title: Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases.
abstract: OBJECTIVE
  To analyze the physiologic effects and tolerance of mechanical insufflation-exsufflation (MI-E) by means of mechanical cough assistance (Cough Assist; JH Emerson Company; Cambridge, MA) for children with neuromuscular disease.
  
  
  METHODS
  Prospective clinical trial.
  
  
  METHODS
  Physiology laboratory of a pediatric pulmonary department of a university hospital.
  
  
  METHODS
  Seventeen children with Duchenne muscular dystrophy (n = 4), spinal muscular atrophy (n = 4), or other congenital myopathy (n = 9) who were in a stable state.
  
  
  METHODS
  Pressures of 15, 30, and 40 cm H(2)O were cycled to each patient, with 2 s for insufflation and 3 s for exsufflation. One application consisted of six cycles at each pressure for a total of three applications.
  
  
  RESULTS
  Airway pressure and airflow were measured during every application. Breathing pattern, vital capacity (VC), sniff nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), and respiratory comfort were evaluated at baseline and after each application. The tolerance of the patients was excellent, with a significant increase in the respiratory comfort score in all of the patients (p = 0.02). Expired volume during the MI-E application increased significantly to reach twice the VC at 40 cm H(2)O. Mean and maximal inspiratory and expiratory flows increased in a pressure-dependent manner. Breathing pattern did not change after the MI-E applications and pulse oximetric saturation remained stable within normal values, but the mean end-tidal carbon dioxide pressure decreased significantly. VC did not change, but the mean SNIP and PEF improved significantly after MI-E applications.
  
  
  CONCLUSIONS
  Our results confirm the good tolerance and physiologic short-term benefit of the MI-E in children with neuromuscular disease who were in a stable state.
authors: Assistance Publique-Hôpitaux de Paris, Hôpital Armand Trousseau, Pediatric Pulmonary Department, Research unit INSERM UMR-S 719, Université Pierre et Marie Curie-Paris 6, 28 Ave du Docteur Arnold Netter, Paris, F-75012 France. brigitte.fauroux@trs.aphp.fr
authors: Brigitte
authors: B
authors: Fauroux
authors: Nathalie
authors: N
authors: Guillemot
authors: Guillaume
authors: G
authors: Aubertin
authors: Nadia
authors: N
authors: Nathan
authors: Agathe
authors: A
authors: Labit
authors: Annick
authors: A
authors: Clément
authors: Frédéric
authors: F
authors: Lofaso
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003371:Cough; D005260:Female; D006801:Humans; D007327:Insufflation; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000628:therapy; D020388:Muscular Dystrophy, Duchenne / Q000628:therapy; D009468:Neuromuscular Diseases / Q000628:therapy*; D011446:Prospective Studies; D012129:Respiratory Function Tests; D012143:Respiratory Physiological Phenomena