山东省血友病患者的病例信息分析

摘要

Objective To summarize the general condition,regional distribution,prevalence and clinical characteristics of Shandong province based on hemophilia case registry information.Methods A retrospective study was carried out on 1 979 hemophilia patients registered at Shandong Hemophilia Registration Center.Results The 1 979 cases have included 1 704 hemophilia A and 275 hemophilia B patients.Hemophilia A was characterized as severe in 1021 patients (59.9%),moderate in 483 patients (28.4%),and mild in 200 patients (1 1.7%);while hemophilia B was characterized as severe in 125 patients (45.4%),moderate in 1 1 6 patients (42.2%),and mild in 34 patients (12.4%).The median age was 23 years (ranging from 1 month to 81 years),and most were young patients.Joint deformity occurred in 963 patients,and 948 patients had a family history of hemophilia.All counties of Shandong province had patients except for Changdao county and Fushan district of Yantai city.The prevalence of Heze city and Dongying city (3.39/100 000 and 3.05/100 000,respectively)were relatively higher.Conclusion The above data revealed epidemiological and clinical characteristics of Shandong Province. Patient-centered registry system allowed a more detailed and accurate patient information,and promoted the comprehensive care of hemophilia,which also suggested the necessity for the establishment and improvement of the National Hemophilia Registry System.%目的：根据山东省血友病患者的登记信息,总结血友病患者的一般情况、地区分布、患病率及临床特征,为血友病防治工作提供依据。方法对2010年1月至2015年4月在山东省血友病诊疗中心病例信息管理中心登记注册的1979例血友病患者的相关资料进行回顾分析。结果共收集甲型血友病1704例,其中重型1021例(59.9%),中型483例(28.4%),轻型200例(11.7%)；乙型血友病275例,其中重型125例(45.4%),中型116例(42.2%),轻型34例(12.4%)。中位年龄23岁,最小年龄为1个月,最大年龄为81岁,其中以青少年患者居多。患者中963例有关节残疾,948例有家族史。山东省17地市中除烟台市的长岛县和福山区外,其他县区均有患者登记,其中菏泽市和东营市血友病患病率较高,分别为3.39/10万和3.05/10万。结论上述数据较为全面地反映了山东省血友病的流行病学及临床特点,以患者为中心的登记系统可以提供更加详细准确的患者信息,促进血友病综合关怀工作的开展,同时也提示了建立和完善全国范围内血友病登记系统的必要性。