Sabrina Aloui1, Sonia Khirani2, Adriana Ramirez3, Marina Colella4, Bruno Louis5, Alessandro Amaddeo6, Brigitte Fauroux7. 1. AP-HP, Hôpital Necker Enfants-Malades, Pediatric Noninvasive Ventilation and Sleep Unit, 149 rue de Sèvres, 75015 Paris, France. 2. 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. 3. 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. 4. Clinica Pediatrica, Istituto G. Gaslini, Genoa, Italy. 5. INSERM U 955, Equipe 13, Université Paris Est, 94000 Créteil, France. 6. 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. 7. 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.
Abstract
RATIONALE: 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.
RATIONALE: 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.