title: Long-term nasal intermittent positive pressure ventilation in advanced Duchenne's muscular dystrophy.
abstract: The aim of our study was to evaluate the long-term effect of nasal ventilation in patients with advanced Duchenne's muscular dystrophy (DMD). To this end, we compared the clinical and pulmonary function course of five subjects affected with chronic ventilatory failure due to DMD and treated with nasal intermittent positive pressure ventilation (NIPPV) with that of an unventilated comparison group; the latter consisted of another five patients with DMD, with a similar degree of clinical and respiratory functional impairment, who refused long-term mechanical ventilation. The duration of the follow-up was 24 months. At the conclusion of the trial, all patients treated with NIPPV were still alive; in contrast, four of five patients who underwent simple conservative treatment had already died (mean survival, 9.7 +/- 5.8 months). After 6 months of follow-up, mean loss of FVC and maximal voluntary ventilation was considerably higher in nonventilated subjects (respectively: -0.23 L vs +0.03 L and -5 L/min vs -1.5 L/min). These are the first comparative results confirming that long-term NIPPV helps to stabilize pulmonary function and to prolong the expectancy of life of patients with DMD.
authors.affiliation: Department of Respiratory Pathophysiology, University-City Hospital of Padua, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Vianello
authors.forename: M
authors.initials: M
authors.lastname: Bevilacqua
authors.forename: V
authors.initials: V
authors.lastname: Salvador
authors.forename: C
authors.initials: C
authors.lastname: Cardaioli
authors.forename: E
authors.initials: E
authors.lastname: Vincenti
mesh_terms: D000293:Adolescent; D000328:Adult; D002245:Carbon Dioxide / Q000097:blood; D002648:Child; D002908:Chronic Disease; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D006935:Hypercapnia / Q000097:blood / Q000503:physiopathology; D007385:Intermittent Positive-Pressure Ventilation / Q000295:instrumentation / Q000379:methods*; D008297:Male; D008397:Masks; D008451:Maximal Voluntary Ventilation / Q000502:physiology; D009136:Muscular Dystrophies / Q000150:complications* / Q000503:physiopathology; D010100:Oxygen / Q000097:blood; D012131:Respiratory Insufficiency / Q000097:blood / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D015996:Survival Rate; D014797:Vital Capacity / Q000502:physiology
chemical_list: D002245:Carbon Dioxide; D010100:Oxygen