1.Analysis of Bone Health Status in Adult Hemophilia Patients
Ying LIU ; Ying GE ; Mingnan SHI ; Li ZHANG ; Chengjie YIN ; Lixia CHEN ; Weibo XIA
JOURNAL OF RARE DISEASES 2025;4(4):446-452
To investigate the bone health status and potential influencing factors of bone mineral density in adult patients with hemophilia, providing a reference for improving their bone health and for the prevention, treatment, and rehabilitation intervention of osteoporosis. This study is a retrospective analysis. Adult male patients with hemophilia A who visited the department of rehabilitation medicine at Peking Union Medical College Hospital from July 2022 to February 2024 were selected. Dual-energy X-ray absorptiometry (DXA) and high-resolution peripheral quantitative computed tomography (HR-pQCT) were used to assess the bone mineral density (BMD) of the patients. Anterolateral X-rays and Pettersson radiology scores were performed on the left ankle joint. Hemophilia Joint Health Score (HJHS) version 2.1 was used to calculate the HJHS score of the left ankle and evaluate the joint health status of the included patients. Serum bone metabolism indexes including total procollagen Ⅰ N-terminal propeptide (TP1NP) and C-terminal crosslinking β-isomerized carboxy-telopeptide of type Ⅰ collagen (β-CTX), and serum 25-(OH)D3 were detected. The patients' body composition parameters were measured, including the body mass index(BMI)and the skeletal muscle mass index (SMI). The correlation analysis of BMD detection values and its possible influencing factors was carried out. A total of 33 adult male patients with hemophilia A were selected, including 22 severe patients and 11 moderate patients, with an average age of 31.1±8.4 years. The hip BMD of the included patients was lower than the predicted value of age to varying degrees, and the minimum The hip BMD of adult hemophilia patients decreased.Joint dysfunction may be one of the factors associated with reduced bone density in patients with hemophilia. The increase in osteoclast activity may be accompanied by an increase in compensatory osteoblast activity.
2.Factors related to the physical activity levels of Chinese adults with haemophilia
Shufen LIU ; Houqiang ZHANG ; Weihong SHI ; Runlin SHI ; Mingnan SHI ; Lixia CHEN
Chinese Journal of Physical Medicine and Rehabilitation 2024;46(8):726-730
Objective:To explore the level of physical activity of Chinese adults with haemophilia and its related factors so as to provide a reference for their comprehensive management.Methods:The daily physical activity of adults with haemophilia was surveyed using the International Physical Activity Questionnaire (IPAQ). A total of 110 questionnaires were eligible for analysis at last. The results were compared with published data from other societies using independent sample t-tests and chi squared tests. Spearman correlation analysis and multivariate logistic regression analysis were applied to explore factors related to the subjects′ physical activity levels.Results:Fully 30.9% of the subjects claimed to maintain a high level of physical activity. This was significantly different from the distribution of physical activity observed in Japan (12.4%), Ireland (50.9%) and the United Kingdom (43.1%). According to the Spearman correlation analysis, the number of joints affected, anxiety, and depression are all significantly related to the level of physical activity among such adults. Multivariate logistic regression analysis showed that depression was an independent factor affecting the patients′ level of physical activities.Conclusion:Chinese and Japanese adults with hemophilia have relatively low levels of physical activity, possibly influenced by depression.
3.The minimum clinically-important difference in rehabilitation for children with haemophilic knee joint contracture
Houqiang ZHANG ; Shufen LIU ; Mingnan SHI ; Weihong SHI ; Lixia CHEN
Chinese Journal of Physical Medicine and Rehabilitation 2022;44(12):1095-1099
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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