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Am J Phys Med Rehabil. 2014 Jul;93(7):595-9. doi: 10.1097/PHM.0000000000000074.

Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support.

Author information

1
From 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).

Abstract

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.

PMID:
24743468
DOI:
10.1097/PHM.0000000000000074
[Indexed for MEDLINE]

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