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Objective:To determine the minimum clinically-important difference (MCID) in the rehabilitation effect among children with haemophilic knee joint contracture.Methods:The data describing 28 children with an average age of 13.89±3.00 years and haemophilic knee joint contracture who received no less than 10 sessions of physiotherapy in the Department of Rehabilitation Medicine at the Peking Union Medical College Hospital were analyzed. The therapeutic effect of the treatement was quantified in terms of Haemophilia Joint Health Scores (HJHSs) for their knees. The MCID after the therapy was evaluated using the mean change method, multivariate linear regression, receiver operating characteristics, and the distribution-based method.Results:The MCID for the improvement of knee HJHS was 5.13 by the mean change method, 4.31 by multivariate linear regression, 3.50 according to the ROC curve and 1.64 by the distribution-based method. Taking all of them into consideration, 4.31 was found to be an appropriate value.Conclusions:The MCID after physical therapy for the improvement in knee HJHS for a child with haemophilic knee contracture is 4.31. Improvements greater than 4.31 can be considered clinically significant.
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Objective To investigate the correlation between the quality of life and joint health status of adult hemophilia patients. Methods Forty-nine adult hemophilia patients were selected.The quality of life and joint health status of patients were evaluated by hemophilia-specific health related quality of life questionnaire for adults (Hemo-QOL-A) and hemophilia joint health score (HJHS). Results The Hemo-QOL-A score of adult hemophilia patients was (39.34 ± 18.97) scores, and HJHS score was(33.16 ± 13.17)scores.The Pearson correlation analysis result showed that Hemo-QOL-A score was positively related with HJHS score (r = 0.490, P<0.05), and Hemo-QOL-A score was positively correlated with muscular condition,joint activity and joint friction sound score of HJHS score(r=0.469, 0.396 and 0.290; P<0.05). Hemo-QOL-A score was not correlated with swelling, swelling duration, arthralgia and gait of HJHS score (P>0.05). In 294 joints of 49 patients, the total abnormal rate of the joint was 82.7%(243/294),the abnormal rate of the elbow joint was 72.4%(71/98),the abnormal rate of the knee joint was 90.8%(89/98),and the abnormal rate of ankle joint was 84.7%(83/98).Conclusions Decreased joint health status, mainly including the surrounding muscle condition and joint mobility, results in lower quality of life in patients with hemophilia.
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Objective To explore the joint health of children with haemophilia and its influencing factors.Methods Sixty boys with haemophilia (aged 4-18 years) were recruited from Peking Union Medical College Hospital.They were divided by age into a five to seven year-old group (group A,n=16),an eight to twelve year-old group (group B,n =18) and a thirteen to eighteen year-old group (group C,n =26).The haemophilia joint health score (HJHS) (version 2.1) was used to evaluate the condition of their elbows,knees and ankles.Spearman's method was applied to analyze the influencing factors.Results The average HJHSs of the knees,elbows and ankles of all of subjects were 8.65±6.41,5.30±5.30,and 5.40±4.25 respectively.The average for the knees was significantly higher than for the elbows and ankles.The average HJHS for the elbows of group A was significantly lower than that of their knees.The average HJHS for their knees was not significantly different from that of their ankles.For those aged 13-18 years,the average HJHS for their knees was significantly higher than that for their elbows and ankles.There was no significant difference between the HJHSs for the elbows,knees and ankles of the 8-12 year-olds.The HJHSs were positively correlated with age but negatively correlated with average therapeutic dose and exercise.Conclusion The joint health of haemophilic boys,especially that of the knees,decreases with age.Their joint health can be improved by medication and by taking reasonable physical exercise.