{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T14:18:34Z","timestamp":1780582714119,"version":"3.54.1"},"reference-count":28,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2025,7,25]],"date-time":"2025-07-25T00:00:00Z","timestamp":1753401600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Cystic fibrosis (CF) is a multi-system disorder characterized by chronic respiratory failure, malnutrition, and impaired growth. Achieving linear growth above the 50th percentile is associated with better pulmonary outcomes. Since October 2022, Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) has been approved in Italy for children aged \u22656 years. However, data on its impact on height velocity (HV) remain lacking. This study aims to evaluate growth patterns by HV and explore differences according to the CFTR variant genotype. Methods: We conducted a prospective single-center study at the CF Unit of Bambino Ges\u00f9 Children\u2019s Hospital involving 24 children aged 6\u201311 years eligible for ETI treatment. Baseline assessments included height, weight, body mass index (BMI), bone mineral density (BMD), body composition (via bioelectrical impedance analysis, BIA), and muscle strength (one-minute sit-to-stand test (1STST)). Height, weight, HV, and BMI standard deviation scores (SDS) were calculated for the 6 months before and after ETI initiation. Results: The mean age of the cohort was 8.7 \u00b1 1.9 years (F\/M: 12\/12), with most patients na\u00efve to CFTR modulators. A significant increase in HV was observed post-ETI: from 4.2 \u00b1 2.0 cm\/year (\u22121.96 \u00b1 2.4 SDS) in the 6 months before treatment to 7.1 \u00b1 3.0 cm\/year (+1.5 \u00b1 3.7 SDS) after treatment initiation (p &lt; 0.0001). Patients with F508del\/minimal function (F\/MF) genotypes (n = 11) showed significantly greater HV compared to those with F508del\/F508del (F\/F, n = 5) and F508del\/residual function (F\/RF, n = 8) genotypes (p &lt; 0.0001). No significant differences were observed among genetic groups in baseline BMD or lean mass. Conclusions: ETI treatment significantly and rapidly improves HV in children with CF, particularly in those with F\/MF genotypes. These findings underscore the role of CFTR modulator therapy in promoting linear growth, a key indicator of health in pediatric CF populations.<\/jats:p>","DOI":"10.3390\/jcm14155259","type":"journal-article","created":{"date-parts":[[2025,7,25]],"date-time":"2025-07-25T08:13:31Z","timestamp":1753431211000},"page":"5259","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Height Velocity in Pediatric Cystic Fibrosis Under Triple CFTR Modulator Therapy: A Real-Life Monocentric Experience"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8723-7224","authenticated-orcid":false,"given":"Alessandra","family":"Boni","sequence":"first","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-1683-7460","authenticated-orcid":false,"given":"Francesco","family":"d\u2019Aniello","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetology Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grazia","family":"Ubertini","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetology Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5647-7519","authenticated-orcid":false,"given":"Marco","family":"Cappa","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetology Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1493-9767","authenticated-orcid":false,"given":"Fabiana","family":"Ciciriello","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Majo","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7677-6775","authenticated-orcid":false,"given":"Luca","family":"Cristiani","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4333-791X","authenticated-orcid":false,"given":"Federico","family":"Alghisi","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1759-2896","authenticated-orcid":false,"given":"Enza","family":"Montemitro","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergio","family":"Bella","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"De Marchis","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7711-5672","authenticated-orcid":false,"given":"Renato","family":"Cutrera","sequence":"additional","affiliation":[{"name":"Pulmonology and Cystic Fibrosis Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2549-0523","authenticated-orcid":false,"given":"Alessandro G.","family":"Fiocchi","sequence":"additional","affiliation":[{"name":"Allergy Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"Elborn","year":"2016","journal-title":"Lancet"},{"key":"ref_2","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_3","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1016\/j.pcl.2016.04.009","article-title":"Endocrine Disorders in Cystic Fibrosis","volume":"63","author":"Blackman","year":"2016","journal-title":"Pediatr. 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