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.
De term soft control wordt gebruikt voor een keur aan sturings- en beheersmaatregelen die organisaties helpen op koers te blijven. Ze hebben vooral betrekking op gedrag en cultuur. Uit ons inventariserend onderzoek blijkt dat er geen eenduidigheid over de definitie van soft control is en al helemaal niet over de manier waarop soft control wordt uitgewerkt. Hierdoor zijn veel uitwerkingen en meetinstrumenten voor soft control in omloop. In dit artikel vragen we ons af hoe we de kwaliteit van meetinstrumenten voor soft control kunnen beoordelen. Op basis van een aantal methodologische criteria ontwikkelen we een ordeningskader van deze instrumenten. Hiermee scheppen we helderheid in de fascinerende wereld van sociaalwetenschappelijke meetinstrumenten.
This book describes how policy workers and administrators of local councils and non-profit organisations can contribute to a social domain in which the people’s strengths are better utilized within the framework of the Social Support Act (Wmo). The book is built up around the three assignments a government sees itself faced with: - The transformation from less of a system world to more of a lifeworld. - The transformation from ‘steering’ in a less top-down to a more bottom-up way. - The transformation from less deductive to more inductive learning and development. In this book they describe how representatives of steering force can contribute to this transformation. In their conclusion, the authors state these representatives cannot ultimately fulfill the role of director in the transformation of the social domain geared to the strengths in the lifeworld. They can help to make the transformation less dependent on ‘higher powers’. The authors end their book with recommendations and suggestions for further research on the topic of WMO. This book is a translated version of “Outreachend besturen in tijden van transitie”, published by Movisie.