{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T02:14:42Z","timestamp":1740104082877,"version":"3.37.3"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,1,14]],"date-time":"2020-01-14T00:00:00Z","timestamp":1578960000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Lung clearance index (LCI<jats:sub>2.5<\/jats:sub>) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple\u2010breath washout test and symptoms\u2010limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross\u2010sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventy\u2010seven patients (38 females) were included. S<jats:sub>acin<\/jats:sub> and LCI<jats:sub>2.5<\/jats:sub> were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (<jats:italic>b<\/jats:italic>\u2009=\u20095.25; 95% confidence interval [CI]\u2009=\u20091.53\u20108.98), forced expiratory volume in 1 second (FEV<jats:sub>1<\/jats:sub>; <jats:italic>b<\/jats:italic>\u2009=\u20093.71; 95% CI\u2009=\u20091.96\u20105.46), <jats:italic>Pseudomonas aeruginosa<\/jats:italic> chronic infection (<jats:italic>b<\/jats:italic>\u2009=\u2009\u22128.84; 95% CI\u2009=\u2009\u221215.84 to \u22121.84) but not with LCI<jats:sub>2.5<\/jats:sub>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI<jats:sub>2.5<\/jats:sub> over FEV<jats:sub>1<\/jats:sub> and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24525","type":"journal-article","created":{"date-parts":[[2020,1,14]],"date-time":"2020-01-14T13:52:33Z","timestamp":1579009953000},"page":"394-400","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross\u2010sectional study"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6225-2989","authenticated-orcid":false,"given":"Simone","family":"Gambazza","sequence":"first","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Cystic Fibrosis Centre Milan Italy"},{"name":"Department of Clinical Sciences and Community Health Universit\u00e0 degli Studi di Milano Milan Italy"},{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico U.O.C. Direzione delle Professioni Sanitarie Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riccardo","family":"Guarise","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Cystic Fibrosis Centre Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Carta","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Cystic Fibrosis Centre Milan Italy"},{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico U.O.C. Direzione delle Professioni Sanitarie Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9358-011X","authenticated-orcid":false,"given":"Federico","family":"Ambrogi","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences and Community Health Universit\u00e0 degli Studi di Milano Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina","family":"Mirabella","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico U.O.C. Direzione delle Professioni Sanitarie Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Brivio","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Cystic Fibrosis Centre Milan Italy"},{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico U.O.C. 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