{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T05:11:11Z","timestamp":1769836271782,"version":"3.49.0"},"reference-count":49,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T00:00:00Z","timestamp":1766016000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene, leading to multi-system impairment. Sleep respiratory disorders (SRDs) are frequent in individuals with CF\u2014even in those with normal or mildly impaired lung function\u2014and may adversely affect overall health. The triple combination of elexacaftor, tezacaftor, and ivacaftor (ETI) has markedly improved clinical outcomes in CF; however, its long-term impact on SRDs remains unclear. This study aimed to assess the effects of ETI on nocturnal cardiorespiratory parameters in individuals with CF over a two-year period. Thirty-five clinically stable patients aged \u226513 years, eligible for ETI therapy, were enrolled. Nocturnal cardiorespiratory polygraphy and spirometry were performed at baseline (T0), one year (T1), and two years (T2) after ETI initiation. After one year, significant improvements were observed in mean oxygen saturation (mSpO2), time with SpO2 \u2264 90% (t \u2264 90%), and respiratory rate. Spirometric indices (FEV1, FVC, FEF) also significantly increased (p &lt; 0.05). Correlation analysis revealed positive associations between mSpO2 and FEV1 (\u03c1 = 0.515, p = 0.002) and between FEV1 and FVC (\u03c1 = 0.894, p &lt; 0.001), while t \u2264 90% negatively correlated with FEV1 (\u03c1 = \u22120.404, p = 0.016). No additional significant changes were found at T2. ETI therapy resulted in sustained improvements in nocturnal oxygenation and lung function, supporting the importance of nocturnal respiratory monitoring during follow-up.<\/jats:p>","DOI":"10.3390\/life15121942","type":"journal-article","created":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T13:45:07Z","timestamp":1766065507000},"page":"1942","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Long-Term Effects of Elexacaftor\/Tezacaftor\/Ivacaftor on Nocturnal Cardiorespiratory Polygraphy Parameters in Patients with Cystic Fibrosis: A Prospective Study"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4230-6860","authenticated-orcid":false,"given":"Monica","family":"Tosto","sequence":"first","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4291-0195","authenticated-orcid":false,"given":"Giuseppe Fabio","family":"Parisi","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4879-5418","authenticated-orcid":false,"given":"Santiago","family":"Presti","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Papale","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-3381-9844","authenticated-orcid":false,"given":"Giulia","family":"Pecora","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9965-9192","authenticated-orcid":false,"given":"Enza","family":"Mul\u00e8","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vittorio","family":"Ornato","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donatella","family":"Aloisio","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7664-3083","authenticated-orcid":false,"given":"Sara","family":"Manti","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"},{"name":"Department of Human Pathology in Adult and Developmental Age \u201cGaetano Barresi\u201d, University of Messina, Via Consolare Valeria 1, 98124 Messina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2642-6400","authenticated-orcid":false,"given":"Salvatore","family":"Leonardi","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Via Carlo Azeglio Ciampi, 95121 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,12,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1001\/jama.2023.8120","article-title":"Cystic Fibrosis: A 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Care Med."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1942\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T13:56:47Z","timestamp":1766066207000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1942"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,18]]},"references-count":49,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["life15121942"],"URL":"https:\/\/doi.org\/10.3390\/life15121942","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,18]]}}}