BACKGROUND: This cross-sectional controlled study aims to assess health-related quality of life (HRQoL) of children and adolescents with a molecular diagnosis of Marfan syndrome (MFS) or related disorders and to evaluate the factors associated with HRQoL in this population. Sixty-three children with MFS and 124 age- and sex-matched healthy children were recruited. HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL™) generic questionnaire. The correlation between HRQoL scores and the different continuous parameters (age, body mass index, disease severity, systemic score, aortic sinus diameter, and aerobic physical capacity) was evaluated using Pearson\'s or Spearman\'s coefficient. A multiple linear regression analysis was performed on the two health summary self-reported PedsQL™ scores (physical and psychosocial) to identify the factors associated with HRQoL in the MFS group.
RESULTS: Except for emotional functioning, all other domains of HRQoL (psychosocial and physical health, social and school functions) were significantly lower in children with MFS compared to matched healthy children. In the MFS group, the physical health summary score was significantly lower in female than in male patients (self-report: absolute difference [95%CI] = -8.7 [-17.0; -0.47], P = 0.04; proxy-report: absolute difference [95%CI] = -8.6 [-17.3; 0.02], P = 0.05) and also negatively correlated with the systemic score (self-report: R = -0.24, P = 0.06; proxy-report: R = -0.29, P = 0.03) and with the height Z-score (proxy-report: R = -0.29, P = 0.03). There was no significant difference in the physical health summary scores between the different genetic subgroups. In the subgroup of 27 patients who performed a cardiopulmonary exercise test, self- and proxy-reported physical health summary scores were highly correlated with their aerobic physical capacity assessed by peak oxygen consumption (VO2max) and ventilatory anaerobic threshold (VAT). In the multivariate analysis, the most important independent predictors of decreased physical health were increased height, decreased body mass index, decreased VAT and use of prophylactic therapy.
CONCLUSIONS: This study reports an impaired HRQoL in children and adolescents with MFS or related conditions, in comparison with matched healthy children. Educational and rehabilitation programs must be developed and evaluated to improve exercise capacity and HRQoL in these patients.
BACKGROUND: ClinicalTrials.gov, NCT03236571 . Registered 28 July 2017.

Time constraints comprise one limiting factor for implementing school-based physical activity programs. The aim of this pilot cluster randomized controlled study was to explore the effects of a cycle ergometer intervention during regular lessons on physical fitness, body composition, and health-related blood parameters.
Participants attended one of 2 classes selected from one school, which were randomly assigned to an intervention group (n = 23, 11.2 [0.5] y) consisting of cycling on classroom-based ergometers during 3 lessons per week at a self-selected intensity and a control group (n = 21, 11.3 [0.5] y) not receiving any treatment. Prior to and after the 5-month intervention period, physical fitness (with ventilatory threshold as primary outcome), body composition, and parameters of glucose and lipid metabolism were assessed.
A significant time × group interaction was revealed for ventilatory threshold (P = .035), respiratory compensation point (P = .038), gross efficiency (P < .001), maximal aerobic power (P = .024), triglycerides (P = .041), and blood glucose levels (P = .041) with benefits for the intervention group. Peak oxygen uptake and body composition were not affected.
Children\'s aerobic capacity benefited from the low-intensity school-based cycling intervention, while body composition and most blood parameters were not affected. The intervention using cycle ergometers is a feasible and time-saving strategy to elevate submaximal physical fitness.

[Purpose] This study aimed to determine the impact of aerobic exercise on aerobic capacity, balance, and treadmill time in patients with thermal burn injury. [Subjects and Methods] Burned adult patients, aged 20-40 years (n=30), from both sexes, with second degree thermal burn injuries covering 20-40% of the total body surface area (TBSA), were enrolled in this trial for 3 months. Patients were randomly divided into; group A (n=15), which performed an aerobic exercise program 3 days/week for 60 min and participated in a traditional physical therapy program, and group B (n=15), which only participated in a traditional exercise program 3 days/week. Maximal aerobic capacity, treadmill time, and Berg balance scale were measured before and after the study. [Results] In both groups, the results revealed significant improvements after treatment in all measurements; however, the improvement in group A was superior to that in group B. [Conclusion] The results provide evidence that aerobic exercises for adults with healed burn injuries improve aerobic physical fitness and balance.