Although essential for providing optimal adolescent patient support, knowledge of the impact of Marfan syndrome in adolescence is limited. To explore adolescents’ perceived impact of Marfan syndrome on (physical) functioning (activities, participation), disability (limitations, restrictions), contextual factors and support needs, we interviewed 19 adolescents with Marfan syndrome. Audio-recordings were transcribed, coded and analysed using thematic analysis. Identified themes were “difficulties in keeping up with peers” and “being and feeling different from peers”. Furthermore, an adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY) model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and its contextual factors. Adolescents perceived problems in keeping up with peers in school, sports, leisure and friendships/relationships, and they could not meet work requirements. Moreover, participants perceived to differ from peers due to their appearance and disability. Contextual factors: coping with Marfan syndrome, self-esteem/image, knowledge about Marfan syndrome, support from family/friends/teachers, ability to express needs and peer-group acceptation acted individually as barrier or facilitator for identified themes.
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Purpose: Literature detailing the effectiveness of school-based physical activity promotion in- terventions in prevocational adolescents was reviewed to identify effective intervention characteristics.Methods: The search strategy assessed studies against inclusion criteria study design, study population, school setting, language, and construct. The risk of bias of the included studies was assessed, and extractions were made of the physical activity (PA) level outcome measures and intervention characteristics regarding organizational, social, and content features. A meta-analysis was conducted to determine the overall effect of the interventions on the PA level. Identification of effective intervention characteristics was done by subgroup analyses. Meta-regression analysis was performed with PA level as dependent variable and intervention characteristics as covariates. Results: A total of 40 eligible studies was included for meta-analyses. Among the included studies, the overall intervention effect on increasing the PA level of prevocational adolescents was weak (standardized mean difference [SMD] .19, 95% confidence interval [CI] .12e.27). Intervention characteristics that improve the effect size to a moderate level were intracurricular PA (SMD .43, 95% CI .19e.68), involving school staff in an intracurricular intervention (SMD .37, 95% CI .16e.58) and a tailored intracurricular intervention (SMD .35, 95% CI .13e.58). Meta-regression analysis confirmed PA as a positive predictor.Conclusions: The effect of a school-based PA intervention was small to moderate. A sensible choice in the assembly of a multicomponent school-based PA intervention increases the effectiveness considerably. Physical education teachers, school administrators, and policy makers should consider organizational (intracurriculum, short and medium duration), personal (tailoring, participation), social (school staff) and content (PA) determinants.
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BackgroundIn adolescents with non-pathological and pathological joint hypermobility, gait deviations have been associated with pain and fatigue. It remains unclear what distinguishes the non-pathological form of joint hypermobility (JH) from pathological forms (i.e. hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD). Our objective was to identify discriminative clinical characteristics and biomechanical gait features between adolescents with hEDS/HSD, JH, and healthy controls (HC).MethodsThirty-two adolescents were classified into three subgroups (hEDS/HSD=12, JH=5, HC=15). Clinical characteristics (e.g. pain intensity and surface, fatigue, functional disability) were inventoried.The gait pattern was assessed using a three-dimensional, eight-camera VICON MX1.3 motion capture system, operating at a sample rate of 100 Hz (VICON, Oxford, UK). Spatiotemporal parameters, joint angles (sagittal plane), joint work, joint impulse, ground reaction force and gait variability expressed as percentage using Principal Component Analysis (PCA) were assessed and analysed using multivariate analysis. Multivariate analysis data is expressed in mean differences(MD), standard error(SE) and P-values.ResultsThe hEDS/HSD-group had significantly higher fatigue score (+51.5 points, p = <0.001) and functional disability (+1.6, p < .001) than the HC-group. Pain intensity was significantly higher in the hEDS/HSD-group than the other subgroups (JH; +37 mm p = .004, HC; +38 mm, p = .001). The hEDS/HSD-group showed significantly more gait variability (JH; +7.2(2.0)% p = .003, HC; + 7.8(1.4)%, p = <0.001) and lower joint work (JH; −0.07(0.03)J/kg, p = .007, HC; − 0.06(0.03)J/kg, p = .013) than the other subgroups. The JH-group showed significantly increased ankle dorsiflexion during terminal stance (+5.0(1.5)degree, p = .001) compared to hEDS/HSD-group and knee flexion during loading response compared to HC-group (+5.7(1.8) degree, p = .011).SignificanceA distinctive difference in gait pattern between adolescents with non-pathological and pathological joint hypermobility is found in gait variability, rather than in the biomechanical features of gait. This suggests that a specific gait variability metric is more appropriate than biomechanical individual joint patterns for assessing gait in adolescents with hEDS/HSD.
