{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T12:44:29Z","timestamp":1782823469902,"version":"3.54.5"},"reference-count":23,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T00:00:00Z","timestamp":1782691200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Alveolus Biomedical BV"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: Young children with Cystic Fibrosis (CwCF) are at risk of fat-soluble vitamin (FSV) deficiencies due to pancreatic insufficiency, despite pancreatic enzyme supplementation. CFTR modulator therapy such as elexacaftor\/tezacaftor\/ivacaftor (ETI) may improve pancreatic function, but its impact on FSV levels in this age group remains unclear. We evaluated changes in FSV serum levels in a cohort transitioning to ETI, taking a CF-specific, multivitamin formulation with beta-carotene as the primary source of vitamin A (DEKAs Plus Liquid, DPL). Methods: Retrospective data with paired analysis from 23 CwCF (2\u20135 years old) were analyzed. Serum FSV levels (D, E, A) and fecal elastase-1 (FE-1) were measured at baseline and 12 months post-ETI initiation. Daily FSV doses and covariates (FE-1, naive of CFTR modulators and ETI dose) were documented. Statistical analyses included Wilcoxon signed-rank tests and general linear models. Results: No significant changes were observed in serum FSV levels, while FSV dosing and formulation remained unchanged. Covariates did not influence serum level changes. Interestingly, ETI significantly improved FE-1 (p = 0.018), with 6\/18 children achieving pancreatic sufficiency (FE-1 &gt; 200 \u00b5g\/g). Conclusions: In young CwCF initiating ETI, FSV levels were unchanged while pancreatic function improved. The use of provitamin A (beta-carotene) as in DPL resulted in no change in vitamin A levels and aligns with consensus guidance to prioritize provitamin A in ETI-treated children. Further validation in larger cohorts is warranted.<\/jats:p>","DOI":"10.3390\/children13070868","type":"journal-article","created":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T10:01:17Z","timestamp":1782813677000},"page":"868","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Impact of Elexacaftor\/Tezacaftor\/Ivacaftor on Fat-Soluble Vitamin Status in 2 to 5 Year-Old Children Using a Cystic Fibrosis-Specific Multivitamin Formulation"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2331-278X","authenticated-orcid":false,"given":"Anne","family":"Munck","sequence":"first","affiliation":[{"name":"CF Center, Hospital Necker Enfants Malades, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4646-018X","authenticated-orcid":false,"given":"Jeanne","family":"Languepin","sequence":"additional","affiliation":[{"name":"CHU Dupuytren, CF Center, 87000 Limoges, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9493-5218","authenticated-orcid":false,"given":"Raphael","family":"Enaud","sequence":"additional","affiliation":[{"name":"CF Center, CIC, Hospital Pellegrin-Enfants, 33076 Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederique","family":"Chedevergne","sequence":"additional","affiliation":[{"name":"CF Center, Hospital Necker Enfants Malades, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Wizla","sequence":"additional","affiliation":[{"name":"Paediatric CF Center, University Hospital Lille, 59037 Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4849-9034","authenticated-orcid":false,"given":"Natascha","family":"Remus","sequence":"additional","affiliation":[{"name":"Center on Rare Respiratory Diseases, Intercommunal Hospital, 94010 Creteil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2385-495X","authenticated-orcid":false,"given":"Marie","family":"Mittaine","sequence":"additional","affiliation":[{"name":"Paediatric CF Center, University Hospital des Enfants, 31059 Toulouse, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3576-2518","authenticated-orcid":false,"given":"Stephanie","family":"Bui","sequence":"additional","affiliation":[{"name":"Paediatric CF Center, Hospital Pellegrin, 33076 Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelie","family":"Arrouy","sequence":"additional","affiliation":[{"name":"CIC, University Hospital des Enfants, 31059 Toulouse, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1032-4052","authenticated-orcid":false,"given":"Megan","family":"Quinn","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Epidemiology, College of Public Health, East Tennessee State University, Johnson City, TN 37614, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6134-0573","authenticated-orcid":false,"given":"Amy","family":"Wahlquist","sequence":"additional","affiliation":[{"name":"Center for Rural Health and Research, Department of Biostatistics and Epidemiology, College of Public Health, East Tennessee State University, Johnson City, TN 37614, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Sermet-Gaudelus","sequence":"additional","affiliation":[{"name":"University Paris Cite, Hospital Necker-Enfants Malades, CF Center, INSERM U1151, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.clnu.2023.12.017","article-title":"ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis","volume":"43","author":"Wilschanski","year":"2024","journal-title":"Clin. 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