唐山地区城乡血友病患者临床特征分析

Clinical Characteristics of Patients With Hemophilia in Tangshan Urban and Rural Areas

目的探讨唐山地区城乡血友病患者的临床特征及其差异性。方法选取2010年5月—2011年6月在华北理工大学附属医院就诊的血友病患者83例。患者在就诊时填写由课题组自行设计的《唐山市血友病患者信息管理中心登记表》,内容主要包括年龄、性别、家族史;血友病类型、凝血因子水平和首次出血、诊断、治疗年龄及出血部位、关节畸形、替代治疗制品、抑制物检测;是否合并HBV、HCV、HIV感染。结果 83例患者全部为男性;年龄为8个月~71岁,平均为（19.4±11.2）岁;城市48例（57.8%）,农村35例（42.2%）;血友病A 75例（90.4%）,血友病B 8例（9.6%）;轻型13例（15.7%）,中型45例（54.2%）,重型25例（30.1%）;出血部位：关节出血30例（36.2%）,肌肉出血24例（28.9%）,皮下血肿18例（21.7%）,血尿7例（8.4%）,牙龈出血4例（4.8%）;出血类型：损伤后出血52例（62.6%）,自发性出血31例（37.4%）;家族史阳性率为23.0%（19/83）,肝炎病毒感染阳性率为12.0%（10/83）,未发现HIV感染病例,关节畸形发生率为60.2%（50/83）;在替代治疗制品选择上,64例（77.1%）患者使用凝血因子Ⅷ（FⅧ）,7例（8.4%）使用凝血酶原复合物,12例（14.5%）使用冷沉淀或其他;仅13例（15.7%）患者进行了抑制物检测,其中6例（7.2%）呈阳性;城市与农村患者年龄、血友病类型、凝血因子水平分型、出血类型、家族史阳性率、肝炎病毒感染阳性率间差异无统计学意义（P〉0.05）,而出血部位、关节畸形发生率间差异有统计学意义（P〈0.05）。城市患者首次出血年龄（1.3±0.2）岁,农村患者（0.9±0.2）岁;城市患者首次诊断年龄（3.1±0.2）岁,农村患者（3.7±0.2）岁;城市患者首次治疗年龄（4.4±0.4）岁,农村患者（5.3±0.3）岁;城市与农村患者首次出血、诊断、治疗年龄间差异均有统计学意义（P〈0.05）。结论唐山地区城乡血友病患者诊断及治疗时间均明显延后,凝血因子是主要的替代治疗选择;农村患者首次出血年龄早于城市患者,而首次诊断、治疗年龄晚于城市患者,关节畸形发生率高于城市患者。

Objective To analyze the clinical characteristics and the differences of patients with hemophilia in Tangshan urban and rural areas. Methods We enrolled 83 hemophiliac patients who received treatment in North China University of Science and Technology Affiliated Hospital from May 2010 to June 2011. Survey was conducted when the patients were receiving treatment by a self-designed registration form of Tangshan Hemophiliac Patient Information Management Center which covered age,gender, family history of hemophilia, type of hemophilia, blood coagulation factor level, age at initial bleeding,diagnosis,and treatment, bleeding sites, joint deformity, replacement therapeutic products, detection of inhibitor, and whether the infection of HBV,HCV and HIV was combined. Results The 83 patients were all males aged 8 months to 71 years,with an average age of（ 19. 4 ± 11. 2）. Among the patients,48（ 57. 8%） patients were from urban area and 35（ 42. 2%）patients were from rural area,and 75（ 90. 4%） patients were with hemophilia A and 8（ 9. 6%） patients were with hemophilia B. The number of patients with mild, medium and severe hemophilia was 13（ 15. 7%）,45（ 54. 2%） and 25（ 30. 1%）respectively. As for bleeding sites, the number of patients with joint bleeding, muscle bleeding, subcutaneous hematoma,hematuresis and gum bleeding was 30（ 36. 2%）, 24（ 28. 9%）, 18（ 21. 7%）, 7（ 8. 4%）, and 4（ 4. 8%）respectively. As regards hemorrhage type,52（ 62. 6%） patients had bleeding after injury and 31（ 37. 4%） patients had spontaneous bleeding. The family history of positive hemophilia was 23. 0%（ 19 /83）. The rate of positive hepatitis virus infection was 12. 0%（ 10 /83）,and no HIV infection cases were found. The incidence of joint deformity was 60. 2%（ 50 /83）. With regards to the choice of alternative therapeutic products,64（ 77. 1%） patients used FⅧ and 7（ 8. 4%） patients used prothrombin complex,with cryoprecipitation and others used by 12（ 14. 5%） patients. There were just 13（ 15. 7%） patients who had detection of inhibitor,and the results of 6（ 7. 2%） patients were positive. There were no significant differences in age,type of hemophilia, blood coagulation factor classification, hemorrhage type, family history of positive hemophilia and the positive rate of hepatitis virus infection between urban patients and rural patients（ P 〈0. 05）, but there were significant differences in bleeding sites and the incidence of joint deformity（ P 〈0. 05）. For urban patients,the age at initial bleeding,diagnosis and treatment was（ 1. 3 ± 0. 2）,（ 3. 1 ± 0. 2） and（ 4. 4 ± 0. 4） respectively. For rural patients,the age at initial bleeding,diagnosis and treatment was（ 0. 9 ± 0. 2）,（ 3. 7 ± 0. 2） and（ 5. 3 ± 0. 3）. Urban patients and rural patients were significantly different in the age at initial bleeding,diagnosis and treatment（ P 〈0. 05）. Conclusion Hemophiliac patients in Tangshan urban and rural areas all have late diagnosis and treatment, and blood coagulation factor is a major replacement treatment option. Rural patients have lower age at initial bleeding than urban patients,but have higher age at initial diagnosis and treatment. The incidence of joint deformity was higher in rural patients than urban patients.