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Updated: May 1, 2026

Assessing Functional Performance in the Mdx Mouse Model
Published on: March 27, 2014
Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support
Marcello Villanova1, Beatrice Brancalion, Anokhi D Mehta
1From the Unità di Recupero e Riabilitazione Funzionale Malattie Neuromuscolari, Ospedale Nigrisoli, Bologna, Italy (MV, BB); and Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, Newark, NJ (ADM).
Objective:
American, Japanese, and Canadian centers have demonstrated that noninvasive intermittent positive pressure ventilatory support (NVS) can be used continuously and in the long-term by people with Duchenne muscular dystrophy as a definitive alternative to tracheostomy mechanical ventilation. The aim of this study was to report this for the first time in Europe.
Design:
In this study, more than 300 patients with Duchenne muscular dystrophy were followed. End-tidal carbon dioxide, oxyhemoglobin saturation, and vital capacity were measured at each visit. Of the 300 patients, 79 used NVS for 8 hrs or more per day and 20 of these became continuously dependent on NVS.
Results:
A total of 20 patients have continuously depended on NVS for survival, for a total of 336 patient-years, up to 16 yrs in one case. Nocturnal NVS was begun for symptomatic hypoventilation when the vital capacity had decreased to a mean of 831 ± 173 ml, and continuous dependence on NVS was necessary when the vital capacity had decreased below 297 ± 113 ml.
Conclusions:
Noninvasive respiratory management can prolong survival without resorting to tracheotomy and without hospitalization.
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Published on: April 10, 2019
09:18Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
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