大连市血友病患者诊疗及生存状态分析

Analysis on the diagnosis,treatment and survival state of patients with hemophilia in the city of Dalian

目的：了解大连市血友病患者的一般情况、临床特点、输血相关疾病及生活状态等,探讨改善血友病患者生存状态方法。方法：对102例血友病患者的临床资料进行回顾性分析。结果：102例血友病患者中血友病A 83例（81.37%）,血友病B 19例（18.63%）;51例（50.00%）有明确家族史,46例（45.10%）无家族史,5例（4.90%）家族史不详。患者首次出血中位年龄3岁,确诊的中位年龄4岁,二者之间差异有统计学意义（P〈0.01）。发生关节出血81例（79.41%）,关节畸形52例（50.98%）。农村患者28例（27.45%）,城镇患者74例（72.55%）。从未治疗者2.94%,仅在严重出血时进行治疗者68.63%,按需治疗者20.59%,预防治疗者7.84%。输血相关性病毒感染指标HBV、HCV、HIV和梅毒螺旋体的感染率分别为9.30%、11.63%、0和1.16%,总感染率为22.09%。18岁以上文化程度初中以下者19例（18.63%）,18-60岁非学生无工作者30例（29.41%）,花费每年大于1万元者37例（36.27%）。结论：血友病患者存在就诊率低、诊疗延迟、治疗严重不足、致残率高、寿命低、儿童辍学率高、成人文化程度低的特点。应加强对专业医护人员及患者家属的宣教及培训,提高就诊率,从而改善血友病患者的生存现状。

Objective：To understand some features of patients with hemophilia in Dalian,including the general condition,clinical features,blood transfusion related diseases and life status,in order to reveal the methods to improve survival state of patients with hemophilia.Method：Clinical data of 102 hemophilic patients were retrospective analyzed.Result：Among 102 patients,83cases were hemophilia A（81.37%）and 19 cases were hemophilia B（18.63%）.Meanwhile,51cases（50.00%）had hemophilic family history,46cases（45.10%）without hemophilic family history,and 5patients＇ family history was unknown（4.90%）.The median age of initial hemorrhage was 3years old,but the median age of the first diagnosed was 4years old,there was significant difference between them（P〈0.01）.There were 28 rural patients（27.45%）and 74 urban patients（72.55%）.Simultaneously,81cases（79.41%）had arthrorrhagia,52cases（50.98%）had joint deformity.In the 102 patients,2.94% patients never get any treatment,68.63% patients got treatment only in severe bleeding condition,20.59% patients received limited treatment,and 7.84% patients received prevention treatment.The total virus infection rate of HBV,HCV,spiral HIV and MP related to blood transfusion was 9.30%,11.63%,0and 1.16% respectively.Nineteen patients beyond 18 years old with diploma did not reach to junior high school（18.63%）,30non-student patients age from18 to 60years old without any job（29.41%）,37patients（36.27%）spend more than 10 000 yuan per year.Conclusion：Hemophilia patients are characterized with low consultation rate,low diagnosis rate,delayed diagnosis,serious treatment deficiencies,high morbidity,short lifespan,high dropout rate of children and low degree of adult culture.We should strengthen the professional education and medical training for medical staff and patients＇ family members to improve the rate of diagnosis and treatment,thus to improve the survival situation of hemophiliacs.