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We aimed to test the hypothesis that clinical and anthropometric parameters would determine fat-free mass impairment in adolescents with CF. We measured the fat-free mass index (FFMI) using bioelectrical impedance, the lung function using spirometry, the number of shuttles as a measure of exercise tolerance and the reported physical activity in children and young people with CF in a tertiary centre at King\u2019s College Hospital, London, UK. CF-related liver disease was diagnosed by abnormal liver enzymes and\/or ultrasonography. We studied 28 children and young people (11 male) with a median (interquartile range (IQR)) age of 15 (13\u201317) years. They had a median (IQR) FFMI of 13.5 (11.6\u201315.1) kg\/m<jats:sup>2<\/jats:sup>. The FFMI significantly correlated with age (rho\u2009=\u20090.568,<jats:italic>p<\/jats:italic>\u2009=\u20090.002), number of shuttles (rho\u2009=\u20090.691,<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) and reported hours of activity per day (rho\u2009=\u20090.426,<jats:italic>p<\/jats:italic>\u2009=\u20090.024). The median (IQR) FFMI was significantly higher in male [15.1 (13.1\u201318.6) kg\/m<jats:sup>2<\/jats:sup>] compared to female participants [12.7 (11.6\u201314.1) kg\/m<jats:sup>2<\/jats:sup>,<jats:italic>p<\/jats:italic>\u2009=\u20090.008]. The median (IQR) FFMI was significantly lower in the\u00a010 (36%) participants with liver disease [11.9 (11.5\u201313.4) kg\/m<jats:sup>2<\/jats:sup>] compared to the FFMI in the remaining 18 participants without liver disease [14.4 (12.5\u201315.9) kg\/m<jats:sup>2<\/jats:sup>,<jats:italic>p<\/jats:italic>\u2009=\u20090.027].<\/jats:p><jats:p><jats:italic>Conclusion<\/jats:italic>: Fat-free mass increases with increasing age and growth in adolescents with CF. Physical activity exerts a beneficial effect on fat-free mass, and CF-related liver disease negatively affects fat-free mass in adolescents with CF.<jats:table-wrap><jats:table><jats:tbody><jats:tr><jats:td colspan=\"2\"><jats:bold>What is Known:<\/jats:bold><jats:italic>\u2022\u00a0Health behaviours in adolescence influence lifelong health in cystic fibrosis (CF).<\/jats:italic><jats:italic>\u2022 A normal body mass index in CF might fail to reveal a low fat-free mass (FFM), and quality of life in CF is strongly associated with a reduced FFM.<\/jats:italic><\/jats:td><\/jats:tr><jats:tr><jats:td colspan=\"2\"><jats:bold>What is New:<\/jats:bold><jats:italic>\u2022\u00a0FFM increases with increasing age and growth in adolescents with CF.<\/jats:italic><jats:italic>\u2022 Physical activity exerts a beneficial effect, and liver disease negatively affects FFM in adolescents with CF.<\/jats:italic><\/jats:td><\/jats:tr><\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:p>","DOI":"10.1007\/s00431-022-04752-w","type":"journal-article","created":{"date-parts":[[2022,12,7]],"date-time":"2022-12-07T07:03:22Z","timestamp":1670396602000},"page":"769-775","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis"],"prefix":"10.1007","volume":"182","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5258-5301","authenticated-orcid":false,"given":"Theodore","family":"Dassios","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Rafaela","family":"Mitakidou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anil","family":"Dhawan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niovi","family":"Papalexopoulou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atul","family":"Gupta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Greenough","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,12,7]]},"reference":[{"key":"4752_CR1","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1055\/s-0039-1696726","volume":"40","author":"JN Brownell","year":"2019","unstructured":"Brownell JN, Bashaw H, Stallings VA (2019) Growth and nutrition in cystic fibrosis. 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