{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,19]],"date-time":"2026-07-19T02:17:22Z","timestamp":1784427442344,"version":"3.55.0"},"reference-count":29,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,1,16]],"date-time":"2023-01-16T00:00:00Z","timestamp":1673827200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>In Duchenne muscular dystrophy (DMD), lung disease contributes significantly to morbidity and mortality. This study aimed to assess the usefulness of various pulmonary function tests in evaluating DMD severity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study analysed lung function tests of patients with DMD\u2010treated in the multidisciplinary respiratory neuromuscular clinic at Schneiders' Children Medical Center of Israel. Data were analysed according to age, ambulatory status and glucocorticoid treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 90 patients with DMD, 40\/63 (63.5%) ambulatory patients and 22\/27 (81.5%) nonambulatory patients successfully performed spirometry. Significant annual declines were demonstrated among nonambulatory patients, in percentile predicted forced vital capacity (3.8%) and in total lung capacity (5.5%) per year. The decline correlated with age and loss of ambulation but not with steroid treatment. Peak cough flow values were randomly distributed and did not correlate with age, ambulation or treatment. In nonambulatory patients, transcutaneous carbon dioxide measurement correlated significantly with age (<jats:italic>r<\/jats:italic>\u00a0=\u00a00.55, <jats:italic>p<\/jats:italic>\u00a0=\u00a00.02).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Forced vital capacity, total lung capacity and transcutaneous carbon dioxide correlated with the clinical severity of disease in children with DMD. These measures may be useful in follow\u2010up and clinical trials. A comparable correlation was not found for peak cough flow.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.16653","type":"journal-article","created":{"date-parts":[[2023,1,3]],"date-time":"2023-01-03T23:29:33Z","timestamp":1672788573000},"page":"854-860","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Pulmonary function tests for evaluating the severity of Duchenne muscular dystrophy disease"],"prefix":"10.1111","volume":"112","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6772-5784","authenticated-orcid":false,"given":"Hagit","family":"Levine","sequence":"first","affiliation":[{"name":"Pulmonary Institute, Schneider Children's Medical Center  Petah\u2010Tikva Israel"},{"name":"Sackler Faculty of Medicine, Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Itai","family":"Goldfarb","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine, Tel Aviv University  Tel Aviv Israel"},{"name":"Neurology Institute, Schneider Children's Medical Center  Petah\u2010Tikva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Katz","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine, Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moriah","family":"Carmeli","sequence":"additional","affiliation":[{"name":"Pulmonary Institute, Schneider Children's Medical Center  Petah\u2010Tikva Israel"},{"name":"Soroka University Medical School  Beer Sheva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tzippy","family":"Shochat","sequence":"additional","affiliation":[{"name":"Rabin Medical Center  Petah\u2010Tikva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huda","family":"Mussaffi","sequence":"additional","affiliation":[{"name":"Pulmonary Institute, Schneider Children's Medical Center  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