PMID- 36478293 OWN - NLM STAT- MEDLINE DCOM- 20230207 LR - 20230626 IS - 1432-1076 (Electronic) IS - 0340-6199 (Print) IS - 0340-6199 (Linking) VI - 182 IP - 2 DP - 2023 Feb TI - Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis. PG - 769-775 LID - 10.1007/s00431-022-04752-w [doi] AB - Cystic fibrosis (CF) is predominantly a lung disease but is also characterised by impaired skeletal muscularity and a reduction in fat-free mass. 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's 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 (13-17) years. They had a median (IQR) FFMI of 13.5 (11.6-15.1) kg/m(2). The FFMI significantly correlated with age (rho = 0.568, p = 0.002), number of shuttles (rho = 0.691, p < 0.001) and reported hours of activity per day (rho = 0.426, p = 0.024). The median (IQR) FFMI was significantly higher in male [15.1 (13.1-18.6) kg/m(2)] compared to female participants [12.7 (11.6-14.1) kg/m(2), p = 0.008]. The median (IQR) FFMI was significantly lower in the 10 (36%) participants with liver disease [11.9 (11.5-13.4) kg/m(2)] compared to the FFMI in the remaining 18 participants without liver disease [14.4 (12.5-15.9) kg/m(2), p = 0.027]. CONCLUSION: 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. WHAT IS KNOWN: * Health behaviours in adolescence influence lifelong health in cystic fibrosis (CF). * 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. WHAT IS NEW: * FFM increases with increasing age and growth in adolescents with CF. * Physical activity exerts a beneficial effect, and liver disease negatively affects FFM in adolescents with CF. CI - (c) 2022. The Author(s). FAU - Dassios, Theodore AU - Dassios T AUID- ORCID: 0000-0001-5258-5301 AD - Department of Women and Children's Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK. theodore.dassios@kcl.ac.uk. AD - Neonatal Intensive Care Centre, King's College Hospital NHS Foundation Trust, 4th Floor Golden Jubilee Wing, London, SE5 9RS, UK. theodore.dassios@kcl.ac.uk. FAU - Mitakidou, Maria Rafaela AU - Mitakidou MR AD - Department of Women and Children's Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK. FAU - Dhawan, Anil AU - Dhawan A AD - Pediatric Liver, GI & Nutrition Centre and Mowat Labs, King's College Hospital, London, UK. AD - Institute of Liver Studies, King's College London, London, UK. FAU - Papalexopoulou, Niovi AU - Papalexopoulou N AD - Department of Women and Children's Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK. FAU - Gupta, Atul AU - Gupta A AD - Pediatric Respiratory Medicine, Kings College Hospital NHS Foundation Trust, London, UK. FAU - Greenough, Anne AU - Greenough A AD - Department of Women and Children's Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK. AD - Asthma UK Centre in Allergic Mechanisms of Asthma, King's College London, London, UK. AD - NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust and King's College London, London, UK. LA - eng GR - G1000758/MRC_/Medical Research Council/United Kingdom PT - Journal Article DEP - 20221207 PL - Germany TA - Eur J Pediatr JT - European journal of pediatrics JID - 7603873 SB - IM MH - Child MH - Humans MH - Male MH - Female MH - Adolescent MH - Body Composition MH - *Cystic Fibrosis/complications MH - Quality of Life MH - Exercise MH - *Liver Diseases/diagnosis/etiology MH - Body Mass Index PMC - PMC9899174 OTO - NOTNLM OT - Adolescent OT - Body composition OT - Cystic fibrosis OT - Liver diseases COIS- The authors declare no competing interests. EDAT- 2022/12/09 06:00 MHDA- 2023/02/08 06:00 PMCR- 2022/12/07 CRDT- 2022/12/08 12:48 PHST- 2022/05/09 00:00 [received] PHST- 2022/12/02 00:00 [accepted] PHST- 2022/11/30 00:00 [revised] PHST- 2022/12/09 06:00 [pubmed] PHST- 2023/02/08 06:00 [medline] PHST- 2022/12/08 12:48 [entrez] PHST- 2022/12/07 00:00 [pmc-release] AID - 10.1007/s00431-022-04752-w [pii] AID - 4752 [pii] AID - 10.1007/s00431-022-04752-w [doi] PST - ppublish SO - Eur J Pediatr. 2023 Feb;182(2):769-775. doi: 10.1007/s00431-022-04752-w. Epub 2022 Dec 7.