期刊
MUSCLE & NERVE
卷 62, 期 6, 页码 681-687出版社
WILEY
DOI: 10.1002/mus.27039
关键词
distal myopathy; dysphagia; EMST150; nephropathic Cystinosis; respiratory therapy; video fluoroscopy
资金
- Cystinosis Research Foundation
Background Nephropathic cystinosis is a lysosomal storage disorder with late-onset systemic complications, such as myopathy and dysphagia. Currently employed outcome measures lack sensitivity and responsiveness for dysphagia and myopathy, a limitation to clinical trial readiness. Methods We evaluated 20 patients with nephropathic cystinosis in two visits over the course of a year to identify outcomes sensitive to detect changes over time. Patients also underwent an expiratory muscle strength training program to assess any effects on aspiration and dysphagia. Results There were significant differences in the Timed Up and Go Test (TUG) and Timed 25-Foot Walk (25-FW) between baseline and 1-y follow-up (P< .05). Maximum expiratory pressure (MEP) and peak cough flow (PCF) significantly improved following respiratory training (P< .05). Conclusions Improved respiratory outcomes may enhance patients ability to expel aspirated material from the airway, stave off pulmonary sequelae associated with chronic aspiration, and yield an overall improvement in physical health and well-being.
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