{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:53:25Z","timestamp":1790394805180,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,7,21]],"date-time":"2019-07-21T00:00:00Z","timestamp":1563667200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Respiratory Department, CHQ"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2019,11]]},"abstract":"Abstract<\/jats:title>Background<\/jats:title>Spinal muscular atrophy (SMA) causes progressive respiratory muscle weakness but respiratory function (RF) in those using noninvasive ventilation (NIV) is not well described.<\/jats:p><\/jats:sec>Objective<\/jats:title>To describe RF in childhood SMA and assess differences between those using and not using NIV.<\/jats:p><\/jats:sec>Methods<\/jats:title>A cross\u2010sectional study of childhood SMA assessed polysomnography (PSG), spirometry, forced oscillation technique (FOT), lung clearance index (LCI), sniff nasal inspiratory pressures, peak cough flow, maximal inspiratory and expiratory pressure, and NIV use and indication.<\/jats:p><\/jats:sec>Results<\/jats:title>Twenty\u2010five children (median age [interquartile range], 8.96 [5.63] years; 10 F) with SMA 1 (n<\/jats:italic>\u2009=\u20093), 2 (n<\/jats:italic>\u2009=\u200915), and 3 (n<\/jats:italic>\u2009=\u20097) were recruited. Spirometry and FOT testing was feasible in children as young as 3 years. Ten (40%) required NIV, 5 for sleep\u2010disordered breathing (SDB), and 5 initiated during lower respiratory tract infection (LRTI). Children requiring NIV were older (median, 10.52 vs 5.67 years; P<\/jats:italic>\u2009<\u2009.02) with more abnormal forced vital capacity (FVC) z<\/jats:italic>\u2010score (\u22125.70 vs \u22121.39, P<\/jats:italic>\u2009<\u2009.02),\u00a0Rsr8 z<\/jats:italic>\u2010score (1.97 vs 0.50, P<\/jats:italic>\u2009=\u2009.04), and LCI (8.84 vs 7.34, P<\/jats:italic>\u2009=\u2009.01). Two had normal RF and SDB. For FVC z<\/jats:italic>\u2010score less than \u22122.5 and LCI greater than 7.5, the odds ratio for NIV was 10.70 (95% confidence interval [CI], 1.39\u201082.03) and 2 (95% CI, 0.40\u201010.31), respectively. All children with LCI greater than 8 used NIV. FVC z<\/jats:italic>\u2010score and LCI are associated with maximum transcutaneous carbon dioxide on PSG (r<\/jats:italic>\u2009=\u20090.43, P<\/jats:italic>\u2009<\u2009.001).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>NIV is common in SMA. Normal RF does not exclude SDB. Children with more abnormal FVC and LCI should be considered at risk of starting NIV during\/following an LRTI.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24455","type":"journal-article","created":{"date-parts":[[2019,7,22]],"date-time":"2019-07-22T05:29:49Z","timestamp":1563773389000},"page":"1774-1780","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Relationship between respiratory function and need for NIV in childhood SMA"],"prefix":"10.1002","volume":"54","author":[{"given":"Nitin","family":"Kapur","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine Children's Health Queensland South Brisbane Australia"},{"name":"Centre for Children's Health Research Brisbane Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean","family":"Deegan","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Children's Health Queensland South Brisbane Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ankit","family":"Parakh","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Children's Health Queensland South Brisbane Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0819-894X","authenticated-orcid":false,"given":"Leanne","family":"Gauld","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Children's Health Queensland South Brisbane Australia"},{"name":"Centre for Children's Health Research Brisbane 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