National Center on Birth Defects and Developmental Disabilities, Division of Blood Disorders, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA.
Haemophilia. 2011 Mar;17(2):196-203. doi: 10.1111/j.1365-2516.2010.02400.x.
Although body adiposity and disease severity in haemophilia have been found in cross-sectional studies to be negatively associated with joint mobility, it is not clear how these two factors affect the rate of joint mobility loss over time. Over a 10-year period, repeated measures of joint range of motion (ROM) were collected annually using universal goniometers on bilateral hip, knee, ankle, shoulder and elbow joints in 6131 young males with haemophilia A aged ≤ 20 years. Body mass index (BMI) was calculated using data on weight and height during follow up. The effect of body adiposity, adjusted for disease severity, on the rate of joint mobility loss over time was assessed using a longitudinal model. Compared with haemophilia males with normal BMI, those who were obese had lower ROM at initial visit and a faster rate of joint mobility loss in the lower limbs. Overweight subjects experienced similar loss in ROM, although to a lesser degree. A decline in ROM with age was also observed in upper limb joints but the rate was not significantly affected by body adiposity. Haemophilia severity, joint bleeding and the presence of an inhibitor were other significant contributors to joint mobility loss in both upper and lower limb joints. Excess body adiposity accelerates joint mobility loss in weight bearing joints particularly among those with severe haemophilia. Our findings suggest that body weight control and effective treatment of bleeds should be implemented together to achieve better joint ROM outcomes in males with haemophilia.
虽然在横断面研究中发现肥胖和血友病的疾病严重程度与关节活动度呈负相关，但目前尚不清楚这两个因素如何影响关节活动度随时间的丧失率。在一项为期 10 年的研究中，对 6131 名年龄≤20 岁的甲型血友病男性患者的双侧髋关节、膝关节、踝关节、肩关节和肘关节进行了每年一次的全关节角度计重复测量，以评估关节活动度。在随访期间使用体重和身高数据计算体重指数（BMI）。使用纵向模型评估了肥胖程度（根据疾病严重程度调整）对关节活动度随时间丧失率的影响。与 BMI 正常的血友病男性相比，肥胖男性在初始就诊时的 ROM 较低，下肢关节活动度丧失速度较快。超重患者的 ROM 丧失程度相似，尽管程度较轻。在上肢关节中也观察到 ROM 随年龄下降，但肥胖程度对其丧失率没有显著影响。关节出血和抑制剂的存在也是上肢和下肢关节 ROM 丧失的其他重要因素。超重会加速承重关节的关节活动度丧失，尤其是在严重血友病患者中。我们的研究结果表明，应控制体重并有效治疗出血，以实现血友病男性更好的关节 ROM 结果。
