Objectives: Health problems in patients with heritable connective tissue disorders (HCTD) are diverse and complex and might lead to lower physical activity (PA) and physical fitness (PF). This study aimed to investigate the PA and PF of children with heritable connective tissue disorders (HCTD).Methods: PA was assessed using an accelerometer-based activity monitor (ActivPAL) and the mobility subscale of the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT). PF was measured in terms of cardiovascular endurance using the Fitkids Treadmill Test (FTT); maximal hand grip strength, using hand grip dynamometry (HGD) as an indicator of muscle strength; and motor proficiency, using the Bruininks-Oseretsky Test of Motor Proficiency-2 (BOTMP-2).Results: A total of 56 children, with a median age of 11.6 (interquartile range [IQR], 8.8–15.8) years, diagnosed with Marfan syndrome (MFS), n = 37, Loeys-Dietz syndrome (LDS), n = 6, and genetically confirmed Ehlers-Danlos (EDS) syndromes, n = 13 (including classical EDS n = 10, vascular EDS n = 1, dermatosparaxis EDS n = 1, arthrochalasia EDS n = 1), participated. Regarding PA, children with HCTD were active for 4.5 (IQR 3.5–5.2) hours/day, spent 9.2 (IQR 7.6–10.4) hours/day sedentary, slept 11.2 (IQR 9.5–11.5) hours/day, and performed 8,351.7 (IQR 6,456.9–1,0484.6) steps/day. They scored below average (mean (standard deviation [SD]) z-score −1.4 (1.6)) on the PEDI-CAT mobility subscale. Regarding PF, children with HCTD scored well below average on the FFT (mean (SD) z-score −3.3 (3.2)) and below average on the HGD (mean (SD) z-score −1.1 (1.2)) compared to normative data. Contradictory, the BOTMP-2 score was classified as average (mean (SD) z-score.02 (.98)). Moderate positive correlations were found between PA and PF (r(39) = .378, p < .001). Moderately sized negative correlations were found between pain intensity and fatigue and time spent actively (r(35) = .408, p < .001 and r(24) = .395 p < .001, respectively).Conclusion: This study is the first to demonstrate reduced PA and PF in children with HCTD. PF was moderately positively correlated with PA and negatively correlated with pain intensity and fatigue. Reduced cardiovascular endurance, muscle strength, and deconditioning, combined with disorder-specific cardiovascular and musculoskeletal features, are hypothesized to be causal. Identifying the limitations in PA and PF provides a starting point for tailor-made interventions.
BACKGROUND: Physical activity (PA) is important for children with a chronic disease. Serious games may be useful to promote PA levels among these children.OBJECTIVE: The primary purpose of this systematic review was to evaluate the effectiveness of serious games on PA levels in children with a chronic disease.METHODS: PubMed, EMBASE, PsycINFO, ERIC, Cochrane Library, and CINAHL were systematically searched for articles published from January 1990 to May 2018. Both randomized controlled trials and controlled clinical trials were included to examine the effects of serious games on PA levels in children with a chronic disease. Two investigators independently assessed the intervention, methods, and methodological quality in all articles using the Cochrane risk of bias tool. Both qualitative and quantitative analyses were performed.RESULTS: This systematic review included 9 randomized controlled trials (886 participants). In 2 of the studies, significant between-group differences in PA levels in favor of the intervention group were reported. The meta-analysis on PA levels showed a nonsignificant effect on moderate to vigorous PA (measured in minutes per day) between the intervention and control groups (standardized mean difference 0.30, 95% CI -0.15 to 0.75, P=.19). The analysis of body composition resulted in significantly greater reductions in BMI in the intervention group (standardized mean difference -0.24, 95% CI -0.45 to 0.04, P=.02).CONCLUSIONS: This review does not support the hypothesis that serious games improve PA levels in children with a chronic disease. The meta-analysis on body composition showed positive intervention effects with significantly greater reductions in BMI in favor of the intervention group. A high percentage of nonuse was identified in the study of serious games, and little attention was paid to behavior change theories and specific theoretical approaches to enhance PA in serious games. Small sample sizes, large variability between intervention designs, and limited details about the interventions were the main limitations. Future research should determine which strategies enhance the effectiveness of serious games, possibly by incorporating behavior change techniques.
The six-minute walking test (6MWT) may be a practical test for the evaluation functional exercise capacity in children with end-stage renal disease (ESRD). The aim of this study was to investigate the 6MWT performance in children with ESRD compared to reference values obtained in healthy children and, secondly, to study the relationship between 6MWT performance with anthropometric variables, clinical parameters, aerobic capacity and muscle strength. Twenty patients (13 boys and seven girls; mean age 14.1 ± 3.4 years) on dialysis participated in this study. Anthropometrics were taken in a standardized manner. The 6MWT was performed in a 20-m-long track in a straight hallway. Aerobic fitness was measured using a cycle ergometer test to determine peak oxygen uptake (V⋅O2peak)(V⋅O2peak), peak rate (Wpeak) and ventilatory threshold (VT). Muscle strength was measured using hand-held myometry. Children with ESRD showed a reduced 6MWT performance (83% of predicted, p < 0.0001), irrespective of the reference values used. The strongest predictors of 6MWT performance were haematocrit and height. Regression models explained 59% (haematocrit and height) to 60% (haematocrit) of the variance in 6MWT performance. 6MWT performance was not associated with V⋅O2peakV⋅O2peak, strength, or other anthropometric variables, but it was significantly associated with haematocrit and height. Children with ESRD scored lower on the 6MWT than healthy children. Based on these results, the 6MWT may be a useful instrument for monitoring clinical status in children with ESRD, however it cannot substitute for other fitness tests, such as a progressive exercise test to measure V⋅O2peakV⋅O2peak or muscle strength tests.
