血友病患者关节评估及治疗的研究进展  

Research progress in joint evaluation and treatment of patients with hemophilia

作　　者：王稳 胡群[1] Wang Wen;Hu Qun(Department of Pediatric Hematology,Tongji Hospital,Tongji Medical College of Huazhong University of Science and Technology,Wuhan 430030,Hubei Province,China)

机构地区：[1]华中科技大学同济医学院附属同济医院儿童血液科,武汉430030

出　　处：《国际输血及血液学杂志》2021年第5期456-460,共5页International Journal of Blood Transfusion and Hematology

基　　金：Hemophilia Experiences,Results,and Opportunities(HERO)基金(2018033)。

摘　　要：血友病是一组X连锁隐性遗传性出血性疾病。以自发性出血或者轻微外伤后过度出血为主要临床表现,其中关节出血最常见。反复的关节出血可引起退行性关节病,因此早期进行关节评估极为重要。目前,凝血因子替代治疗仍是血友病的首选治疗,但是患者经反复多次替代治疗后,可产生相应的凝血因子抑制物,影响疗效。多项针对血友病的新治疗方案已取得显著进展,但其安全性仍待进一步研究。笔者拟就血友病多种关节评估及治疗方法进行综述,分析其优、缺点,以期为血友病患者选择合适的诊疗方案提供参考。Hemophilia is an X-linked recessive hereditary bleeding disorder.The main clinical manifestations were spontaneous bleeding or excessive bleeding after minor trauma,among which joint bleeding represents the most commonly reported type of hemorrhage in patients.Repeated bleeding episodes may lead to degenerative arthropathy,so early joint assessment is extremely important.At present,blood coagulation factors replacement therapy is still the preferred treatment for hemophilia,but repeated usage can produce corresponding blood coagulation factors inhibitors and affect the therapeutic effect.Several new treatments have made significant progress,but their safety remains to be further studied.This paper reviews various joint evaluation methods and treatment methods of hemophilia,and analyzes their advantages and disadvantages,in order to provide reference for appropriate diagnosis and treatment schemes for hemophilia patients.

关 键 词：血友病A 血友病B 关节积血 日常生活活动 药物疗法 基因治疗 

分 类 号：R554.1[医药卫生—血液循环系统疾病]