Abstract | UNLABELLED: METHODS: We evaluated the reliance upon mechanical ventilation (MV), ventilatory kinematics, unassisted tidal volumes, and maximal respiratory pressures in 14 individuals with CNMs, including 10 boys with XLMTM. RESULTS: Thirteen participants required full-time, invasive MV. Maximal inspiratory pressures were higher in subjects who breathed unsupported at least 1 hour/day as compared with 24-hour MV users [33.7 (11.9-42.3) vs. 8.4 (6.0-10.9) cm H(2)O, P < 0.05]. Years of MV dependence correlated significantly with MEP (r = -0.715, P < 0.01). CONCLUSIONS: Respiratory function in CNMs may be related to deconditioning from prolonged MV and/or differences in residual respiratory muscle strength. Results from this study may assist in evaluating severe respiratory insufficiency in neuromuscular clinical care and research.
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Authors | Barbara K Smith, Markus S Renno, Meghan M Green, Terry M Sexton, Lee Ann Lawson, Anatole D Martin, Manuela Corti, Barry J Byrne |
Journal | Muscle & nerve
(Muscle Nerve)
Vol. 53
Issue 2
Pg. 214-21
(02 2016)
ISSN: 1097-4598 [Electronic] United States |
PMID | 26351754
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
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Copyright | © 2015 Wiley Periodicals, Inc. |
Topics |
- Adolescent
- Adult
- Aged
- Child
- Child, Preschool
- Female
- Humans
- Infant
- Male
- Middle Aged
- Myopathies, Structural, Congenital
(complications, therapy)
- Pressure
- Respiration Disorders
(diagnosis, etiology)
- Respiration, Artificial
(methods)
- Respiratory Function Tests
(methods)
- Respiratory Muscles
(physiopathology)
- Young Adult
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