title: Whistle and cough pressures in children with neuromuscular disorders.
abstract: BACKGROUND
  Expiratory muscle strength is a determinant of cough function. Maximal static expiratory pressure (PEmax) manoeuvres are widely used but are limited by patient motivation and technique. The study hypothesized that whistle mouth (PmW) and cough gastric (PgasCough) pressures might provide additional tests of expiratory muscle strength in children and young adults with neuromuscular disease (NMD).
  
  
  METHODS
  We retrospectively reviewed the data of lung function and respiratory muscle tests of all the patients with NMD followed in our centre between November 2001 and December 2013. PmW and PgasCough were compared to other common tests.
  
  
  RESULTS
  Three hundred and four respiratory evaluations were performed in 143 patients, aged 3-29 years old. Seventy-two patients had 2 to 8 evaluations. Median [interquartiles] PEmax (38 [28-54] cmH2O) did not differ significantly from PgasCough (45 [30-60] cmH2O) and both were significantly greater than PmW (30 [19-44] cmH2O). Significant good correlations were observed between all the expiratory muscle parameters. The best correlation was observed between PEmax and PmW (r = 0.812, p < 0.001). Moreover, good correlations were found between the percentage of predicted forced vital capacity and PmW (r = 0.619, p < 0.001) and PgasCough (r = 0.568, p < 0.001). Concerning the whistle test, the non-invasive measurement highly correlated with invasive measurements.
  
  
  CONCLUSIONS
  PmW and PgasCough are simple and valuable tests to assess expiratory muscle strength in children and young adults with NMD. These tests are particularly useful in children having difficulties to perform PEmax manoeuvre. They have the great advantage of their simplicity, but PgasCough is limited by its invasiveness.
authors.affiliation: AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Aloui
authors.affiliation: AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France; ASV Santé, 125 Avenue Louis Roche, 92230 Gennevilliers, France.
authors.forename: Sonia
authors.initials: S
authors.lastname: Khirani
authors.affiliation: AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France; ADEP ASSISTANCE, 2 rue Benoit Malon, 92150 Suresnes, France.
authors.forename: Adriana
authors.initials: A
authors.lastname: Ramirez
authors.affiliation: Clinica Pediatrica, Istituto G. Gaslini, Genoa, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Colella
authors.affiliation: INSERM U 955, Equipe 13, Université Paris Est, 94000 Créteil, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Louis
authors.affiliation: AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Pediatric Department, Trieste, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Amaddeo
authors.affiliation: AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France; INSERM U 955, Equipe 13, Université Paris Est, 94000 Créteil, France; Paris Descartes University, 75000 Paris, France. Electronic address: brigitte.fauroux@aphp.fr.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003371:Cough / Q000503:physiopathology*; D045853:Exhalation / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D009468:Neuromuscular Diseases / Q000503:physiopathology*; D011312:Pressure; D012129:Respiratory Function Tests; D012132:Respiratory Muscles / Q000503:physiopathology*; D012189:Retrospective Studies; D014797:Vital Capacity; D055815:Young Adult
keywords: Cough; Expiratory muscle strength; Forced vital capacity; Maximal static expiratory pressures; Neuromuscular diseases; Whistle