Unit of Physical Medicine and Rehabilitation, S. Matteo Polyclinic, IRCCS and Foundation, Pavia, Italy.
Unit of Physical Medicine and Rehabilitation, University of Pavia, Pavia, Italy -
Minerva Pediatr. 2020 Apr;72(2):73-78. doi: 10.23736/S0026-4946.17.04681-3. Epub 2017 Feb 17.
The Ehlers-Danlos Syndrome (EDS) is a rare disorder affecting the connective tissue. EDS patients may suffer of proprioception and balance impairment but all the studies dealing with such symptoms have been addressed to adult subjects. The Study of such impairment in younger patients may lead to a better awareness of own motor abilities and to a focused rehabilitative intervention. Therefore, our work aims to assess the occurrence of these alterations in a pediatric cohort of EDS patients.
The Research was designed as a cross-sectional study with a matching control group and performed on a pediatric cohort of 12 subjects with Ehlers-Danlos Syndrome (Classic and Hypermobility type) and on 12 healthy controls, during a follow-up visit at the Department of Pediatrics and at the Rehabilitation Unit of the Foundation IRCCS Policlinico San Matteo, in Pavia from April 2015 to October 2015. A square forceplatform was used to obtain the CoP (center of pressure) displacement during quiet standing during an open and a closed eyes trials. The comparisons between EDS and control group were performed using a t-test for independent data. P<0.05 was considered statistically significant. All tests were two-sided.
All the postural parameters considered raised at closed eyes, no significant modifications without vision were found only for the standard deviation along the antero-posterior (AP) axis for the two groups. Both at open eyes and at closed eyes, Patients with EDS showed the postural parameters significantly greater than controls (P≤0.05) and this observation was most notably for the Sway.
According to our results, a planned monitoring of age-related changes in postural parameters of patients with EDS could be really interesting to provide a perspective of the development of postural control in these patients. In fact, considering our results, it could be interesting to apply rehabilitative strategies to enhance motor coordination and postural reflexes so improving their postural control as soon as possible. Further studies about the postural control in EDS children and adolescents are required to confirm our results.
埃勒斯-当洛斯综合征（EDS）是一种罕见的影响结缔组织的疾病。EDS 患者可能会出现本体感觉和平衡障碍，但所有涉及此类症状的研究都针对成年患者。研究这些在年轻患者中的损伤可能会提高对自身运动能力的认识，并进行有针对性的康复干预。因此，我们的工作旨在评估儿科 EDS 患者群体中这些改变的发生情况。
该研究设计为一项横断面研究，有匹配的对照组，并在 2015 年 4 月至 10 月期间，在帕维亚的儿科系和圣马特奥基金会 IRCCS 综合医院康复科，对 12 名 Ehlers-Danlos 综合征（经典型和高活动型）患儿和 12 名健康对照者进行了研究。使用方形测力台在睁眼和闭眼试验中获得安静站立时的 CoP（中心压力）位移。使用独立数据 t 检验比较 EDS 组和对照组。P<0.05 被认为具有统计学意义。所有测试均为双侧。
在闭眼时，所有考虑的姿势参数都升高，只有两组的前-后（AP）轴标准差没有明显的无视觉变化。在睁眼和闭眼时，EDS 患者的姿势参数都明显大于对照组（P≤0.05），这一观察在摇摆方面尤为明显。
根据我们的结果，对 EDS 患者的姿势参数进行与年龄相关的监测可能会很有趣，可以提供这些患者的姿势控制发展的视角。事实上，考虑到我们的结果，应用康复策略来增强运动协调和姿势反射可能会很有趣，从而尽快改善他们的姿势控制。需要进一步研究 EDS 儿童和青少年的姿势控制，以确认我们的结果。
