血友病患者诊疗及健康状况调查分析

Investigation on treatment and health condition of children with hemophilia

目的了解中国4～18岁血友病患者的诊疗及健康现状,为开展血友病综合关怀工作做好准备。方法对中国血友病协作组登记在册的215例血友病患者及其父母进行患者一般情况、健康状况及自理能力调查。结果血友病患者首次出血年龄为(1.4±1.4)岁,疾病诊断年龄为(2.8±2.6)岁(与出血年龄比较,P<0.01);首次关节出血年龄(3.2±2.5)岁,开始替代治疗年龄(3.7±3.7)岁(与关节出血年龄比较,P<0.05);出血27.2次/年、病假68.9d/年,凝血因子用量(252.0±250.7)U/(kg.年),相关并发症发生率8.8%～61.4%,自理能力得分23.2±3.8,医疗费用(13336.4±11840.6)元/年;119例患者进行了感染相关性病毒的检测,其中2.5%感染乙型肝炎病毒(HBV)、10.1%感染丙型肝炎病毒(HCV)、3.4%感染艾滋病病毒(HIV),总感染率为16.0%。农村患者替代治疗年龄、凝血因子用量、医疗费用、肌肉及骨关节并发症发生率及自理能力得分与城市患者比较,差异有统计学意义(P<0.05,P<0.01)。结论血友病患者诊疗延迟,凝血因子用量不足,并发症发病率高,自理能力差,尤其是农村患者。应加强对血友病专业医护人员培训,在此基础上建立初级综合关怀团队,提供综合关怀,以提高血友病患者早诊断、早治疗及其有效率。

Objective To investigate treatment and health condition of hemophilia children in China, and to prepare for conducting comprehensive caring. Methods A total of 215 hemophilia children aged 4 to 18 years were surveyed in terms of their demographics, health condition and self-care ability. Results The mean age of initial hemorrhage and diagnosis was （1.4 ±1.4）, （2.8 ± 2.6） re- spectively, and a significant difference was found between them（P〈0.01）. The mean age of initial joint hemorrhage was （3.2± 2.5）, which was significantly earlier than that of initial treatment （3.7±3.7,P〈0.05）. The hemorrhagic frequency was 27.2 times per year, the total absence from school was 68.9 d per year, the amount of consumption of coagulation factor was （252.0±250.7）U · kg-1 ·year-1 , the prevalence of related complications ranged form 8.8% to 61.4%, Functional Independence Score in Hemophilia （FISH） scored （23.2±3.8）, the medical expense was（13 336.4±11 840.6）Yuan per year. Of 119 children had done related virus infection detection, 3 （2. 5 %）, 12 （ 10.1 %） and 4 （3.4 %） had been infected with HBV, HCV, and HIV respectively, the total infection rate was 16.0%. Significant differences were found in the age of replacement therapy, in the amount of con- sumption of coagulation factor, in the medical expense, in the occurrence rate of complications of muscle and joints, and in the FISH score between rural and urban children（P〈0.05,P〈0.01）. Conclusion There is a delay of diagnosis, insufficiency of coagu- lation factor consumption, high prevalence of complications, and poor self-care ability in hemophilia children, especially those who come from countryside. Training program should be provided for health care staff, and comprehensive care team should be set up to provide overall caring, so as to achieve early diagnosis and treatment, and improve efficacy of treatment.