{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T05:26:48Z","timestamp":1793424408687,"version":"4.5.0"},"reference-count":31,"publisher":"European Respiratory Society (ERS)","issue":"2","license":[{"start":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:00:00Z","timestamp":1759968000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2025,9,2]]},"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>While studies have consistently reported improvements in lung function during elexacaftor\/tezacaftor\/ivacaftor (ETI) therapy for people with cystic fibrosis (pwCF), individual response predictors remain poorly understood and there is limited real-world data relating to children aged 6\u201311 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Lung function data from the German CF Registry 2020\u20132024 were analysed before and up to 24 months after ETI therapy initiation in adults, adolescents and children aged \u22656 years. Data were stratified for percent predicted forced expiratory volume in 1\u2005s (ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      ) increase during therapy (&lt;5%\n                      <jats:italic>versus<\/jats:italic>\n                      &gt;5%), underlying mutation and pre-treatment disease severity.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Findings<\/jats:title>\n                    <jats:p>\n                      Data from 2375 pwCF were analysed. After starting ETI, ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      increased most in adolescents with baseline ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      &gt;40\u201360% (median increase 16.8\u201320.2%); the corresponding increase in adults with the same initial lung function was 11.2\u201311.5%. After an initial increase, ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      remained stable for 24 months in all age groups. No predictors for greater improvement (&gt;5% ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      gain) were identified but pwCF with normal ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      before ETI therapy had smaller increases during treatment. Forced mid-expiratory flow at 25\u201375% of forced vital capacity increased to a similar extent in children and adolescents (+13%) and adults (+9.5%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>\n                      Our real-world data showed significant improvements in lung function, including large and small airways, during 24 months\u2019 ETI therapy in pwCF aged \u22656 years. Notable improvements, particularly in small airways, were observed even in children and adolescents with normal ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      before ETI. These findings underscore the importance of early ETI therapy initiation to prevent irreversible lung damage.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1183\/23120541.00813-2025","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T16:05:28Z","timestamp":1760025928000},"page":"00813-2025","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Long-term impact of elexacaftor\/tezacaftor\/ivacaftor on small and large airways in people with cystic fibrosis aged \u22656 years: 24-month real-world evidence from the German Cystic Fibrosis Registry"],"prefix":"10.1183","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6020-8717","authenticated-orcid":false,"given":"Stefanie","family":"Dillenh\u00f6fer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2680-3541","authenticated-orcid":false,"given":"Katharina","family":"Sch\u00fctz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Burkhart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Ellemunter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kappler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Sieber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lutz","family":"Naehrlich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Folke","family":"Brinkmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sivagurunathan","family":"Sutharsan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2025,10,9]]},"reference":[{"key":"2026071300351395000_12.2.00813-2025.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2100665"},{"key":"2026071300351395000_12.2.00813-2025.2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202008-3176LE"},{"key":"2026071300351395000_12.2.00813-2025.3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202202-0392OC"},{"key":"2026071300351395000_12.2.00813-2025.4","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202108-1986OC"},{"key":"2026071300351395000_12.2.00813-2025.5","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2023.1171544"},{"key":"2026071300351395000_12.2.00813-2025.6","doi-asserted-by":"crossref","first-page":"100690","DOI":"10.1016\/j.lanepe.2023.100690","article-title":"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","volume":"32","author":"Sutharsan","year":"2023","journal-title":"Lancet Reg Health Eur"},{"key":"2026071300351395000_12.2.00813-2025.7","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202301-0021OC"},{"key":"2026071300351395000_12.2.00813-2025.8","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A phase 3 open-label study of elexacaftor\/tezacaftor\/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele","volume":"203","author":"Zemanick","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026071300351395000_12.2.00813-2025.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.03.002"},{"key":"2026071300351395000_12.2.00813-2025.10","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02029-2022"},{"key":"2026071300351395000_12.2.00813-2025.11","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202201-0219OC"},{"key":"2026071300351395000_12.2.00813-2025.12","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"2026071300351395000_12.2.00813-2025.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.04.013"},{"key":"2026071300351395000_12.2.00813-2025.14","unstructured":"N\u00e4hrllich L , Burkhart M , Wosniok J . German Cystic Fibrosis Registry annual report 2022. Date last accessed: 10 December 2024. Date last updated: 30 May 2023. www.muko.info\/fileadmin\/user_upload\/was_wir_tun\/register\/berichtsbaende\/annual_report_2022.pdf"},{"key":"2026071300351395000_12.2.00813-2025.15","doi-asserted-by":"publisher","DOI":"10.1002\/pds.70076"},{"key":"2026071300351395000_12.2.00813-2025.16","unstructured":"Neuhauser H , Schienkiewitz A , Rosario AS , et al. Referenzperzentile f\u00fcr anthropometrische Ma\u00dfzahlen und Blutdruck aus der Studie zur Gesundheit von Kindern und Jugendlichen in Deutschland (KiGGS). Berlin, Robert Koch-Institut, 2013. https:\/\/edoc.rki.de\/bitstream\/handle\/176904\/3254\/28jWMa04ZjppM.pdf"},{"key":"2026071300351395000_12.2.00813-2025.17","first-page":"S79","volume-title":"J Cyst Fibros","volume":"10","author":"Bilton","year":"2011"},{"key":"2026071300351395000_12.2.00813-2025.18","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.jcf.2020.04.008","article-title":"Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: data from the UK CF registry","volume":"20","author":"Caley","year":"2021","journal-title":"J Cyst Fibros"},{"key":"2026071300351395000_12.2.00813-2025.19","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22946"},{"key":"2026071300351395000_12.2.00813-2025.20","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.03.001"},{"key":"2026071300351395000_12.2.00813-2025.21","doi-asserted-by":"publisher","DOI":"10.1186\/s41687-022-00466-7"},{"key":"2026071300351395000_12.2.00813-2025.22","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"2026071300351395000_12.2.00813-2025.23","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"2026071300351395000_12.2.00813-2025.24","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"2026071300351395000_12.2.00813-2025.25","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1080\/17476348.2023.2179989","article-title":"Managing cystic fibrosis in children aged 6\u201311yrs: a critical review of elexacaftor\/tezacaftor\/ivacaftor combination therapy","volume":"17","author":"Jordan","year":"2023","journal-title":"Expert Rev Respir Med"},{"key":"2026071300351395000_12.2.00813-2025.26","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1164\/rccm.202102-0278OC","article-title":"Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis","volume":"204","author":"Stahl","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026071300351395000_12.2.00813-2025.27","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01008-2023"},{"key":"2026071300351395000_12.2.00813-2025.28","doi-asserted-by":"crossref","first-page":"2792","DOI":"10.1002\/ppul.27125","article-title":"Effectiveness and safety of elexacaftor\/tezacaftor\/ivacaftor treatment in children aged 6\u201311 years with cystic fibrosis in a real-world setting","volume":"59","author":"Dacc\u00f2","year":"2024","journal-title":"Pediatr Pulmonol"},{"key":"2026071300351395000_12.2.00813-2025.29","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1164\/rccm.202308-1317OC","article-title":"Improvement in lung clearance index and chest computed tomography scores with elexacaftor\/tezacaftor\/ivacaftor treatment in people with cystic fibrosis aged 12 years and older \u2013 the RECOVER trial","volume":"208","author":"McNally","year":"2023","journal-title":"Am J Respir Crit Care Med"},{"key":"2026071300351395000_12.2.00813-2025.30","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.03.002"},{"key":"2026071300351395000_12.2.00813-2025.31","doi-asserted-by":"crossref","first-page":"20406223221108627","DOI":"10.1177\/20406223221108627","article-title":"Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?","volume":"13","author":"Niedermayr","year":"2022","journal-title":"Ther Adv Chronic Dis"}],"updated-by":[{"DOI":"10.1183\/23120541.50813-2025","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T00:00:00Z","timestamp":1783900800000}}],"container-title":["ERJ Open Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/23120541.00813-2025","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T07:35:24Z","timestamp":1783928124000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/23120541.00813-2025"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,9]]},"references-count":31,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,3,16]]},"published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1183\/23120541.00813-2025"],"URL":"https:\/\/doi.org\/10.1183\/23120541.00813-2025","relation":{},"ISSN":["2312-0541"],"issn-type":[{"value":"2312-0541","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,9]]}}}