Objective. Repeated hemarthrosis in hemophilia causes arthropathy with pain and dysfunction. The Hemophilia Joint Health Score (HJHS) was developed to be more sensitive for detecting arthropathy than the World Federation of Hemophilia (WFH) physical examination scale, especially for children and those using factor prophylaxis. The HJHS has been shown to be highly reliable. We compared its validity and sensitivity to the WFH scale. Methods. We studied 226 boys with mild, moderate, and severe hemophilia at 5 centers. The HJHS was scored by trained physiotherapists. Study physicians at each site blindly determined individual and total joint scores using a series of visual analog scales. Results. The mean age was 10.8 years. Sixty-eight percent were severe (93% of whom were treated with prophylaxis), 15% were moderate (24% treated with prophylaxis), and 17% were mild (3% treated with prophylaxis). The HJHS correlated moderately with the physician total joint score (r s ؍ 0.42, P < 0.0001) and with overall arthropathy impact (r s ؍ 0.42, P < 0.0001). The HJHS was 97% more efficient than the WFH at differentiating severe from mild and moderate hemophilia. The HJHS was 74% more efficient than the WFH at differentiating subjects treated with prophylaxis from those treated on demand. We identified items on the HJHS that may be redundant or rarely endorsed and could be removed from future versions. Conclusion. Both the HJHS and WFH showed evidence of strong construct validity. The HJHS is somewhat more sensitive for mild arthropathy; its use should be considered for studies of children receiving prophylaxis.
Objective: The aim of this study was to develop a 12-week exercise training program (comprising aerobic and strength exercises), and to study the feasibility and efficacy of this exercise program in children who survived acute lymphoblastic leukemia.Sample and methods: A 12-week exercise program was developed and tested for feasibility in nine children who survived cancer.Results: From the 16 eligible children for the intervention, 9 participated, while 4 were able to complete the entire program. Feasibility of the program was scored by five children, two of them reported the program as being too demanding. The participating physiotherapists were satisfied with training methodology and training progress. The efficacy of the program on muscle strength, exercise capacity, functional mobility and fatigue showed no significant differences between pre and post training.Conclusion: In designing a community-based exercise training program, not only the stage of the disease needs to be considered, but more so the age of the children, the variety of exercises, the location of implementation and even more importantly the views and motivation of the parents concerning the execution of an exercise training program. A careful balance between these parameters could lead to a greater adherence and by that, to a better outcome of these programs.
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