The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis

Scully, Kevin; Jay, Laura; Freedman, Steven D.; Sawicki, Gregory S.; Uluer, Ahmet; Finkelstein, Joel S.; Putman, Melissa S.

doi:10.3390/nu14020310

Cited by 9 publications

(9 citation statements)

References 41 publications

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“…In our study, we measured a younger population at a median age of 15 years and reported a median FEV 1 of 77.3% predicted and a FFMI of 37.7 kg. The actual values of the fat-free mass in our subjects are, as expected, lower compared to healthy children and young people of a similar age [22]. In our study, we report a median fat-free mass of 37.7 kg, while population studies in healthy 13-18 year old children have reported mean fat-free mass values of 38.9-41.8 kg [23].…”

Section: Discussionsupporting

confidence: 63%

“…Our population is clinically comparable to previous studies of body composition in CF. Scully and colleagues studied 38 adolescents and adults with CF at a mean age of 28 years with a mean FEV 1 of 73% predicted and reported a mean lean mass of 42.2 kg measured by DEXA [22]. In our study, we measured a younger population at a median age of 15 years and reported a median FEV 1 of 77.3% predicted and a FFMI of 37.7 kg.…”

Section: Discussionmentioning

confidence: 81%

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Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis

et al. 2022

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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/m2. 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/m2] compared to female participants [12.7 (11.6–14.1) kg/m2, 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/m2] compared to the FFMI in the remaining 18 participants without liver disease [14.4 (12.5–15.9) kg/m2, 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.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 81%

Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis

et al. 2022

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“…There are number of potential reasons for the high percent body fat found in many of our patients at the time of admission. In addition to acute illness, these include a small skeletal muscle mass, owing to a previous sedentary lifestyle, 31 and preference for or family reliance on high glycemic, low protein foods 32,33 . Yet another factor is CFTR modulator therapy 6,7,34–36 .…”

Section: Discussionmentioning

confidence: 99%

“…In addition to acute illness, these include a small skeletal muscle mass, owing to a previous sedentary lifestyle, 31 and preference for or family reliance on high glycemic, low protein foods. 32,33 Yet another factor is CFTR modulator therapy. 6,7,[34][35][36] Multiple contributing factors preclude simple interventions aimed at normalizing body composition.…”

Section: Admission Considerationsmentioning

confidence: 99%

Body composition and functional correlates of CF youth experiencing pulmonary exacerbation and recovery

Boat

Hente

Hardie

et al. 2022

Pediatric Pulmonology

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Background: Youth with cystic fibrosis (CF) and pulmonary exacerbation (PEx) often experience weight loss, then rapid weight gain. Little is known about body composition and its relationship to functional outcomes during this critical period. Methods: Twenty CF youth experiencing PEx were assessed on the day following admission and 7-17 days later at discharge for body mass index (BMI), fat mass index (FMI), lean mass index (LMI), skeletal muscle mass index (SMMI), and functional measures: percent predicted forced expiratory volume in 1 second (FEV1) (ppFEV1), maximal inspiratory and expiratory pressures (MIPs and MEPs), and handgrip strength (HGS). Changes from admission to discharge and correlations among body composition indices and functional measures at both times are reported. Results: Upon admission, participant BMI percentile and ppFEV1 varied from 2 to 97 and 29 to 113, respectively. Thirteen had an LMI below the 25th percentile and nine had a percent body fat above the 75th percentile. BMI and FMI increased significantly (p = 0.03, 0.003) during hospitalization. LMI and SMMI did not change.FEV1 and MIPS increased (p = 0.0003, 0.007), independent of weight gain, during treatment. HGS did not improve. Conclusions: Many youth with CF, independent of BMI, frequently carried a small muscle mass and disproportionate fat at the time of PEx. During hospital treatment, weight gain largely represented fat deposition; muscle mass and strength did not improve. A need for trials of interventions designed to augment muscle mass and function, and limit fat mass accretion, at the time of PEx is suggested by these observations.

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“…Malnutrition in patients with cystic fibrosis is often a problem associated with lung deterioration, reduced exercise tolerance, growth retardation and reduced quality of life. Dietary deficiencies in cystic fibrosis are associated with gastrointestinal complications such as nutritional deficiencies, including fat and fat-soluble vitamins, diabetes, liver dysfunction and cholelithiasis [70]. In pediatric cystic fibrosis patients, chemerin levels were comparable to healthy controls and did not correlate with nutritional status and levels of interleukin-1 beta (IL-1b), interleukin-6 (IL-6) and TNF α [68].…”

Section: Chemerin In Other Diseases In Childrenmentioning

confidence: 99%

Chemerin as Potential Biomarker in Pediatric Diseases: A PRISMA-Compliant Study

2022

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Adipose tissue is the main source of adipokines and therefore serves not only as a storage organ, but also has an endocrine effect. Chemerin, produced mainly in adipocytes and liver, is a natural ligand for chemokine-like receptor 1 (CMKLR1), G-protein-coupled receptor 1 (GPR1) and C-C motif chemokine receptor-like 2 (CCRL2), which have been identified in many tissues and organs. The role of this protein is an active area of research, and recent analyses suggest that chemerin contributes to angiogenesis, adipogenesis, glucose homeostasis and energy metabolism. Many studies confirm that this molecule is associated with obesity in both children and adults. We conducted a systematic review of data from published studies evaluating chemerin in children with various disease entities. We searched PubMed to identify eligible studies published prior to February 2022. A total of 36 studies were selected for analysis after a detailed investigation, which was intended to leave only the research studies. Moreover, chemerin seems to play an important role in the development of cardiovascular and digestive diseases. The purpose of this review was to describe the latest advances in knowledge of the role of chemerin in the pathogenesis of various diseases from studies in pediatric patients. The mechanisms underlying the function of chemerin in various diseases in children are still being investigated, and growing evidence suggests that this adipokine may be a potential prognostic biomarker for a wide range of diseases.

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The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis

Cited by 9 publications

References 41 publications

Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis

Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis

Body composition and functional correlates of CF youth experiencing pulmonary exacerbation and recovery

Chemerin as Potential Biomarker in Pediatric Diseases: A PRISMA-Compliant Study

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