{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T04:06:29Z","timestamp":1772165189106,"version":"3.50.1"},"reference-count":28,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Fondation Respirer"},{"name":"Cystic Fibrosis Switzerland"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Respir Dis"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>There are currently no early parameters that allow prediction of long-term responses to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator treatment on an individual level.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>To identify early parameters measured within 7 to 14\u2009days after initiation of treatment with a CFTR modulator to assess CFTR modulator efficacy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Study design:<\/jats:title>\n                    <jats:p>Prospective observational study of patients diagnosed with CF who begin elexacaftor\/tezacaftor\/ivacaftor (ETI) therapy at 3 CF clinics in Switzerland (Geneva, Lausanne, Lucerne).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Standardized measurements were taken within 2\u2009months prior to and 7 to 14\u2009days after starting CFTR modulator treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      ETI treatment was started on 47 patients [median age: 12\u2009years] of whom 12 (26%) were switching from lumacaftor\/ivacaftor (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20098) or tezacaftor\/ivacaftor (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094) to ETI. A significant early treatment effect was observed for BMI\n                      <jats:italic>z<\/jats:italic>\n                      -score (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) and inflammatory parameters (white blood cells (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.006), neutrophils (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.006), immunoglobulin G (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.012), and fecal calprotectin (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.002)). In CFTR functional assays, sweat chloride concentration and nasal potential difference testing [\u0394low-chloride+isoproterenol, Sermet score, and Wilschanski index] improved significantly (all\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Improvement was also observed in lung function (FVC, FEV\n                      <jats:sub>1<\/jats:sub>\n                      , MMEF\n                      <jats:sub>25-75<\/jats:sub>\n                      , LCI\n                      <jats:sub>2.5%<\/jats:sub>\n                      ) (all\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). No changes were found for blood pressure, SpO\n                      <jats:sub>2<\/jats:sub>\n                      , respiratory rate, erythrocyte sedimentation rate, C-reactive protein, and fecal elastase.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>This study identified clinical, biologic, and functional parameters showing treatment effect early after initiation of CFTR modulator therapy. These parameters may serve as potential predictors of long-term responses to CFTR modulator treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/17534666251376211","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T11:54:21Z","timestamp":1760010861000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Identification of early changes in multiple biomarkers following CFTR modulator initiation in patients with cystic fibrosis"],"prefix":"10.1177","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-3491-6363","authenticated-orcid":false,"given":"Pascal","family":"Heer","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Children\u2019s Hospital of Central Switzerland, Lucerne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clara","family":"Fernandez Elviro","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Division of Pediatrics, Department Woman-Mother-Child, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1227-1679","authenticated-orcid":false,"given":"Angela","family":"Koutsokera","sequence":"additional","affiliation":[{"name":"Adult Cystic Fibrosis and CFTR-related disorders Center, Lung Transplant Center, Division of Pulmonology, Lausanne University Hospital and University of Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9398-5723","authenticated-orcid":false,"given":"Anne","family":"Mornand","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, University Hospital of Geneva, Geneva, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabelle","family":"Rochat","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Division of Pediatrics, Department Woman-Mother-Child, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6849-6469","authenticated-orcid":false,"given":"Nicolas","family":"Regamey","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Children\u2019s Hospital of Central Switzerland, Lucerne, Switzerland"},{"name":"Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0284-9705","authenticated-orcid":false,"given":"Sylvain","family":"Blanchon","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Division of Pediatrics, Department Woman-Mother-Child, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2025,10,9]]},"reference":[{"issue":"6","key":"e_1_3_4_2_2","article-title":"Use of elexacaftor+tezacaftor+ivacaftor in individuals with cystic fibrosis and at least one F508del allele: a systematic review and meta-analysis","volume":"49","author":"Silva Filho L","year":"2024","unstructured":"Silva Filho L, Athanazio RA, Tonon CR, et al. 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