{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T05:42:44Z","timestamp":1777527764056,"version":"3.51.4"},"reference-count":22,"publisher":"SAGE Publications","issue":"9","license":[{"start":{"date-parts":[[2025,4,12]],"date-time":"2025-04-12T00:00:00Z","timestamp":1744416000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,4,12]],"date-time":"2025-04-12T00:00:00Z","timestamp":1744416000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Clin Pediatr (Phila)"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:p>In 2020, Kaftrio, a combination of Elexacaftor, Tezacaftor, and Ivacaftor, gained approval for treating cystic fibrosis (CF) in patients from the age of 12 years. This study aims to analyze 1 year of treatment with Kaftrio in pediatric patients, comparing their clinical characteristics with pre-treatment data. This is an observational, descriptive, and longitudinal study in patients with CF older than 12 years with at least 1 F508del mutation treated with Kaftrio for 1 year. Forced expiratory volume in 1 second (FEV1) z-score increased by +1.1 (95% confidence interval [CI] = 0.55 to 1.64), forced vital capacity (FVC) by +0.56 (95% CI = 0.10 to 1.04), and maximal mid-expiratory flow (MMEF) 25\/75 improved by +1.53 (95% CI = 0.59 to 2.47). In addition, a reduction of 25.50 points (95% CI = \u221237.95 to \u221213.06) in sweat chloride levels was observed. Body mass index (BMI)-for-age z-score (WHO 2006\/2007) increased +0.39 (95% CI = 0.02 to 0.77). A transient increase in cough and secretions was noted in 61.53% after starting treatment. Kaftrio improves lung function and BMI and also reduces respiratory exacerbations and sweat chloride levels.<\/jats:p>","DOI":"10.1177\/00099228251330121","type":"journal-article","created":{"date-parts":[[2025,4,12]],"date-time":"2025-04-12T05:46:36Z","timestamp":1744436796000},"page":"1289-1295","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Real-Life in Cystic Fibrosis Pediatric Patients Treated With Kaftrio: A Descriptive Observational Study"],"prefix":"10.1177","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2513-1360","authenticated-orcid":false,"given":"Francisco Jos\u00e9","family":"Garc\u00eda D\u00edaz","sequence":"first","affiliation":[{"name":"Servicio de Pediatr\u00eda, Hospital Universitario Virgen del Roc\u00edo, Sevilla, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda","family":"Moreno 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