Cross Blood Center with an aim to provide the basic data for further studies and comprehensive care in Henan. Methods The imaging data of 186 children patients with hemophilia were retrospectively analyzed,who were treated in outpatient department of He＇nan Red Cross Blood Centre from July 2012 to December 2013. Results General information：the 186 children patients came from 18 cities or regions in Henan,whose average age was 12.6 year, and median age was 11.4year（range from 5.5-18 years old）.24 children came from city or town（12.9%）,and 162 children came from village with 87.1%.All the children patients were male.162 cases were hemophilia A（87.1%）,19 cases were hemophilia B（10.2%）,and 5 cases were hemophilia C（2.7%）.The first bleed age of hemophilia A was（1.2±2.1）years old.The age of the confirmed disease was（3.2±1.3）years old.Clinical information：（1）Majority patients came from village,because of the economic,traffic and other common problems,such as not timely treatment and the treatment of shortage;（2）Common symptom of children patients was the joint bleeding,followed by muscle bleeding,mucous bleeding,and subcutaneous hemorrhage.Hematuresis,alimentary tract hemorrhage,and cerebral hemorrhage were relatively rare conditions.（3）After bleeding,there was a high prevalence of severe disability,because exercise capacity was significantly affected by the barrier.（4）The curative effect was better by timely treatment. Conclusion Once symptoms of infection appear,patients with hemophilia children should be timely treatment.The earlier the treatment,the curative effect is better.This paper suggests combining family and hospital cure.