Physical-capacity-related genetic polymorphisms in children with cystic fibrosis.

In patients with cystic fibrosis (CF), physical capacity (PC) has been correlated with mortality risk. In turn, PC is dependent on genetic factors. This study examines several polymorphisms associated with PC and health-related phenotype traits (VO2peak, FEV1, FVC, PImax and muscular strength) in a group of children with CF (n = 66, primary purpose). The same analyses were also performed in a control group of healthy children (n = 113, secondary purpose). The polymorphisms determined were classified as muscle function polymorphisms (ACE rs1799752; AGT rs699; ACTN3 rs1815739; PTK2 rs7843014 and rs7460; MSTN rs1805086; TRHR rs7832552; NOS3 rs2070744) or energy metabolism polymorphisms (PPARGC1A rs8192678; NRF1 rs6949152; NRF2 rs12594956; TFAM rs1937; PPARD rs2267668; ACSL1 rs6552828). No significant polymorphism/phenotype correlations were detected in children with CF, with marginal associations being observed between NOS3 rs2070744 and VO2peak and FEV1, as well as between PPARGC1A rs8192678 and FEV1. Overall, similar findings were observed in the control group, i.e., no major associations. The PC-related polymorphisms examined seem to have no effects on the PC or health of children with CF.

Benefits of combining inspiratory muscle with 'whole muscle' training in children with cystic fibrosis: a randomised controlled trial.

BACKGROUND: The purpose of this study (randomised controlled trial) was to assess the effects of an 8-week combined 'whole muscle' (resistance+aerobic) and inspiratory muscle training (IMT) on lung volume, inspiratory muscle strength (PImax) and cardiorespiratory fitness (VO2 peak) (primary outcomes), and dynamic muscle strength, body composition and quality of life in paediatric outpatients with CF (cystic fibrosis, secondary outcomes). We also determined the effects of a detraining period. METHODS: Participants were randomly allocated with a block on gender to a control (standard therapy) or intervention group (initial n=10 (6 boys) in each group; age 10±1 and 11±1 years). The latter group performed a combined programme (IMT (2 sessions/day) and aerobic+strength exercises (3 days/week, in-hospital)) that was followed by a 4-week detraining period. All participants were evaluated at baseline, post-training and detraining. RESULTS: Adherence to the training programme averaged 97.5%±1.7%. There was a significant interaction (group×time) effect for PImax, VO2peak and five-repetition maximum strength (leg-press, bench-press, seated-row) (all (p<0.001), and also for %fat (p<0.023) and %fat-free mass (p=0.001), with training exerting a significant beneficial effect only in the intervention group, which was maintained after detraining for PImax and leg-press. CONCLUSION: The relatively short-term (8-week) training programme used here induced significant benefits in important health phenotypes of paediatric patients with CF. IMT is an easily applicable intervention that could be included, together with supervised exercise training in the standard care of these patients.

Health-related quality of life of Spanish children with cystic fibrosis.

PURPOSE: To investigate (1) the contributions of sex, age, nutritional status- and physical-fitness-related variables on health-related quality of life (HRQOL) in Spanish children with cystic fibrosis, and (2) the agreement on HRQOL between children and their parents. METHODS: In 28 children aged 6-17 years, body mass index percentile, percentage body fat, physical activity, pulmonary function, cardiorespiratory fitness, functional mobility, and dynamic muscle strength were determined using objective measures. HRQOL was measured using the revised version of the cystic fibrosis questionnaire. Simple and multiple linear regression analyses were performed to determine the variables associated with HRQOL. To assess the agreement on HRQOL between children and parents, intra-class correlation coefficients (ICCs) were calculated. RESULTS: Girls reported worse emotional functioning, a higher treatment burden, and more respiratory problems than boys. Greater functional mobility appeared associated with a less favourable body image and more eating disturbances. Agreement on HRQOL between children and parents was good to excellent, except for the domain of treatment burden. CONCLUSIONS: Sex and age were stronger predictors of HRQOL than nutritional status- or physical-fitness-related variables. Children reported a lower treatment burden than their parents perceived them to have.

Exercise Intervention in Pediatric Patients with Solid Tumors: The Physical Activity in Pediatric Cancer Trial.

INTRODUCTION: The randomized controlled trial "Physical Activity in Pediatric Cancer" determined the effects of an inhospital exercise intervention combining aerobic and muscle strength training on pediatric cancer patients with solid tumors undergoing neoadjuvant chemotherapy. METHODS: Participants were allocated to an exercise (n = 24, 17 boys; mean ± SEM age, 10 ± 1 yr) or control group (n = 25, 18 boys; 11 ± 1 yr). Training included three sessions per week for 19 ± 2 wk. Participants were assessed at treatment initiation, termination, and 2 months after end treatment. The primary endpoint was muscle strength (as assessed by upper and lower-body five-repetition-maximum tests). Secondary endpoints included cardiorespiratory fitness, functional capacity during daily life activities, physical activity, body mass and body mass index, and quality of life. RESULTS: Most sessions were performed in the hospital's gymnasium. Adherence to the program averaged 68% ± 4% and no major adverse events or health issues were noted. A significant interaction (group-time) effect was found for all five-repetition maximum tests (leg/bench press and lateral row; all P < 0.001). Performance significantly increased after training (leg press: 40% [95% confidence interval [CI], 15-41 kg); bench press: 24% [95% CI, 6-14 kg]; lateral row 25% [95% CI, 6-15 kg]), whereas an opposite trend was found in controls. Two-month post values tended to be higher than baseline for leg (P = 0.017) and bench press (P = 0.014). In contrast, no significant interaction effect was found for any of the secondary endpoints. CONCLUSION: An inhospital exercise program for pediatric cancer patients with solid tumors undergoing neoadjuvant treatment increases muscle strength despite the aggressiveness of such therapy.

Levels of moderate--vigorous physical activity are low in Spanish children with cystic fibrosis: a comparison with healthy controls.

BACKGROUND: Physical activity (PA) is recommended as part of the treatment regimen for cystic fibrosis (CF) although objective methods have been scarcely used to monitor achievement of PA guidelines. METHODS: PA was measured by accelerometer in outpatient CF children (n=47) and results were compared with those obtained in age- and gender-matched healthy controls (n=39). RESULTS: 2.1% of the outpatients fulfilled PA guidelines (i.e. ≥60min·day(-1) of moderate-to-vigorous PA (MVPA)) vs. 34.2% of controls. Overall, lower MVPA levels were observed in CF patients than controls despite the former undergoing more light or total PA. Peak oxygen uptake was also lower in the CF group than in controls (37.5±7.8 vs. 43.5±7.6ml·kg(-1)·min(-1), p=0.002) and was correlated with MVPA and vigorous PA in the former. CONCLUSIONS: These findings support a need to promote PA interventions (including MVPA) targeted at improving cardiorespiratory fitness in CF children.

Aerobic fitness is associated with lower risk of hospitalization in children with cystic fibrosis.

BACKGROUND: Children with cystic fibrosis (CF) often have to be hospitalized because of acute exacerbation of their respiratory symptoms. Given the fact that improved peak oxygen uptake (VO2peak ) is positively associated with lung function and overall health in children with CF, this study examined the association between VO2peak and the need for hospitalization in a cohort of pediatric CF patients. METHODS: In a 3-year study, 77 CF children with mild-to-moderately severe CF (forced expiratory volume in 1 sec [FEV1 ] ≥ 50%) underwent a maximal exercise test to determine VO2peak . Anthropometric, lung function and muscle strength measurements were also conducted and dates of hospitalization were recorded for the study period. Associations were then determined between the variables recorded and hospitalization by univariate and multivariate Cox proportional hazards regression analyses. RESULTS: VO2peak was 38.6 ± 6.7 ml kg(-1) min(-1) for boys and 31.9 ± 6.9 ml kg(-1) min(-1) for girls. In multivariate analyses, VO2peak was the only variable significantly associated with time to hospitalization (hazard ratio 0.91, P = 0.03). CONCLUSION: A significant association was detected between greater aerobic fitness, and lower risk of hospitalization. Because hospitalization due to respiratory exacerbation is a powerful prognostic factor, our findings provide further support for the importance of aerobic fitness evaluation in the management of children with mild-to-moderately severe CF.

Physical Activity in Pediatric Cancer patients with solid tumors (PAPEC): trial rationale and design.

BACKGROUND: This randomized controlled trial on Physical Activity in Pediatric Cancer (PAPEC) was designed to assess the impact of an exercise program on pediatric cancer patients undergoing chemotherapy for solid tumors. METHODS AND DESIGN: 60 pediatric patients of both sexes, aged 4 to 18 years and undergoing treatment for extracranial primary solid tumors will be recruited for this trial. Each participant will be randomly assigned (with blocking on sex) to either an intervention or control (normal care) group. The intervention group will participate in combined inpatient physical training (aerobic + strength) for the duration of neoadjuvant chemotherapy. The intervention will include 3 weekly 60-70 min exercise sessions in the child's room or in a pediatric gym at the hospital, depending on the child's health state. In both groups, determination of several primary (cardio-respiratory fitness, muscle strength, functional capacity, physical activity levels, body weight and quality of life) and secondary outcomes [immune function and inflammatory profile (blood levels of 47 cytokines)] will be made at the following time points: (i) before the exercise intervention (immediately after diagnosis and before treatment onset); (ii) after the exercise intervention (upon termination of neoadjuvant chemotherapy); and (iii) after a detraining period (2 months after the intervention). DISCUSSION: The PAPEC trial will provide relevant new information on biological mechanisms and inform on the potential clinical use of exercise during pediatric cancer treatment as a simple way to prevent future long-term treatment effects and improve the general health state of pediatric cancer patients.

Intrahospital weight and aerobic training in children with cystic fibrosis: a randomized controlled trial.

PURPOSE: The purpose of our study was to assess the effects of an 8-wk intrahospital combined circuit weight and aerobic training program performed by children with cystic fibrosis (of low-moderate severity and stable clinical condition) on the following outcomes: cardiorespiratory fitness (VO2peak) and muscle strength (five-repetition maximum (5RM) bench press, 5RM leg press, and 5RM seated row) (primary outcomes) and pulmonary function (forced vital capacity, forced expiratory volume in 1 s), weight, body composition, functional mobility (Timed Up and Down Stairs and 3-m Timed Up and Go tests), and quality of life (secondary outcomes). We also determined the effects of a detraining period (4 wk) on the aforementioned outcomes. METHODS: We performed a randomized controlled trial design. Eleven participants in each group (controls: 7 boys, age = 11 ± 3 yr, body mass index = 17.2 ± 0.8 kg · m(-2) (mean ± SEM); intervention: 6 boys, age = 10 ± 2 yr, body mass index = 18.4 ± 1.0 kg · m(-2)) started the study. RESULTS: Adherence to training averaged 95.1% ± 7.4%. We observed a significant group × time interaction effect (P = 0.036) for VO2peak. In the intervention group, VO2peak significantly increased with training by 3.9 mL · kg(-1) · min(-1) (95% confidence interval = 1.8-6.1 mL · kg(-1) · min(-1), P = 0.002), whereas it decreased during the detraining period (-3.4 mL · kg(-1) · min(-1), 95% confidence interval = -5.7 to -1.7 mL · kg(-1) · min(-1), P = 0.001). In contrast, no significant changes were observed during the study period within the control group. Although significant improvements were also observed after training for all 5RM strength tests (P < 0.001 for the interaction effect), the training improvements were not significantly decreased after the detraining period in the intervention group (all P > 0.1 for after training vs detraining). We found no significant training benefits in any of the secondary outcomes. CONCLUSIONS: A short-term combined circuit weight and aerobic training program performed in a hospital setting induces significant benefits in the cardiorespiratory fitness and muscle strength of children with cystic fibrosis.

Does resistance training improve the functional capacity and well being of very young anorexic patients? A randomized controlled trial.

PURPOSE: We determined the effects of a 3-month low-moderate-intensity strength training program (2 sessions/week) on functional capacity, muscular strength, body composition, and quality of life (QOL) in 22 young (12-16 yrs) anorexic outpatients. METHODS: Patients were randomly assigned to a training or control group (n=11 [10 females] each). Training sessions were of low intensity (loads for large muscle groups ranging between 20%-30% and 50%-60% of six repetitions maximum [6RM] at the end of the program). We measured functional capacity by the time up and go and the timed up and down stairs tests. Muscular strength was assessed by 6RM measures for seated bench and leg presses. We estimated percent body fat and muscle mass. We assessed patients' QOL with the Short Form-36 items. RESULTS: The intervention was well tolerated and did not have any deleterious effect on patients' health, and did not induce significant losses in their body mass. The only studied variable for which a significant interaction (group x time) effect was found (p=.009) was the 6RM seated lateral row test. CONCLUSIONS: Low-moderate-intensity strength training does not seem to add major benefits to conventional psychotherapy and refeeding treatments in young anorexic patients.

Does creatine supplementation improve functional capacity in elderly women?

The purpose of this study was to determine the effects of short-term (7 days) oral creatine supplementation (0.3 g.kg(-1)) in elderly women during exercise tests that reflect functional capacity during daily living tasks. We assessed several indices of endurance capacity (1-mile walk test, gross mechanical efficiency, ventilatory threshold, and peak oxygen intake determined during cycle-ergometry) and lower-extremity functional performance (time to complete sit-stand test). Subjects were assigned to a creatine (n = 10; age 67 +/- 6 years) or placebo (n = 6; age 68 +/- 4 years) group. We found a significant improvement only after creatine loading in the sit-stand test (placebo: 9.7 +/- 0.9 seconds for pretest and 9.3 +/- 0.7 seconds for posttest, p > 0.05; creatine: 10.0 +/- 0.7 seconds for pretest and 8.8 +/- 1.1 seconds for posttest). Significance was recorded at p < 0.05 for the interaction effect (group [creatine, placebo] x time [pretest, posttest]). In elderly women, short-term oral creatine supplementation does not improve endurance capacity but increases the ability to perform lower-body functional living tasks involving rapid movements.