{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:54:43Z","timestamp":1786978483783,"version":"build-2736575974"},"reference-count":28,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,12,13]],"date-time":"2023-12-13T00:00:00Z","timestamp":1702425600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Iron deficiency is highly prevalent in people with cystic fibrosis (PwCF). While elexacaftor\/tezacaftor\/ivacaftor (ETI) has shown remarkable improvements in respiratory symptoms in PwCF, the effect of ETI on iron status remains unknown. This study aims to identify the effect of ETI on iron status in PwCF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A single\u2010center retrospective cohort study of 127 adult PwCF was conducted to assess the impact of ETI on iron, ferritin, transferrin levels, and percent saturation of transferrin (PSAT). Data were collected from the electronic medical record from January 2017 to September 2022, encompassing 2 years before and after ETI initiation. The primary outcome was serum iron parameters: iron, ferritin, transferrin, and PSAT levels following ETI treatment. Secondary outcomes analyzed iron supplementation. Univariate and multivariate mixed\u2010effects models were used for the analysis of ETI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      After adjusting for covariates, following ETI initiation, the mean iron level increased by 20.24\u2009\u03bcg\/dL (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.001), ferritin levels were 31.4% (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.001) higher, PSAT showed a 5.09 percentage point increase (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.001), and transferrin levels increased by 2.71\u2009mg\/dL (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.439). Patients with and without iron supplementation experienced a significant increase in iron after ETI (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>ETI is associated with a significant increase in iron, ferritin, and PSAT levels. Patients with and without iron supplementation demonstrated a significant increase in iron. This study shows the benefits of ETI on iron status in PwCF. However, further translational studies are required to understand the impact of ETI on iron absorption and metabolism in PwCF.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.26805","type":"journal-article","created":{"date-parts":[[2023,12,13]],"date-time":"2023-12-13T05:48:56Z","timestamp":1702446536000},"page":"669-678","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Analysis of iron status after initiation of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis"],"prefix":"10.1002","volume":"59","author":[{"given":"Amber","family":"James","sequence":"first","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Galvin","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Statistics University of Virginia Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhonda","family":"List","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4285-1260","authenticated-orcid":false,"given":"Kevin","family":"Lonabaugh","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Pulmonology and Critical Care Medicine University of Virginia Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron D.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Barros","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"},{"name":"Department of Medicine, Division of Pulmonology and Critical Care Medicine University of Virginia Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7052-0640","authenticated-orcid":false,"given":"Lindsay","family":"Somerville","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"},{"name":"Department of Medicine, Division of Pulmonology and Critical Care Medicine University of Virginia Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana","family":"Albon","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Virginia School of Medicine Charlottesville Virginia USA"},{"name":"Department of Medicine, Division of Pulmonology and Critical Care Medicine University of Virginia Charlottesville 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