혈우병관절건강점수와 페터슨점수로 평가한 혈우병 환자의 관절 건강

Abstract

Background: Comprehensive clinical and radiologic follow-up is needed to preserve joint functions and quality of life in hemophilia using clinimetric tools such as Hemophilia joint health score (HJHS) or Pettersson score (PS). We investigated the joint health status evaluated using the tools in Korean hemophilia patients. Methods: We reviewed retrospectively medical records to collect clinical parameters, HJHS and PS, who were followed up in Severance Hospital, Seoul, Korea. The correlation between HJHS and PS, and the effect of the prophylaxis for hemophilia on the outcomes measured with the scores were evaluated. The prophylaxis proportion (PP) was calculated as the proportion of prophylaxis duration to each patient’s life time. Results: Total of 28 patients with severe hemophilia were enrolled. Twelve patients (42.8%) were less than 20 years old. Total of 23 patients had experienced prophylaxis during their lives, and median PP was 39.7%. There was significant correlation between HJHS and PS (P＜0.001). Each score was positively correlated with patient’s age (P＜0.001). PP was negatively correlated with either HJHS or PS (P＜0.001, respectively). There was significant correlation between either HJHS or PS and the PP in the group of patients ＜20 years old, but there was no correlation in the group of ＞20 years old. Conclusion: HJHS and PS were positively correlated. Each score increased as the patient’s age increased. The prophylaxis had protective effect on joint health. The prospective evaluation of HJHS and PS will be needed to prove the effect of proper management on the joint health status.