{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:37:57Z","timestamp":1787027877629,"version":"build-2736575974"},"reference-count":55,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T00:00:00Z","timestamp":1675036800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: Improved therapy in CF has led to an overall improvement in nutritional status. The objectives of our study are: to cross-sectionally assess nutritional status and serum levels of fat-soluble vitamins; to retrospectively evaluate the efficacy of modulators on nutritional status and fat-soluble vitamin levels. Methods: In patients younger than 2 years of age, we evaluated growth, in patients aged 2\u201318 years, we assessed BMI z-scores, and in adults, we assessed absolute BMI values. Levels of 25(OH)D, vitamins A, and E were measured. Results: A cross-sectional analysis was conducted on 318 patients, 109 (34.3%) with pancreatic sufficiency. Only three patients were under 2 years old. In 135 patients aged 2\u201318 years, the median BMI z-score was 0.11, and 5 (3.7%) patients had malnutrition (z-score \u2264 2SD). In 180 adults, the median BMI was 21.8 kg\/m2. Overall, 15 (13.7%) males (M) and 18 (25.3%) females (F) were underweight (18 &lt; BMI &gt; 20); 3 (2.7%) M and 5 (7.0%) F had a BMI &lt; 18. Suboptimal 25(OH)D levels were found in patients with pancreatic insufficiency. The prevalence of deficiency of vitamins A and E is low. After one year of treatment with modulators, the increase in BMI was more consistent (M: 1.58 \u00b1 1.25 kg\/m2 F: 1.77 \u00b1 1.21 kg\/m2) in elexacaftor\/tezacaftor\/ivacaftor (ETI)-treated patients compared with other modulators, with a significant increase in levels of all fat-soluble vitamins. Conclusions: Malnutrition is present in a limited number of subjects. The prevalence of subjects with suboptimal 25(OH)D levels is high. ETI showed a beneficial effect on nutritional status and circulating levels of fat-soluble vitamins.<\/jats:p>","DOI":"10.3390\/children10020252","type":"journal-article","created":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T07:34:41Z","timestamp":1675064081000},"page":"252","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5277-4284","authenticated-orcid":false,"given":"Michela","family":"Francalanci","sequence":"first","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1106-4424","authenticated-orcid":false,"given":"Vito","family":"Terlizzi","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3642-8928","authenticated-orcid":false,"given":"Cristina","family":"Fevola","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Di Rosa","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Pierattini","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Professional Dietetics, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Roselli","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Professional Dietetics, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Bonomi","sequence":"additional","affiliation":[{"name":"Freelance Statistician, 20146 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Chiara","family":"Cavicchi","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Galici","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Silvia","family":"Neri","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Bianchimani","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Campana","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Dolce","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Novella","family":"Ravenni","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erica","family":"Camera","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3724-7501","authenticated-orcid":false,"given":"Tommaso","family":"Orioli","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9448-5381","authenticated-orcid":false,"given":"Giovanni","family":"Taccetti","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic 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Fibros."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/10\/2\/252\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:19:57Z","timestamp":1760120397000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/10\/2\/252"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,30]]},"references-count":55,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2023,2]]}},"alternative-id":["children10020252"],"URL":"https:\/\/doi.org\/10.3390\/children10020252","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,30]]}}}