中国甘肃省血友病中心223例血友病患者临床分析

Clinical Characteristics of 223 Chinese Patients with Hemophilia in A Medical Center of Gansu Province in China

目的:了解中国甘肃省血友病患者的发病及诊疗现状。方法:回顾性分析我中心2010年1月至2015年5月223例血友病患者的临床资料。共收集到来自甘肃14个市及周边临近省份223例病例,其中血友病A(HA)203例,血友病B(HB)20例;在223例中男性222例,女性1例,农村患者177例,占79.4%。结果:首次出血的中位年龄2岁,确诊的平均年龄5.6±6.5岁,HA和HB患者诊断延迟时间分别为2.50±4.91年和2.07±4.76年。在所有患者中合并关节出血168例(75.3%),关节畸形123例(55.2%);91.6%患者按需治疗,乙型肝炎病毒(HBV)和丙型肝病毒(HCV)的感染率分别为1.7%和6.2%。86.9%的患者首次就诊医院为3级以下医院,其中仅15.9%首诊医生考虑患者为血友病。结论:甘肃省血友病患者的确诊率较低,诊断延迟时间较长,合并关节出血多,累计关节畸形比例高,丙肝感染率高。

Objective：To analyze the clinical characteristics,diagonsis and treatment of patients with hemophilia in Gansu province of China.Methods：The clinical data of 223 cases of hemophilia in our center between January 2010 and May 2015 were collected and analyzed retrospectively,these 223 cases of hemophilia were from 14 cities in Gansu and neighboring provinces,including 203 cases of hemophili A（HA） and 20 cases of hemophili B（HB）,among them 222 cases were male,only 1 female（HA）,177 cases were from Rural areas（79.4%）.Results：The median age of first bleeding was 2 years old,and the average age of confirmed as hemophilia was 5.6±6.5 years,the delayed time of diagnoses of HA and HB was 2.50±4.91 and 2.07±4.76 years,respetively,among all the patients 168 caese complicated with joint hemorrhage（75.3%）,123 cases with joint deformities（55.2%）.91.6%of the patients were treated according to demand,the HBV and HCV infection rates were 1.7%and 6.2%respectively.The first-visited hospital of 86.9%patients was hospitalized below 3 grade of level,only 15.9%of these patients were considered to diagnose as hemophili.Conclusion：The accurate level of diagnosis rate for hemophiliacs in Gansu province is low,the delay time of diagnosis is longer,the ratios of complicated joint hemorrhage,total accumulative joint deformity were high,HCV infection rate is also high.