475例血友病患儿登记与管理的回顾性分析

目的 通过对笔者医院血友病中心近年来儿童血友病患者的登记和管理数据进行回顾性分析，以总结经验、提高对血友病登记和管理的认识，为进一步制定符合中国国情的儿童血友病综合管理模式提供依据。方法 收集笔者医院血友病中心自2007年12月1日～2014年10月31日登记的所有儿童血友病病例有关数据并进行统计分析。结果 共收集到475例病例资料，分别来自全国28个省、市、自治区；血友病A414例（87.16%），血友病B61例（12.84%）；重型359例（75.58%），中间型74例（15.58%），轻型42例（8.84%）；仅132例（27.79%）有血友病家族史。临床分型与初次出血年龄有相关性（P<0.01）初次出血年龄越小的，临床分型越重；初次出血年龄与关节受损有相关性（P<0.01）初次出血年龄越小，越易出现关节受损。结论 目前我国血友病登记管理工作尚处于起步阶段，缺少血友病综合管理的研究和经验，登记系统仍不完善，这需要各级卫生部门能够加强对血友病中心的建设力度，提高对患者的追踪管理水平，改善血友病患儿的生活质量。

A Retrospectively Analysis on Registration and Management of 475 Children with Hemophilia

Objective To introduce the registration and management system of children with hemophilia in our hemophilia treatment center, and retrospectively analyzed our data to provide experience and improve the understanding of hemophilia registration and management, in order to provide an integrated management mode which accords with the situation of China for children with hemophilia. Methods We collected the data of children with hemophilia for statistical analysis, who were registered since December 1, 2007 to October 31, 2014. Results The collected data included 475 cases, respectively from 28 provinces, municipalities and autonomous regions; among them Hemophilia A was 414 cases (87.16%), hemophilia B 61 was cases (12.84%); the severe type was 359 cases (75.58%), the intermediate type was 74 cases (15.58%), the light type was 42 cases (8.84%); only 132 cases (27.79%) of them had a family history of hemophilia. Clinical classification was related to the age for the first bleeding (P<0.01). The smaller the age for the first bleeding, the heavier clinical classification the children had. The age for first bleeding was correlated with joint damage (P<0.01). The first time bleeding age was smaller, the joint damaged more easily. Conclusion Hemophilia registration and management work in China is still in its infancy, because the lack of the research and experience of hemophilia integrated management, the registration system is not perfect. It requires the health departments at all levels to strengthen the construction of hemophilia center, to improve the level of tracking management of patients, to improve the life quality of children with hemophilia.