title: Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support.
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.
  
  
  METHODS
  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.
authors.affiliation: 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).
authors.forename: Marcello
authors.initials: M
authors.lastname: Villanova
authors.forename: Beatrice
authors.initials: B
authors.lastname: Brancalion
authors.forename: Anokhi D
authors.initials: AD
authors.lastname: Mehta
mesh_terms: D000328:Adult; D002245:Carbon Dioxide / Q000097:blood; D005500:Follow-Up Studies; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation; D008136:Longevity / Q000502:physiology; D008297:Male; D008875:Middle Aged; D020388:Muscular Dystrophy, Duchenne / Q000097:blood / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy*; D063087:Noninvasive Ventilation; D010108:Oxyhemoglobins / Q000032:analysis; D011674:Pulse; D012890:Sleep / Q000502:physiology; D013147:Spirometry; D013990:Tidal Volume / Q000502:physiology; D014797:Vital Capacity / Q000502:physiology; D055815:Young Adult
chemical_list: D010108:Oxyhemoglobins; D002245:Carbon Dioxide