{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T21:13:10Z","timestamp":1770066790192,"version":"3.49.0"},"reference-count":68,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2025,8,22]],"date-time":"2025-08-22T00:00:00Z","timestamp":1755820800000},"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: Cystic fibrosis (CF) is a multisystemic disorder caused by CFTR gene mutations, leading to impaired protein function and affecting pulmonary, gastrointestinal, hepatobiliary, skeletal, and nutritional health. The advent of CFTR modulators\u2014especially the triple therapy elexacaftor\/tezacaftor\/ivacaftor (ETI)\u2014has revolutionized clinical management, offering genotype-specific benefits beyond pulmonary outcomes. Pediatric gastroenterologists must now recognize and address emerging gastrointestinal and nutritional challenges introduced by modulator therapy. Methods: A narrative review was conducted to assess the impact of CFTR modulators on gastrointestinal function, nutritional status, bone health, and hepatobiliary involvement in pediatric patients. A structured literature search was performed using PubMed, EMBASE, and Scopus databases. Filters included articles in English or Spanish. Following full-text review based on relevance and quality, 68 articles were selected for inclusion in this review. Results: CFTR modulators have demonstrated potential improvements in gastrointestinal function, nutrient absorption, weight gain, and bone mineral density. In pediatric populations, ETI therapy has been associated with early increases in lean mass, enhanced vitamin levels, and promising trends in bone microarchitecture. However, variable outcomes regarding liver function and bone mineral density highlight the need for careful monitoring. Conclusions: While CFTR modulators present novel opportunities for systemic improvement in CF, their long-term impact on digestive and skeletal health in children remains under investigation. Pediatric gastroenterologists play a pivotal role in monitoring nutritional and hepatobiliary outcomes, optimizing treatment plans, and guiding personalized care strategies in the era of CFTR modulation.<\/jats:p>","DOI":"10.3390\/children12091104","type":"journal-article","created":{"date-parts":[[2025,8,22]],"date-time":"2025-08-22T11:50:31Z","timestamp":1755863431000},"page":"1104","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Treatment with CFTR Modulators for Cystic Fibrosis: What a Pediatric Gastroenterologist Needs to Know"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8696-9194","authenticated-orcid":false,"given":"David Gonzalez","family":"Jimenez","sequence":"first","affiliation":[{"name":"Cystic Fibrosis Unit, Hospital Universitario Central de Asturias, 33011 Oviedo, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3017-1340","authenticated-orcid":false,"given":"Ruth","family":"Garc\u00eda Romero","sequence":"additional","affiliation":[{"name":"Hospital Infantil Miguel Servet, 50009 Zaragoza, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2221-6868","authenticated-orcid":false,"given":"Alejandro Rodr\u00edguez","family":"Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, UGC de Pediatr\u00eda Hospital Infantil\u2014HH.UU, Virgen del Roc\u00edo Avda, Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saioa","family":"Vicente Santamaria","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Hospital Universitario Ramon y Cajal, Ctra, de Colmenar Viejo km. 9,100, 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.jcf.2017.07.014","article-title":"Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine","volume":"16","author":"Dorsey","year":"2007","journal-title":"J. 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