BACKGROUND: Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. OBJECTIVES: To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. SELECTION CRITERIA: All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. MAIN RESULTS: Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low-to low-quality evidence from both short-and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training (or a combination of both) has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. No study reported on mortality; two studies reported on adverse events (moderate-quality evidence); one of each study reported on pulmonary exacerbations (low-quality evidence) and diabetic control (very low-quality evidence). Although improvements were not consistent between studies and ranged from no effects to clearly positive effects, the most consistent effects of the heterogeneous exercise training modalities and durations were found for maximal aerobic exercise capacity (in four out of seven studies) with unclear effects on forced expiratory volume in one second (in two out of 11 studies) and health-related quality of life (in two out of seven studies). AUTHORS' CONCLUSIONS: Evidence about the efficacy of physical exercise training in cystic fibros...

Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight children.

Our children 5 to 7 years old born at VLBW or ELBW, with or without CLD, have some degree of pulmonary dysfunction at rest and following exercise and a higher prevalence of EIB with no reduction in maximal aerobic exercise performance. The findings suggest that the pulmonary limitations are associated with low birthweight even in the absence of CLD. The higher O2 uptake at a given mechanical power in the CLD group may cause early fatigability during prolonged exercise, even when aerobic performance is normal.

The present study tested the effect of a school-based physical activity (PA) program on quality of life (QoL) in 540 elementary school children. First and fifth graders were randomly assigned to a PA program or a no-PA control condition during one academic year. QoL was assessed by the Child Health Questionnaire at baseline and postintervention. Based on mixed linear model analyses, physical QoL in first graders and physical and psychosocial QoL in fifth graders were not affected by the intervention. In first graders, the PA intervention had a positive impact on psychosocial QoL (effect size [d], 0.32; p < .05). Subpopulation analyses revealed that this effect was caused by an effect in urban (effect size [d], 0.38; p < .05) and overweight first graders (effect size [d], 0.45; p < .05). In conclusion, a school-based PA intervention had little effect on QoL in elementary school children.