{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:52:18Z","timestamp":1786978338436,"version":"build-2736575974"},"reference-count":30,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T00:00:00Z","timestamp":1775174400000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Children with Cystic Fibrosis (CF) are at risk of fat\u2010soluble vitamin deficiencies, studies have shown Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) therapy to substantially improve nutritional status. This study aimed to investigate the effect of ETI on fat\u2010soluble vitamin levels A, D, and E, for children aged 6\u201316 years with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>\n                      We performed a retrospective pre\u2010post study of children with CF. Data collected included demographics, clinical characteristics, serum Vitamin A, D and E and vitamin supplementation dosages. Seven timepoints were decided for statistical analysis: T1\u2013T3\u2014pre\u2010ETI and T4\u2013T7\u2014at 6 month intervals post\u2010ETI. The significance of changes in vitamin levels and supplementation was assessed using a linear mixed\u2010effects model. A\n                      <jats:italic>p<\/jats:italic>\n                      \u2010value\u2009&lt;\u20090.05 was used to indicate significance.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Fifty\u2010six children met the inclusion criteria, with 262, 257, and 252 complete observations for Vitamins A, D, and E, respectively. Median Vitamin D levels increased following ETI initiation (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). There was no significant increase in median Vitamin A levels however post\u2010ETI there was a significant association between supplementation dose and serum Vitamin A levels (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.008). There were no significant changes in median Vitamin E. Over the timepoints there was an increase in the proportion of patients receiving no vitamin supplementation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This study found following ETI initiation vitamin D levels increased, and the requirement for fat\u2010soluble vitamin supplementation appeared to decrease. Whilst ETI was not associated with increased Vitamin A and E, the burden of supplementation decreased, and Vitamin A supplementation maybe more effective.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71596","type":"journal-article","created":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T07:35:32Z","timestamp":1775201732000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Impact of Elexacaftor\/Tezacaftor\/Ivacaftor on Fat\u2010Soluble Vitamin Status and Supplementation in a Pediatric Cystic Fibrosis Cohort"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-9261-4293","authenticated-orcid":false,"given":"Fern","family":"Kimber","sequence":"first","affiliation":[{"name":"School of Medicine, Queen's Medical Centre University of Nottingham Nottingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7709-0110","authenticated-orcid":false,"given":"Darren","family":"Sills","sequence":"additional","affiliation":[{"name":"School of Medicine, Queen's Medical Centre University of Nottingham Nottingham UK"},{"name":"Nutrition and Dietetics Nottingham University Hospitals NHS Trust, City Hospital Nottingham UK"},{"name":"NIHR Nottingham Biomedical Research Centre, Queen's Medical Centre University of Nottingham Nottingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,4,3]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsbmb.2015.09.013"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ncp.10899"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201306-171BC"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1024\/0300-9831\/a000025"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201306-171BC"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2023.12.017"},{"issue":"7","key":"e_1_2_10_8_1","first-page":"500","article-title":"CFTR Modulators for the Treatment of Cystic Fibrosis","volume":"39","author":"Pettit R. S.","year":"2014","journal-title":"P & T: A Peer\u2010Reviewed Journal for Formulary Management"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_10_11_1","unstructured":"Cystic Fibrosis Trust. UK Cystic Fibrosis Registry 2024 Annual Data Report. 2025;."},{"key":"e_1_2_10_12_1","unstructured":"MHRA. Cystic Fibrosis Drugs Kaftrio and Kalydeco Licensed for Patients Aged Two to Five Years Old [Internet]. GOV.UK. 2023 [cited 2024 Jan 14].https:\/\/www.gov.uk\/government\/news\/cystic-fibrosis-drugs-kaftrio-and-kalydeco-licensed-for-patients-aged-two-to-five-years-old#:~:text=The%20Medicines%20and%20Healthcare%20products two%20to%20five%20years%20old."},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.3390\/children10020252"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2023.1130790"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.08.002"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3390\/antiox10030483"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.04.019"},{"key":"e_1_2_10_18_1","unstructured":"Dietary Supplement Ingredient Database [Internet]. National Institutes of Health Office of Dietary Supplements. [cited 2023 Nov 13].https:\/\/dsid.od.nih.gov\/Conversions.php."},{"key":"e_1_2_10_19_1","unstructured":"Haliborange. 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