title: Impact of Noninvasive Ventilation on Lung Volumes and Maximum Respiratory Pressures in Duchenne Muscular Dystrophy.
abstract: BACKGROUND
  Duchenne muscular dystrophy (DMD) is a sex-linked genetic disorder in which progressive impairment of skeletal muscle function eventually leads to severe respiratory failure requiring continuous noninvasive ventilation (NIV) at home. A current focus of debate is whether NIV may slow the decline in respiratory function or, on the contrary, worsen respiratory function when started early. Our objective here was to describe the effects of NIV on vital capacity (VC) and maximum respiratory pressures in DMD.
  
  
  METHODS
  We analyzed retrospective data from 71 subjects with DMD, including VC, maximum static respiratory pressures, and sniff nasal inspiratory pressure before and after NIV initiation. The declines in these variables from the highest value to the most recent value were computed.
  
  
  RESULTS
  Although respiratory function continued to deteriorate over time, NIV introduction was followed by significant slowing in the annual rates of decline in VC (from 4.28 to 1.36 percent predicted), maximum inspiratory pressure (from 2.77 to 1.48 cm H2O), and maximum expiratory pressure (from 2.00 to 1.00 cm H2O). NIV had no effect on sniff nasal inspiratory pressure.
  
  
  CONCLUSIONS
  Introducing NIV in subjects with DMD was followed by slowing of the declines in VC and in maximum static inspiratory and expiratory pressures.
authors: INSERM U 1179-Université de Versailles Saint Quentin en Yvelines, Versailles, France.|Centro de Fisioterapia e Reabilitação-Hospital Universitário de Brasília, Universidade de Brasília, Brasilia, Brazil.
authors: Dante
authors: D
authors: Brasil Santos
authors: CIC 1429-INSERM-APHP, Hôpital Raymond Poincaré, Garches, France.
authors: Isabelle
authors: I
authors: Vaugier
authors: INSERM U 1179-Université de Versailles Saint Quentin en Yvelines, Versailles, France.
authors: Ghilas
authors: G
authors: Boussaïd
authors: CIC 1429-INSERM-APHP, Hôpital Raymond Poincaré, Garches, France.|Service de Réanimation, APHP-Hôpital Raymond Poincaré, Garches, France.
authors: David
authors: D
authors: Orlikowski
authors: INSERM U 1179-Université de Versailles Saint Quentin en Yvelines, Versailles, France.|Physiologie-Explorations Fonctionnelles, APHP, Hôpital Raymond Poincaré, Garches, France.
authors: Hélène
authors: H
authors: Prigent
authors: INSERM U 1179-Université de Versailles Saint Quentin en Yvelines, Versailles, France. f.lofaso@aphp.fr.|Physiologie-Explorations Fonctionnelles, APHP, Hôpital Raymond Poincaré, Garches, France.
authors: Frédéric
authors: F
authors: Lofaso
mesh_terms: D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008168:Lung / Q000503:physiopathology; D008176:Lung Volume Measurements / Q000379:methods; D008297:Male; D000072277:Maximal Respiratory Pressures / Q000379:methods*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000503:physiopathology* / Q000628:therapy; D063087:Noninvasive Ventilation / Q000379:methods*; D010102:Oxygen Inhalation Therapy / Q000379:methods*; D012119:Respiration; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D013990:Tidal Volume; D016896:Treatment Outcome; D014797:Vital Capacity / Q000502:physiology; D055815:Young Adult
keywords: Duchenne muscular dystrophy; follow-up; neuromuscular disease; noninvasive ventilation