Background Shoe inserts, orthopaedic shoes, ankle-foot orthoses (AFOs) are important devices in Charcot-Marie-Tooth disease (CMT) management, but data about use, benefits and tolerance are scanty. Methods We administered to Italian CMT Registry patients an online ad hoc questionnaire investigating use, complications and perceived benefit/tolerability/emotional distress of shoe inserts, orthopaedic shoes, AFOs and other orthoses/aids. Patients were also asked to fill in the Quebec User Evaluation of Satisfaction with assistive Technology questionnaire, rating satisfaction with currently used AFO and related services. Results We analysed answers from 266 CMT patients. Seventy per cent of subjects were prescribed lower limb orthoses, but 19% did not used them. Overall, 39% of subjects wore shoe inserts, 18% orthopaedic shoes and 23% AFOs. Frequency of abandonment was high: 24% for shoe inserts, 28% for orthopaedic shoes and 31% for AFOs. Complications were reported by 59% of patients and were more frequently related to AFOs (69%). AFO users experienced greater emotional distress and reduced tolerability as compared with shoe inserts (p<0.001) and orthopaedic shoes (p=0.003 and p=0.045, respectively). Disease severity, degree of foot weakness, customisation and timing for customisation were determinant factors in AFOs’ tolerability. Quality of professional and follow-up services were perceived issues. Conclusions The majority of CMT patients is prescribed shoe inserts, orthopaedic shoes and/or AFOs. Although perceived benefits and tolerability are rather good, there is a high rate of complications, potentially inappropriate prescriptions and considerable emotional distress, which reduce the use of AFOs. A rational, patient-oriented and multidisciplinary approach to orthoses prescription must be encouraged. Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information. Data relevant to the study are included in the article or uploaded as online supplemental information. Data supporting study results are deposited in an ad hoc repository and are available from the Principal Investigator (DP) to be shared anonymously on request from any qualified investigator.

中文翻译：

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矫形器在腓骨肌萎缩症中的使用、耐受性、益处和副作用

背景 鞋垫、矫形鞋、踝足矫形器 (AFO) 是腓骨肌萎缩症 (CMT) 治疗的重要设备，但有关使用、益处和耐受性的数据却很少。方法 我们向意大利 CMT 登记患者进行了一份在线临时问卷调查，调查鞋垫、矫形鞋、AFO 和其他矫形器/辅助器具的使用、并发症和感知益处/耐受性/情绪困扰。患者还被要求填写魁北克辅助技术满意度用户评估调查问卷，对当前使用的 AFO 和相关服务进行满意度评级。结果 我们分析了 266 名 CMT 患者的答案。 70% 的受试者佩戴了下肢矫形器，但 19% 的受试者没有使用。总体而言，39% 的受试者穿着鞋垫，18% 的受试者穿着矫形鞋，23% 的受试者穿着 AFO。废弃频率很高：鞋垫为 24%，矫形鞋为 28%，AFO 为​​ 31%。 59% 的患者报告了并发症，并且更常见的是与 AFO 相关 (69%)。与鞋垫 (p<0.001) 和矫形鞋 (分别为 p=0.003 和 p=0.045) 相比，AFO 使用者经历了更大的情绪困扰和更低的耐受性。疾病严重程度、足部无力程度、定制和定制时间是 AFO 耐受性的决定因素。专业和后续服务的质量被认为是问题。结论 大多数 CMT 患者都使用鞋垫、矫形鞋和/或 AFO。尽管感知到的益处和耐受性相当好，但并发症发生率很高，可能存在不适当的处方和相当大的情绪困扰，这些都减少了 AFO 的使用。必须鼓励对矫形器处方采取合理的、以患者为导向的多学科方法。数据可根据合理要求提供。与研究相关的所有数据都包含在文章中或作为补充信息上传。与研究相关的数据包含在文章中或作为在线补充信息上传。支持研究结果的数据存放在临时存储库中，首席研究员 (DP) 可以提供这些数据，并可根据任何合格研究人员的要求匿名共享。