OBJECTIVE: Juvenile dermatomyositis (DM) is an inflammatory myopathy in which the immune system targets the microvasculature of the skeletal muscle and skin, leading to significant muscle weakness and exercise intolerance, although the precise etiology is unknown. The goal of this study was to investigate the changes in exercise capacity in children with myositis during active and inactive disease periods and to study the responsiveness of exercise parameters.METHODS: Thirteen children with juvenile DM (mean+/-SD age 11.2+/-2.6 years) participated in this study. Patients performed a maximal exercise test using an electronically braked cycle ergometer and respiratory gas analysis system. Exercise parameters were analyzed, including peak oxygen uptake (VO2peak), peak work rate (Wpeak), and ventilatory anaerobic threshold (VAT). All children were tested during an active period of the disease and during a remission period. From these data, 4 different response statistics were calculated.RESULTS: The children performed significantly better during a remission period compared with a period of active disease. Most exercise parameters showed a very large response. The 5 most responsive parameters were Wpeak, Wpeak (percent predicted), oxygen pulse, VO2peak, and power at the VAT.CONCLUSION: We found in our longitudinal study that children with active juvenile DM had significantly reduced exercise parameters compared with a remission period. Moreover, we found that several parameters had very good responsiveness. With previously established validity and reliability, exercise testing has been demonstrated to be an excellent noninvasive instrument for the longitudinal followup of children with myositis.
IntroductionMechanical power of ventilation, a summary parameter reflecting the energy transferred from the ventilator to the respiratory system, has associations with outcomes. INTELLiVENT–Adaptive Support Ventilation is an automated ventilation mode that changes ventilator settings according to algorithms that target a low work–and force of breathing. The study aims to compare mechanical power between automated ventilation by means of INTELLiVENT–Adaptive Support Ventilation and conventional ventilation in critically ill patients.Materials and methodsInternational, multicenter, randomized crossover clinical trial in patients that were expected to need invasive ventilation > 24 hours. Patients were randomly assigned to start with a 3–hour period of automated ventilation or conventional ventilation after which the alternate ventilation mode was selected. The primary outcome was mechanical power in passive and active patients; secondary outcomes included key ventilator settings and ventilatory parameters that affect mechanical power.ResultsA total of 96 patients were randomized. Median mechanical power was not different between automated and conventional ventilation (15.8 [11.5–21.0] versus 16.1 [10.9–22.6] J/min; mean difference –0.44 (95%–CI –1.17 to 0.29) J/min; P = 0.24). Subgroup analyses showed that mechanical power was lower with automated ventilation in passive patients, 16.9 [12.5–22.1] versus 19.0 [14.1–25.0] J/min; mean difference –1.76 (95%–CI –2.47 to –10.34J/min; P < 0.01), and not in active patients (14.6 [11.0–20.3] vs 14.1 [10.1–21.3] J/min; mean difference 0.81 (95%–CI –2.13 to 0.49) J/min; P = 0.23).ConclusionsIn this cohort of unselected critically ill invasively ventilated patients, automated ventilation by means of INTELLiVENT–Adaptive Support Ventilation did not reduce mechanical power. A reduction in mechanical power was only seen in passive patients.Study registrationClinicaltrials.gov (study identifier NCT04827927), April 1, 2021URL of trial registry recordhttps://clinicaltrials.gov/study/NCT04827927?term=intellipower&rank=1
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Objective: To investigate the effects of a school-based once-a-week sports program on physical fitness, physical activity, and cardiometabolic health in children and adolescents with a physical disability. Methods: This controlled clinical trial included 71 children and adolescents from four schools for special education [mean age 13.7 (2.9) years, range 8–19, 55% boys]. Participants had various chronic health conditions including cerebral palsy (37%), other neuromuscular (44%), metabolic (8%), musculoskeletal (7%), and cardiovascular (4%) disorders. Before recruitment and based on the presence of school-based sports, schools were assigned as sport or control group. School-based sports were initiated and provided by motivated experienced physical educators. The sport group (n = 31) participated in a once-a-week school-based sports program for 6 months, which included team sports. The control group (n = 40) followed the regular curriculum. Anaerobic performance was assessed by the Muscle Power Sprint Test. Secondary outcome measures included aerobic performance, VO2 peak, strength, physical activity, blood pressure, arterial stiffness, body composition, and the metabolic profile. Results: A significant improvement of 16% in favor of the sport group was found for anaerobic performance (p = 0.003). In addition, the sport group lost 2.8% more fat mass compared to the control group (p = 0.007). No changes were found for aerobic performance, VO2 peak, physical activity, blood pressure, arterial stiffness, and the metabolic profile. Conclusion: Anaerobic performance and fat mass improved following a school-based sports program. These effects are promising for long-term fitness and health promotion, because sports sessions at school eliminate certain barriers for sports participation and adding a once-a-week sports session showed already positive effects for 6 months.
