{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T20:23:07Z","timestamp":1792959787780,"version":"4.5.0"},"reference-count":42,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T00:00:00Z","timestamp":1764806400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"},{"start":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T00:00:00Z","timestamp":1764806400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"funder":[{"DOI":"10.13039\/100011022","name":"Vertex Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011022","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pulm Ther"],"published-print":{"date-parts":[[2026,3]]},"DOI":"10.1007\/s41030-025-00334-x","type":"journal-article","created":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T10:19:33Z","timestamp":1764843573000},"page":"265-282","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Real-World Impact of Elexacaftor\/Tezacaftor\/Ivacaftor (ELX\/TEZ\/IVA) in Italy: A Retrospective Study from a Cystic Fibrosis Center"],"prefix":"10.1007","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5652-7248","authenticated-orcid":false,"given":"Marco","family":"Cipolli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2856-752X","authenticated-orcid":false,"given":"Teja","family":"Thorat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0310-3529","authenticated-orcid":false,"given":"Pia C.","family":"Pafundi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9664-0306","authenticated-orcid":false,"given":"Alessandro","family":"Roggeri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4676-4164","authenticated-orcid":false,"given":"Daniela P.","family":"Roggeri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9133-971X","authenticated-orcid":false,"given":"Carlotta","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Pintani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Meneghelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5294-9301","authenticated-orcid":false,"given":"Francesca","family":"Lucca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Fratoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Vega-Hernandez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,12,4]]},"reference":[{"key":"334_CR1","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.jcf.2022.01.009","volume":"21","author":"J Guo","year":"2022","unstructured":"Guo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022;21:456\u201362. https:\/\/doi.org\/10.1016\/j.jcf.2022.01.009.","journal-title":"J Cyst Fibros"},{"key":"334_CR2","doi-asserted-by":"publisher","first-page":"893","DOI":"10.1111\/apa.15155","volume":"109","author":"K De Boeck","year":"2020","unstructured":"De Boeck K. Cystic fibrosis in the year 2020: a disease with a new face. Acta Paediatr. 2020;109:893\u20139. https:\/\/doi.org\/10.1111\/apa.15155.","journal-title":"Acta Paediatr"},{"key":"334_CR3","unstructured":"Cystic Fibrosis Foundation. CF Foundation estimates an increase in CF population. https:\/\/www.cff.org\/news\/2022-07\/cf-foundation-estimates-increase-cf-population. Accessed May 25, 2025."},{"key":"334_CR4","unstructured":"Cystic Fibrosis Foundation. 2023 Patient Registry. https:\/\/www.cff.org\/medical-professionals\/patient-registry. Accessed May 25, 2025."},{"key":"334_CR5","unstructured":"Campagna, G., Amato, A., Majo, F.: Italian Cystic Fibrosis Registry (ICFR). Report 2021\u20132022. https:\/\/registroitalianofibrosicistica.it\/wp-content\/uploads\/2024\/07\/rifc_report2021-2022.pdf. Accessed May 25, 2025."},{"key":"334_CR6","doi-asserted-by":"publisher","first-page":"958","DOI":"10.1513\/AnnalsATS.202209-829OC","volume":"20","author":"R Szczesniak","year":"2023","unstructured":"Szczesniak R, Andrinopoulou ER, Su W. Lung function decline in cystic fibrosis: impact of data availability and modeling strategies on clinical interpretations. Ann Am Thorac Soc. 2023;20:958\u201368. https:\/\/doi.org\/10.1513\/AnnalsATS.202209-829OC.","journal-title":"Ann Am Thorac Soc"},{"key":"334_CR7","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1055\/s-0039-1698464","volume":"40","author":"AC Blanchard","year":"2019","unstructured":"Blanchard AC, Waters VJ. Microbiology of cystic fibrosis airway disease. Semin Respir Crit Care Med. 2019;40:727\u201336. https:\/\/doi.org\/10.1055\/s-0039-1698464.","journal-title":"Semin Respir Crit Care Med"},{"key":"334_CR8","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/j.jcf.2010.12.005","volume":"10","author":"D Salvatore","year":"2011","unstructured":"Salvatore D, Buzzetti R, Baldo E. An overview of international literature from cystic fibrosis registries. Part 3. Disease incidence, genotype\/phenotype correlation, microbiology, pregnancy, clinical complications, lung transplantation, and miscellanea. J Cyst Fibros. 2011;10:71\u201385. https:\/\/doi.org\/10.1016\/j.jcf.2010.12.005.","journal-title":"J Cyst Fibros"},{"key":"334_CR9","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1136\/thx.2006.060889","volume":"62","author":"CH Goss","year":"2007","unstructured":"Goss CH, Burns JL. Exacerbations in cystic fibrosis. 1: Epidemiology and pathogenesis. Thorax. 2007;62:360\u20137. https:\/\/doi.org\/10.1136\/thx.2006.060889.","journal-title":"Thorax"},{"key":"334_CR10","doi-asserted-by":"publisher","first-page":"00449\u20132022","DOI":"10.1183\/23120541.00449-2022","volume":"9","author":"E Hatziagorou","year":"2023","unstructured":"Hatziagorou E, Fieuws S, Orenti A, ECFSPR Collaborative Group. Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry. ERJ Open Res. 2023;9:00449\u20132022. https:\/\/doi.org\/10.1183\/23120541.00449-2022.","journal-title":"ERJ Open Res"},{"issue":"3","key":"334_CR11","doi-asserted-by":"publisher","first-page":"479","DOI":"10.3390\/nu15030479","volume":"15","author":"EM Zani","year":"2023","unstructured":"Zani EM, Grandinetti R, Cunico D. Nutritional care in children with cystic fibrosis. Nutrients. 2023;15(3):479. https:\/\/doi.org\/10.3390\/nu15030479.","journal-title":"Nutrients"},{"key":"334_CR12","doi-asserted-by":"publisher","first-page":"248","DOI":"10.5863\/1551-6776-26.3.248","volume":"26","author":"SL Tjugum","year":"2021","unstructured":"Tjugum SL, Hansen K, McKinzie CJ. Association of medication regimen complexity with clinical endpoints in pediatric patients with cystic fibrosis. J Pediatr Pharmacol Ther. 2021;26:248\u201352. https:\/\/doi.org\/10.5863\/1551-6776-26.3.248.","journal-title":"J Pediatr Pharmacol Ther"},{"issue":"1","key":"334_CR13","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.jcf.2022.10.002","volume":"22","author":"KW Southern","year":"2023","unstructured":"Southern KW, Castellani C, Lammertyn E, et al. Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis. J Cyst Fibros. 2023;22(1):17\u201330.","journal-title":"J Cyst Fibros"},{"key":"334_CR14","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1016\/j.prrv.2020.05.002","volume":"42","author":"IM Balfour-Lynn","year":"2022","unstructured":"Balfour-Lynn IM, King JA. CFTR modulator therapies\u2014effect on life expectancy in people with cystic fibrosis. Paediatr Respir Rev. 2022;42:3\u20138. https:\/\/doi.org\/10.1016\/j.prrv.2020.05.002.","journal-title":"Paediatr Respir Rev"},{"key":"334_CR15","doi-asserted-by":"publisher","first-page":"1463","DOI":"10.1164\/rccm.201009-1478CI","volume":"183","author":"M Cohen-Cymberknoh","year":"2011","unstructured":"Cohen-Cymberknoh M, Shoseyov D, Kerem E. Managing cystic fibrosis. Am J Respir Crit Care Med. 2011;183:1463\u201371. https:\/\/doi.org\/10.1164\/rccm.201009-1478CI.","journal-title":"Am J Respir Crit Care Med"},{"key":"334_CR16","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/j.jcf.2008.09.007","volume":"8","author":"GS Sawicki","year":"2009","unstructured":"Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros. 2009;8:91\u20136. https:\/\/doi.org\/10.1016\/j.jcf.2008.09.007.","journal-title":"J Cyst Fibros"},{"key":"334_CR17","unstructured":"European Medicines Agency (EMA). KAFTRIO (elexacaftor\/tezacaftor\/ivacaftor) summary of product characteristics. First published: August 21, 2020. https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/kaftrio-epar-product-information_en.pdf. Accessed May 25, 2025."},{"key":"334_CR18","unstructured":"Vertex Pharmaceuticals Press Release. Vertex announces new portfolio reimbursement agreement in Italy including KAFTRIO\u00ae, SYMKEVI\u00ae and additional indications of ORKAMBI\u00ae and KALYDECO\u00ae for eligible patients with cystic fibrosis. https:\/\/investors.vrtx.com\/news-releases\/news-release-details\/vertex-announces-new-portfolio-reimbursement-agreement-italy. Accessed May 25, 2025."},{"key":"334_CR19","unstructured":"HealthDesk. Cystic fibrosis: Aifa approves the reimbursement of KAFTRIO for patients between 6 and 11 years of age. https:\/\/www.healthdesk.it\/cronache\/fibrosi-cistica-aifa-approva-rimborsabilit-kaftrio-pazienti-6-11-anni. Accessed May 25, 2025."},{"key":"334_CR20","doi-asserted-by":"publisher","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","volume":"381","author":"P Middleton","year":"2019","unstructured":"Middleton P, Mall M, Drevinek P. Elexacaftor\u2013tezacaftor\u2013ivacaftor for cystic fibrosis with a single phe508del allele. N Engl J Med. 2019;381:1809\u201319. https:\/\/doi.org\/10.1056\/NEJMoa1908639.","journal-title":"N Engl J Med"},{"key":"334_CR21","doi-asserted-by":"publisher","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","volume":"394","author":"H Heijerman","year":"2019","unstructured":"Heijerman H, McKone E, Downey D. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial. Lancet. 2019;394:1940\u20138. https:\/\/doi.org\/10.1016\/S0140-6736(19)32597-8.","journal-title":"Lancet"},{"key":"334_CR22","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1002\/ppul.1950150204","volume":"15","author":"X Wang","year":"1993","unstructured":"Wang X, Dockery DW, Wypij D, Fay ME, Ferris BG Jr. Pulmonary function between 6 and 18 years of age. Pediatr Pulmonol. 1993;15:75\u201388. https:\/\/doi.org\/10.1002\/ppul.1950150204.","journal-title":"Pediatr Pulmonol"},{"key":"334_CR23","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1164\/ajrccm.159.1.9712108","volume":"159","author":"JL Hankinson","year":"1999","unstructured":"Hankinson JL, Odencrantz JR, Fedan KB. Spirometric reference values from a sample of the general U.S. population. Am J Respir Crit Care Med. 1999;159:179\u201387. https:\/\/doi.org\/10.1164\/ajrccm.159.1.9712108.","journal-title":"Am J Respir Crit Care Med"},{"key":"334_CR24","unstructured":"R Core Team. R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna, Austria. https:\/\/www.r-project.org\/ (2023). Accessed December 16, 2024."},{"key":"334_CR25","doi-asserted-by":"publisher","first-page":"100690","DOI":"10.1016\/j.lanepe.2023.100690","volume":"32","author":"S Sutharsan","year":"2023","unstructured":"Sutharsan S, Dillenhoefer S, Welsner M. Impact of elexacaftor\/tezacaftor\/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry. Lancet Reg Health Eur. 2023;32:100690. https:\/\/doi.org\/10.1016\/j.lanepe.2023.100690.","journal-title":"Lancet Reg Health Eur"},{"key":"334_CR26","doi-asserted-by":"publisher","first-page":"S1569\u20131993(25)0","DOI":"10.1016\/j.jcf.2025.04.012","volume":"15","author":"G Vega-Hernandez","year":"2025","unstructured":"Vega-Hernandez G, MacGregor G, Wilfin A. Longitude: an observational study of the long-term effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people aged \u226512 years with cystic fibrosis using data from the United Kingdom Cystic Fibrosis Registry\u20142-year analysis. J Cyst Fibros. 2025;15:S1569-1993(25)01467-5. https:\/\/doi.org\/10.1016\/j.jcf.2025.04.012.","journal-title":"J Cyst Fibros"},{"key":"334_CR27","doi-asserted-by":"crossref","unstructured":"Baxter, C., Vega-Hernandez Ordaz, A.: Hospital healthcare resource utilization in people with CF treatment with elexacaftor\/tezacaftor\/ivacaftor in Portugal: a non-interventional, retrospective study. Poster EE543. Presented at: ISPOR Europe. Barcelona, Spain: November 17\u201320, 2024.","DOI":"10.1016\/j.jval.2024.10.823"},{"key":"334_CR28","doi-asserted-by":"publisher","first-page":"1115","DOI":"10.1093\/cid\/ciac117","volume":"75","author":"A Miller","year":"2022","unstructured":"Miller A, Harris L, Cavanaugh J. The rapid reduction of infection-related visits and antibiotic use among people with cystic fibrosis after starting elexacaftor\/tezacaftor\/ivacaftor. Clin Infect Dis. 2022;75:1115\u201322. https:\/\/doi.org\/10.1093\/cid\/ciac117.","journal-title":"Clin Infect Dis"},{"key":"334_CR29","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1016\/j.jcf.2023.03.002","volume":"22","author":"JK Bower","year":"2023","unstructured":"Bower JK, Volkova N, Ahluwalia N. Real-world safety and effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: interim results of a long-term registry-based study. J Cyst Fibros. 2023;22:730\u20137. https:\/\/doi.org\/10.1016\/j.jcf.2023.03.002.","journal-title":"J Cyst Fibros"},{"key":"334_CR30","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1007\/s41030-023-00241-z","volume":"9","author":"MS Schechter","year":"2023","unstructured":"Schechter MS, Sabater-Anaya N, Oster G. Impact of elexacaftor\/tezacaftor\/ivacaftor on healthcare resource utilization and associated costs among people with cystic fibrosis in the US: a retrospective claims analysis. Pulm Ther. 2023;9:479\u201398. https:\/\/doi.org\/10.1007\/s41030-023-00241-z.","journal-title":"Pulm Ther"},{"key":"334_CR31","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1186\/s12931-024-03089-2","volume":"26","author":"A Gramegna","year":"2025","unstructured":"Gramegna A, Cimino G, Cipolli M. Impact of elexacaftor\/tezacaftor\/ivacaftor (ETI) on the prescription refill rate (PRR) for inhaled medications in people with CF: an Italian multi-center analysis. Respir Res. 2025;26:19. https:\/\/doi.org\/10.1186\/s12931-024-03089-2.","journal-title":"Respir Res"},{"key":"334_CR32","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1016\/j.jcf.2024.11.004","volume":"24","author":"HK R\u00e5ket","year":"2025","unstructured":"R\u00e5ket HK, Jensen CB, Wang JN. Impact of elexacaftor\/tezacaftor\/ivacaftor on utilization of routine therapies in cystic fibrosis: Danish nationwide register study. J Cyst Fibros. 2025;24:105\u201311. https:\/\/doi.org\/10.1016\/j.jcf.2024.11.004.","journal-title":"J Cyst Fibros"},{"key":"334_CR33","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/j.prrv.2016.03.002","volume":"20","author":"SN Harun","year":"2016","unstructured":"Harun SN, Wainwright C, Klein K, Hennig S. A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis. Paediatr Respir Rev. 2016;20:55\u201366. https:\/\/doi.org\/10.1016\/j.prrv.2016.03.002.","journal-title":"Paediatr Respir Rev"},{"key":"334_CR34","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1080\/17476348.2018.1519400","volume":"12","author":"O Breuer","year":"2018","unstructured":"Breuer O, Caudri D, Stick S, Turkovic L. Predicting disease progression in cystic fibrosis. Expert Rev Respir Med. 2018;12:905\u201317. https:\/\/doi.org\/10.1080\/17476348.2018.1519400.","journal-title":"Expert Rev Respir Med"},{"key":"334_CR35","doi-asserted-by":"publisher","first-page":"680","DOI":"10.1136\/thx.2011.161117","volume":"66","author":"K de Boer","year":"2011","unstructured":"de Boer K, Vandemheen KL, Tullis E. Exacerbation frequency and clinical outcomes in adult patients with cystic fibrosis. Thorax. 2011;66:680\u20135. https:\/\/doi.org\/10.1136\/thx.2011.161117.","journal-title":"Thorax"},{"key":"334_CR36","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1183\/09031936.00159111","volume":"40","author":"V Waters","year":"2012","unstructured":"Waters V, Stanojevic S, Atenafu EG. Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis. Eur Respir J. 2012;40:61\u20136. https:\/\/doi.org\/10.1183\/09031936.00159111.","journal-title":"Eur Respir J"},{"key":"334_CR37","doi-asserted-by":"publisher","first-page":"1711","DOI":"10.3390\/jcm13061711","volume":"13","author":"A Vitiello","year":"2024","unstructured":"Vitiello A, Blasi F, Sabbatucci M. The impact of antimicrobial resistance in cystic fibrosis. J Clin Med. 2024;13:1711. https:\/\/doi.org\/10.3390\/jcm13061711.","journal-title":"J Clin Med"},{"key":"334_CR38","doi-asserted-by":"publisher","first-page":"423","DOI":"10.2147\/ceor.S162021","volume":"10","author":"M Hassan","year":"2018","unstructured":"Hassan M, Bonafede MM, Limone BL, Hodgkins P, Sawicki GS. The burden of cystic fibrosis in the Medicaid population. Clinicoecon Outcomes Res. 2018;10:423\u201331. https:\/\/doi.org\/10.2147\/ceor.S162021.","journal-title":"Clinicoecon Outcomes Res"},{"key":"334_CR39","doi-asserted-by":"publisher","first-page":"250","DOI":"10.1016\/j.jcf.2010.04.002","volume":"9","author":"TG Liou","year":"2010","unstructured":"Liou TG, Elkin EP, Pasta DJ. Year-to-year changes in lung function in individuals with cystic fibrosis. J Cyst Fibros. 2010;9:250\u20136. https:\/\/doi.org\/10.1016\/j.jcf.2010.04.002.","journal-title":"J Cyst Fibros"},{"key":"334_CR40","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1016\/j.jcf.2012.03.009","volume":"11","author":"MW Konstan","year":"2012","unstructured":"Konstan MW, Wagener JS, Vandevanter DR. Risk factors for rate of decline in FEV1 in adults with cystic fibrosis. J Cyst Fibros. 2012;11:405\u201311. https:\/\/doi.org\/10.1016\/j.jcf.2012.03.009.","journal-title":"J Cyst Fibros"},{"key":"334_CR41","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1016\/jcf.2024.12.006","volume":"24","author":"E Zemanick","year":"2025","unstructured":"Zemanick E, Ramsey B, Sands D. Sweat chloride reflects CFTR function and correlates with clinical outcomes following CFTR treatment. J Cyst Fibros. 2025;24:246\u201354. https:\/\/doi.org\/10.1016\/jcf.2024.12.006.","journal-title":"J Cyst Fibros"},{"key":"334_CR42","doi-asserted-by":"publisher","DOI":"10.3390\/jcm14124335","author":"F Lucca","year":"2025","unstructured":"Lucca F, Meneghelli I, Tridello G. Reported adverse events in patients with CF receiving treatment with Elexacaftor\/Tezacaftor\/ivacaftor: 5 years observational study. J Clin Med. 2025. https:\/\/doi.org\/10.3390\/jcm14124335.","journal-title":"J Clin Med"}],"container-title":["Pulmonary Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s41030-025-00334-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s41030-025-00334-x","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s41030-025-00334-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T13:31:33Z","timestamp":1773667893000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s41030-025-00334-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,4]]},"references-count":42,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["334"],"URL":"https:\/\/doi.org\/10.1007\/s41030-025-00334-x","relation":{},"ISSN":["2364-1754","2364-1746"],"issn-type":[{"value":"2364-1754","type":"print"},{"value":"2364-1746","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,4]]},"assertion":[{"value":"29 July 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 November 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 December 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Alessandro Roggeri and Daniela Paola Roggeri report consulting fees from Vertex Pharmaceuticals Incorporated. Francesca Lucca reports payment or honoraria for lectures, presentations, speaker bureaus, manuscript writing or educational events and support for attending meetings and\/or travel from Vertex Pharmaceuticals Incorporated. Marco Cipolli reports participation on a Data Safety Monitoring Board or Advisory Board for the International Medical Rare Mutations Advisory Panel, Vertex Pharmaceuticals Incorporated and ITA Medical Rare Mutations Advisory Board, Vertex Pharmaceuticals Incorporated.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study was approved by the Ethics Committee for Clinical Trials of the Province of Verona and Rovigo (Comitato etico Territoriale Area Sud-Ovest Veneto) in April 2023, which also served as the internal review board for this work (protocol approval number 4232CESC). The CF center database is fully compliant with Data Protection legislation, and the study adhered to the International Society of Pharmacoepidemiology (ISPE) Guidelines for Good Pharmacoepidemiology Practice, the Helsinki Declaration of 1964 and its later amendments, and any applicable national laws, guidelines and regulations. All patients provided informed consent to include their de-identified data in the study and publication.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}]}}