{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:37:29Z","timestamp":1787027849357,"version":"build-2736575974"},"reference-count":42,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T00:00:00Z","timestamp":1763942400000},"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: Triple therapy with cystic fibrosis transmembrane conductance regulator (CFTR) modulators in patients with cystic fibrosis (CF) has led to a shift in the nutritional management of the disease. Correct assessment of nutritional status is crucial due to its significant impact in pulmonary function and overall patient survival. This study aims to provide mid-term data on anthropometric and body composition changes in pediatric patients treated with elexacaftor\u2013tezacaftor\u2013ivacaftor (ETI). Methods: A prospective, longitudinal, multicenter study was conducted involving pediatric CF patients receiving ETI therapy. Anthropometric measurements and bioelectrical impedance analysis (BIA) data were collected at baseline and after 24 months of treatment. Results: A group of 66 pediatric patients, median age of 11.11 years (IQR: 8.2\u201312.5), was studied. The BMI z-score increased from \u22120.63 at baseline to \u22120.38 (p &lt; 0.001) after 2 years of ETI treatment. Somatometric parameters were compared with a retrospective cohort showing no significant results. Significant changes comparing body composition were also observed over the 24 months period: fat mass (FM) increased from 6.2 kg to 7.4 kg (p &lt; 0.001) free fat mass (FFM), increased from 28.2 kg to 34.2 kg (p &lt; 0.001) and body cellular mass (BCM), increased from 7.8 kg to 8.7 kg (p = 0.02). Conclusions: Pediatric patients undergoing ETI treatment showed mid-term improvements in anthropometric parameters, alongside notable changes in body composition. Long-term studies are needed to confirm these findings and to better understand the implications for pediatric CF care.<\/jats:p>","DOI":"10.3390\/children12121598","type":"journal-article","created":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T13:09:25Z","timestamp":1763989765000},"page":"1598","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Impact on Body Composition After Two Years of Elexacaftor\u2013Tezacaftor\u2013Ivacaftor Therapy in Children with Cystic Fibrosis"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-4820-2443","authenticated-orcid":false,"given":"Mar\u00eda","family":"\u00c1lvarez Merino","sequence":"first","affiliation":[{"name":"Pediatrics Department, Central University Hospital of Asturias, 330011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6020-8776","authenticated-orcid":false,"given":"Concepci\u00f3n Marina L\u00f3pez","family":"C\u00e1rdenes","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatrics Department, Ram\u00f3n y Cajal University Hospital, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saray Mesonero","family":"Cavia","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Vall d\u2019Hebron University Hospital, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Encarnaci\u00f3n Torcuato","family":"Rubio","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatric Gastroenterology and Nutrition Section, M\u00e1laga Regional University Hospital, 29010 M\u00e1laga, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2221-6868","authenticated-orcid":false,"given":"Alejandro","family":"Rodr\u00edguez-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Virgen del Roc\u00edo University Hospital, 41005 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2082-8983","authenticated-orcid":false,"given":"Saioa Vicente","family":"Santamar\u00eda","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatrics Department, 12 de Octubre University Hospital, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara Vi\u00f1as","family":"Torne","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Vall d\u2019Hebron University Hospital, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina \u00c1lvarez","family":"Beltr\u00e1n","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Vall d\u2019Hebron University Hospital, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celia Gasc\u00f3n","family":"Galindo","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatrics Department, Ram\u00f3n y Cajal University Hospital, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4683-2043","authenticated-orcid":false,"given":"Mar\u00eda","family":"Garriga Garc\u00eda","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatrics Department, Ram\u00f3n y Cajal University Hospital, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Mu\u00f1oz","family":"Alonso","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Virgen del Roc\u00edo University Hospital, 41005 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6273-2600","authenticated-orcid":false,"given":"Mercedes Murray","family":"Hurtado","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Section, Pediatrics Department, Canarias University Hospital, 38320 Santa Cruz de Tenerife, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Sierra San Nicol\u00e1s","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Section, Pediatrics Department, Canarias University Hospital, 38320 Santa Cruz de Tenerife, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Ortiz-P\u00e9rez","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatric Gastroenterology and Nutrition Section, M\u00e1laga Regional University Hospital, 29010 M\u00e1laga, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 Ram\u00f3n","family":"Guti\u00e9rrez Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Central University Hospital of Asturias, 330011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta Su\u00e1rez","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Central University Hospital of Asturias, 330011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8696-9194","authenticated-orcid":false,"given":"David Gonz\u00e1lez","family":"Jim\u00e9nez","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition Unit, Central University Hospital of Asturias, 330011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0962-8403","authenticated-orcid":false,"given":"Juan Jos\u00e9 D\u00edaz","family":"Mart\u00edn","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition Unit, Central University Hospital of Asturias, 330011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1126\/science.2570460","article-title":"Identification of the cystic fibrosis gene: Genetic 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