血友病骨关节病101例X线表现及临床分析

Clinical and radiological analysis of 101 hemophilic arthropathy cases.

目的 通过单中心较大样本的X线检查及临床分析，了解我国血友病骨关节病的治疗现状。方法 回顾性分析101例血友病患者的临床资料和骨关节的放射学积分。结果 ①患者首次发生异常出血和首次就诊的中位年龄分别为1岁和15岁，诊断时间延迟。②骨关节病在重型、中型、轻型血友病的发生率分别为77．8％、53．6％、20．0％，中型、轻型患者也有较高的罹患率。易受累的关节依次为膝、肘、踝；③在0～9岁、10～19岁、20～29岁、30～39岁、40～49岁、≥50岁等各年龄段，骨关节病的发病率分别为37．5％、59．4％、78．3％、83．3％、76．9％、100％。④治疗组和非治疗组骨关节病的发病率分别为69．4％和62．1％。差异无显著性；⑤骨关节畸形的发生率在具有靶关节组和无靶关节组分别为71．2％和20．0％，差异有显著性。结论 我国血友病患者骨关节病发病率高，成为影响患者健康相关生活质量的主要因素之一。靶关节可以作为判断发生病变骨关节预后的较好指标。

Objective To analyze the situation of hemophilic arthropathy by single center,ease X-ray examination and clinical analysis. Methods Clinical data and radiologieal scores of 101 cases of hemophilic arthropathy were retrospectively investigated. Results The median ages of initial bleeding and initial diagnosis were 1 year old and 15 years old, respectively, which showed that the diagnosis was delayed. The incidences of arthropathy and joint deformity in severe,moderate and mild PWH were 77.8% ,53.6% aid 20.0% respectively. A fairly high rate of arthropathy was also found in patients with mild and moderate PWH. The joints commonly involved were knees,elbows and ankles. The incidences of arthropathy in PWH according to age groups 0-9,10-19,20-29,30-39,40-49 and ≥50 years old were 37.5% ,59.4% ,78.3% ,83.3% ,76.9% and 100% respectively. The incidence of arthropathy in the group treated with replacement therapy and in the group not treated with replacement therapy was 69.4% and 62.1% ,with no statistical difference. The incidence of joint deformity in patients with target joints and without target joints was 71.2% and 20.0%, respectively, with significant difference. Conelusion The higli incidence of hemophilic arthropathy becomes one of the main influencing factors of the quality of life in our country. Target joints can be regarded as better predictors of the prognosis of the joints with lesions.