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Rehabilitation Practice and Science

Translated Title

血友病關節病變與健康相關生活品質之相關性研究

Abstract

Introduction: Hemophilia is an X-linked recessive bleeding disorder caused by a deficiency of coagulation factor VIII (hemophilia A) or IX (hemophilia B). The prevalence of hemophilia is approximately 1 per 105 in the general population. Repeated joint bleeding leads to chronic synovitis, cartilage damage, and bone destruction, yielding reduced range of motion (ROM), painful joints, and muscle atrophy. Hemophilic arthropathy can negatively influence perceptions of health-related quality of life (HRQOL) among hemophilia patients. This study explored the correlation between hemophilic arthropathy and HRQOL among hemophilia patients in Taiwan. Methods: The participants comprised 86 patients enrolled from a hemophilia center. We collected clinical information regarding their age, hemophilia type, disease severity, and factor inhibitor, and their responses to the short form 36 (SF-36). On the same day, the bilateral shoulders, elbows, hips, knees, and ankles were evaluated regarding ROM and assigned a Pettersson score based on an X-ray film. The relations among the SF-36 results and the clinical variables were assessed using the Spearman’s correlation coefficient. Results: Eighty-one hemophilia A and 5 hemophilia B patients were enrolled. The mean age was 30.6 ± 14.1 years. Regarding hemophilic arthropathy, the most commonly affected joints were the ankle (66.3%), elbow (53.5%), and knee (47.7%). The SF-36 scores of the hemophilia patients were worse compared with Taiwanese normative values. The summarized SF-36 and Pettersson scores (r = -0.560, P ＜ .001), ROM (r = 0.538, P ＜ .001), and age (r = -0.426, P ＜ .001) exhibited significant correlations. No significant correlation was observed between hemophilia severity and SF-36. Conclusion: A high Pettersson score, reduced ROM, and increased age were correlated to a low HRQOL among hemophilia patients. The worse the arthropathy was, the lower the HRQOL was. If hemophilic arthropathy can be appropriately managed to prevent ROM deterioration, it could yield a substantial quality of life improvement for hemophilia patients.

Language

Traditional Chinese

First Page

235

Last Page

242

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