Garcia Nathaniel, Lytch Ashley, Cramer Emily, Black William R, Jones Jordan T
University of Kansas School of Medicine, Kansas City, KS.
Children's Mercy Kansas City, Kansas City, MO.
Arch Rehabil Res Clin Transl. 2024 Dec 2;7(1):100416. doi: 10.1016/j.arrct.2024.100416. eCollection 2025 Mar.
To identify how pediatric patients with hypermobile-type Ehlers-Danlos syndrome (hEDS) present to physical therapy (PT) and better understand their musculoskeletal dysfunction.
Patients were included in the study if they had a diagnosis of hEDS and at least 1 completed PT evaluation after diagnosis. A chart review was conducted to assess joint strength and pain characteristics at the initial PT assessment. Physical Function Mobility (PF-Mobility; 4 domains with 1-5 scale with higher score representative of better physical function) as part of the Patient-Reported Outcome Measurement Information System and pain intensity score (visual analog scale, 0-10 scale where a lower number indicates less pain) was completed by participants.
Midwestern tertiary care center.
Sixty-nine participants were included in the study with an average age of 16.4 years (SD 2.8) at the initial PT visit and 96% were female individuals.
Not applicable.
Joint strength, pain intensity and location, and PF-Mobility.
The knee was the most reported location with pain (68%) followed by the shoulder (46%), hip (41%), and back (39%). Average pain intensity was 5.0 (SD 1.8), joint strength scores ranged from 8.2 (SD 0.9) to 9.9 (SD 0.4), and the PF-Mobility mean was 3.3 (SD 0.6). No correlation was noted between joint pain intensity and strength at the joint.
Although the participants perceived poor mobility and significant pain, PT-assessed strength did not reveal significant muscle weakness. Additional work is needed to explore this discrepancy, including altered biomechanics, evaluation methods, and nonmusculoskeletal factors such as neuropathic and psychological components.
确定患有活动过度型埃勒斯-当洛综合征（hEDS）的儿科患者如何接受物理治疗（PT），并更好地了解他们的肌肉骨骼功能障碍。
如果患者被诊断为hEDS且在诊断后至少有1次完整的PT评估，则纳入本研究。进行病历审查以评估初始PT评估时的关节力量和疼痛特征。患者完成了患者报告的结局测量信息系统的一部分——身体功能活动能力（PF-活动能力；4个领域，1-5分制，分数越高代表身体功能越好）以及疼痛强度评分（视觉模拟量表，0-10分制，分数越低表示疼痛越轻）。
中西部三级医疗中心。
69名参与者纳入本研究，初次PT就诊时的平均年龄为16.4岁（标准差2.8），96%为女性。
不适用。
关节力量、疼痛强度和部位以及PF-活动能力。
最常报告疼痛的部位是膝盖（68%），其次是肩膀（46%）、臀部（41%）和背部（39%）。平均疼痛强度为5.0（标准差1.8），关节力量评分在8.2（标准差0.9）至9.9（标准差0.4）之间，PF-活动能力平均值为3.3（标准差0.6）。关节疼痛强度与关节力量之间未发现相关性。
尽管参与者感觉活动能力差且疼痛明显，但PT评估的力量并未显示出明显的肌肉无力。需要进一步研究以探讨这种差异，包括生物力学改变、评估方法以及神经病变和心理因素等非肌肉骨骼因素。
