Examination of sensory reception and integration abilities in children with and without Prader-Willi syndrome.

BACKGROUND: Good postural stability control is dependent upon the complex integration of incoming sensory information (visual, somatosensory, vestibular) with neuromotor responses that are constructed in advance of a voluntary action or in response to an unexpected perturbation. AIMS: To examine whether differences exist in how sensory inputs are used to control standing balance in children with and without Prader-Willi syndrome (PWS). METHODS AND PROCEDURES: In this cross-sectional study, 18 children with PWS and 51 children categorized as obese but without PWS (without PWS) ages 8-11 completed the Sensory Organization Test®. This test measures the relative contributions of vision, somatosensory, and vestibular inputs to the control of standing balance. The composite equilibrium score (CES) derived from performance in all sensory conditions, in addition to equilibrium scores (EQs) and falls per condition were compared between groups. OUTCOMES AND RESULTS: The CES was lower for children with PWS compared to children without PWS (M=53.93, SD=14.56 vs. M=66.17, SD=9.89, p = .001) while EQs declined in both groups between conditions 1 and 4 (F (1.305, 66.577) = 71.381, p < .001). No group differences in the percent of falls were evident in condition 5 but more children with PWS fell in condition 6 (χ2 (1) = 7.468, p = .006). Group differences in frequency of repeated falls also approached significance in conditions 5 (χ2 (3) = 4.630, p = .099) and 6 (χ2 (3) = 5.167, p = .076). CONCLUSIONS AND IMPLICATIONS: Children with PWS demonstrated a lower overall level of postural control and increased sway when compared to children with obesity. Both the higher incidence and repeated nature of falls in children with PWS in conditions 5 and 6 suggest an inability to adapt to sensory conditions in which vestibular input must be prioritized. Postural control training programs in this population should include activities that improve their ability to appropriately weight sensory information in changing sensory environments, with a particular focus on the vestibular system. WHAT DOES THIS STUDY ADD?: This study shows that children with PWS demonstrate a lower level of postural stability. The results suggest that children with PWS show inability to adapt to sensory conditions that require prioritizing vestibular information to maintain postural control. This information can be used to help guide training programs in this population.

Athletes' Coping With the COVID-19 Pandemic: The Role of Self-Compassion and Cognitive Appraisal.

Coping with the COVID-19 pandemic had implications for athletes' mental well-being. This mixed-methods study examined the influence of self-compassion on athletes' coping during the pandemic through the mediator of cognitive appraisal. The prospective design involved 90 athletes completing two online surveys 1 week apart measuring self-compassion, cognitive appraisal, and coping strategies. The PROCESS macro was used for the mediation analysis. A qualitative thematic analysis was used to explore athletes' responses to the pandemic during the second survey. Self-compassion had an indirect negative effect on avoidance-focused coping by appraising the pandemic as less of a threat (95% confidence interval [-0.20, -0.001]) and had a total effect on emotion-focused coping (95% confidence interval [0.02, 0.40]). Based on the thematic analysis, athletes described many raw emotions and a variety of coping strategies during the pandemic. Self-compassion demonstrated promising benefits to athletes who dealt with the challenging situation of the pandemic.

Parental and familial factors related to participation in a home-based physical activity intervention in children with obesity or Prader-Willi syndrome.

Background: Increasing physical activity (PA) participation is vital to promote the development of health behaviors in childhood. This study examined which parental and familial factors predicted completion of and compliance with a home-based family PA program in a cohort of families with a child with Prader-Willi syndrome (PWS; a rare disorder with obesity and developmental disability) or with obesity but with neurotypical development. Methods: Participants (n = 105) were parents of children with PWS (n = 41) and parents of children with obesity but without PWS (n = 64). Parents completed a series of questionnaires documenting their demographic characteristics, self-efficacy, social support, and family environment (active-recreational orientation and cohesion). Relationships between these factors and intervention completion and compliance were evaluated using bivariate correlations and logistic regression (compliance) and multiple regression (completion) analyses with groups together and then separately if the child group was a significant predictor. Results: None of the variables of interest (marital status, employment, employed hours per week, self-efficacy, social support, and family environment) were significant predictors of intervention completion. Intervention compliance was negatively associated with parents working part-time and working full-time and positively associated with family cohesion (Model R2 = 0.107, F(3,100) = 4.011, p = .010). Child group was not a factor. Conclusions: Compliance with a 24-week family home-based PA intervention was related to fewer employment hours of the primary caregiver and family environment factors. Future interventions should consider how to reduce the intervention's burden in working parents along with strategies to foster family cohesion.

Mental Fatigue Uniquely Influences Drop Landing Biomechanics for Individuals With a Concussion History.

CONTEXT: Induced mental fatigue negatively impacts sport performance and neurocognition. However, it is unclear how induced mental fatigue influences landing biomechanics. The purpose of this study was to examine the influence of mental fatigue on drop landing biomechanics in individuals with and without a concussion history. DESIGN: Crossover design. METHODS: Forty-eight (24 per group) recreationally active individuals were matched on age (±3 y), sex, and body mass index (±1 kg/m2). All participants completed an experimental (30-min Stroop task) and control (30-min reading magazines) intervention on separate days separated by a minimum of 24 hours. Drop landings were performed before and after both interventions. Outcomes included peak vertical ground reaction force (vGRF), vertical loading rate (VLR), knee flexion angle, knee abduction angle, external knee flexion moment, external knee abduction moment, and initial ground contact knee flexion and knee abduction angles. Separate 2 (group) × 2 (intervention) between-within analyses of covariance compared drop landing outcomes. Each group's average pre-Stroop and premagazine outcomes were covariates. RESULTS: There was a significant interaction for vGRF (P = .033, ηp2=.097) and VLR (P = .0497, ηp2=.083). The vGRF simple effects were not statistically significantly (P range = .052-.325). However, individuals with a concussion history displayed a medium effect size for greater vGRF post-Stroop compared with their own postmagazine vGRF (mean difference (95% confidence interval [95% CI] = 0.163 (-0.002 to 0.327) bodyweight (BW), p =.052, ηp2=.081. In contrast, the control group displayed a small effect size (mean difference [95% CI] = 0.095 [-0.069 to 0.259] BW, p =.251, ηp2=.029). Individuals with a concussion history displayed greater VLR post-Stroop compared with controls (mean difference [95% CI], 26.29 [6.19 to 46.40] BW/s, P = .012, ηp2=.134) and their own postmagazine values (mean difference [95% CI] = 32.61 [7.80 to 57.42] BW/s, p =.011, ηp2=.135). CONCLUSION: Mental fatigue leads to greater VLR for individuals with a concussion history. Athletic competition and activities of daily living can increase mental fatigue. Training programs may seek to teach mental fatigue reducing strategies to athletes with a concussion history.

Rhabdomyosarcoma With Epithelioid Features And NSD3::FOXO1 Fusion: Evidence For Reconsideration Of Previously Reported FOXO1::FGFR1 Fusion.

Epithelioid rhabdomyosarcoma is a rare rhabdomyosarcoma variant for which no diagnostic recurrent driver genetic events have been identified. Here we report a rapidly progressive and widely metastatic rhabdomyosarcoma with epithelioid features that arose in the thigh of a male infant. Conventional cytogenetics revealed a t(8;13)(p11.2;q14) translocation. Fluorescence in situ hybridization studies showed rearrangement of FOXO1 and amplification of its 3" end, and rearrangement of NSD3 and amplification of its 5` end. Next generation sequencing identified a NSD3::FOXO1 fusion, which is a previously unreported gene fusion. We also review the historic report of a FOXO1::FGFR1 fusion in a solid variant of alveolar rhabdomyosarcoma and propose that NSD3::FOXO1 fusion may have been the more appropriate interpretation of the data presented in that report.

Drop Landing Biomechanics in Individuals With and Without a Concussion History.

Research has identified an increased risk of lower extremity injury postconcussion, which may be due to aberrant biomechanics during dynamic tasks. The purpose of this study was to compare the drop landing biomechanics between individuals with and without a concussion history. Twenty-five individuals with and 25 without a concussion history were matched on age (±3 y), sex, and body mass index (±1 kg/m2). Three-dimensional landing biomechanics were recorded to obtain dependent variables (peak vertical ground reaction force, loading rate, knee flexion angle and external moment, knee abduction angle and external moment, and knee flexion and abduction angle at ground contact). A 1-way multivariate analysis of variance compared outcomes between groups. There was no difference in drop landing biomechanics between individuals with and without a concussion history (F10,39 = 0.460, P = .877, Wilk Λ = .918). There was an effect of time since concussion on knee flexion characteristics. Time since most recent concussion explained a significant amount of variation in both peak (ΔR2 = .177, ß = -0.305, ΔP = .046) and initial ground contact (ΔR2 = .292, ß = -0.204, ΔP = .008) knee flexion angle after covarying for sex and body mass index. Therefore, time since concussion should be considered when evaluating biomechanical patterns.

Improved Motor Proficiency and Quality of Life in Youth With Prader-Willi Syndrome and Obesity 6 Months After Completing a Parent-Led, Game-Based Intervention.

PURPOSE: To determine changes and potential differences in physical activity (PA), gross motor proficiency (MP), and health parameters after a 6-month follow-up (FU) period following participation in a parent-led PA intervention in youth with or without Prader-Willi syndrome (PWS). METHODS: About 42 youth with PWS and 65 youth without PWS but with obesity (body fat percentage >95th percentile for age and sex), aged 8-16 years, participated. The intervention included preplanned PA sessions containing playground and console-based video games scheduled 4 days per week for 24 weeks. Families received training and curriculum materials. PA (accelerometry), MP (Bruininks-Oseretsky Test of MP), and health-related quality of life were obtained before (PRE), after completing the intervention (POST), and at FU. RESULTS: There were no significant changes in PA at any time point. At FU and POST, participants showed higher bilateral coordination (PRE = 9.3 [0.4], POST = 11.7 [0.5], and FU = 11.1 [0.6]); speed and agility (PRE = 9.2 [0.4], POST = 10.8 [0.4], and FU = 11.5 [0.5]); and strength (PRE = 8.0 [0.3], POST = 9.2 [0.3], and FU = 9.2 [0.3]) than at PRE. At FU (80.3 [2.1]) and POST (79.8 [1.7]), youth without PWS showed higher health-related quality of life than PRE (75.0 [1.8]). CONCLUSION: The improvements in MP and health-related quality of life at FU suggest long-term durability of intervention outcomes.

Influence of Parental Physical Activity and Sedentary Behavior on Young Children: Considering Time Together.

Purpose: Parents are key role models for their young child's physical activity (PA) and sedentary behavior. This study examined the relationship between parents and their young child's PA and sedentary behavior considering whether the parent and child were together (present) or apart. Methods: Parent and child dyads (N = 26) wore accelerometers for 10 days and recorded times when they were present or absent from their young children (parental presence) in an online daily diary. Hourly data for PA and sedentary behavior of both the parent and young child were coded for parental presence. Multilevel modeling was used to predict child behavior (sedentary, light PA, and moderate to vigorous PA) using the respective parent behavior, the presence of the parent, and the interaction between parent behavior and presence. Results: The interaction between presence and parent behavior predicted the respective child behavior (p < .05). Parents' behavior was positively related to their young child's behavior when they were together, but the relationship was not present (moderate to vigorous PA) or weaker (sedentary behavior, light PA) when apart. Conclusions: Being active alone was not sufficient for a parent's PA to relate to their young child's PA, but rather being active in the presence of their child was important for young child's PA.

A 24-Week Physical Activity Intervention Increases Bone Mineral Content without Changes in Bone Markers in Youth with PWS.

Bone mineral density (BMD) is of concern in Prader-Willi syndrome (PWS). This study compared responses to a physical activity intervention in bone parameters and remodeling markers in youth with PWS (n = 45) and youth with non-syndromic obesity (NSO; n = 66). Measurements occurred at baseline (PRE) and after 24 weeks (POST) of a home-based active games intervention with strengthening and jumping exercises (intervention group = I) or after a no-intervention period (control group = C). Dual x-ray absorptiometry scans of the hip and lumbar spine (L1-L4) determined BMD and bone mineral content (BMC). Bone markers included fasting bone-specific alkaline phosphatase (BAP) and C-terminal telopeptide of type I collagen (CTx). Both I and C groups increased their hip BMD and BMC (p < 0.001). Youth with PWS-I increased their spine BMC from PRE to POST (p < 0.001) but not youth with PWS-C (p = 1.000). Youth with NSO (I and C) increased their spine BMC between PRE and POST (all p < 0.001). Youth with PWS showed lower BAP (108.28 ± 9.19 vs. 139.07 ± 6.41 U/L; p = 0.006) and similar CTx (2.07 ± 0.11 vs.1.84 ± 0.14 ng/dL; p = 0.193) than those with NSO regardless of time. Likely, the novelty of the intervention exercises for those with PWS contributed to gains in spine BMC beyond growth. Bone remodeling markers were unaltered by the intervention.

The Relationship Between Self-Regulatory Efficacy and Physical Activity in Adolescents With a Caveat: A Cross-Lag Design Examining Weather.

PURPOSE: The use of self-efficacy to predict physical activity has a long history. However, this relationship is complex, as self-efficacy is thought to influence and be influenced by physical activity. The directionality of the self-regulatory efficacy (SRE) and physical activity relationship was examined using a cross-lagged design. A secondary purpose was to examine these relationships across differing weather conditions. METHODS: Canadian adolescents (N = 337; aged between 13 and 18 years) completed the physical activity and SRE measures 4 times during a school year. Structural equation modeling was used to perform a cross-lag analysis. RESULTS: The relationships between physical activity and SRE appeared to be weather dependent. During a more challenging weather period (eg, cold weather), the relationship between physical activity and SRE was bidirectional. However, no relationship emerged when the 2 constructs were assessed during a more optimal weather period (eg, warm weather). CONCLUSIONS: Some support has been provided for the bidirectional nature of the relationship between physical activity and SRE. The relationship appeared to be qualified by climate considerations, suggesting that future research examine how weather may relate not just to physical activity but also to the correlates of physical activity.

Changes in Health-Related Outcomes in Youth With Obesity in Response to a Home-Based Parent-Led Physical Activity Program.

PURPOSE: The purpose of this study was to elucidate whether implementation of a parent-led physical activity (PA) curriculum improved health parameters in youth with obesity. METHODS: This prospective study included 45 youth with Prader-Willi syndrome (PWS) and 66 youth classified as obese without PWS. Participants were quasi-randomly assigned to an intervention (I) group which completed PA sessions (25-45+ minutes long) 4 days/week for 24 weeks or to a control (C) group. Generalized estimating equations analyzed differences in body composition, PA, and health-related quality of life (HRQL) by youth group, time, and treatment group. A secondary analysis in the I-group compared outcomes based on whether youth showed increases (n = 12) or decreases (n = 19) of ≥2 minutes of moderate-to-vigorous PA (MVPA). RESULTS: Body mass index increased from baseline to 24 weeks in youth with obesity (p = .032) but not in youth with PWS. There were no changes in MVPA, total PA, or body fat indicators over time. The I-group demonstrated an increase of 7.2% and 7.6% in social and school HRQL, respectively, and a 3.3% improvement in total HRQL. Youth in the I-group who increased MVPA demonstrated decreased body mass (p = .010), body mass index z-score (p = .018), and body fat mass (p = .011); these changes were not observed in those who decreased MVPA over time. CONCLUSIONS: Participation in a parent-led PA intervention at home can positively influence HRQL in youth with obesity and/or PWS. Increases in MVPA ≥2 minutes above baseline led to decreases in body mass and fat, while maintaining lean mass.

Effectiveness of a Parent-led Physical Activity Intervention in Youth with Obesity.

PURPOSE: Prader-Willi syndrome (PWS) is a complex, rare neurobehavioral syndrome characterized by excessive fat, hypotonia, poor motor skills, and behavioral and cognitive disabilities. We tested the effectiveness of a home-based physical activity (PA) intervention led by parents in youth with obesity with and without PWS to increase moderate-to-vigorous PA (MVPA) and gross motor proficiency. METHODS: Participants were 111 youth age 8 to 16 yr (45 with PWS and 66 without PWS, but categorized as obese). A parallel design was used with the control group (C) receiving the intervention after serving as control. Intervention participants (I) completed a PA curriculum 4 d·wk for 24 wk including warm-up exercises, strengthening exercises, and playground games 2 d·wk and interactive console games 2 d·wk guided by their parents. Pre-post outcomes (baseline to 24 wk) included MVPA (7-d accelerometry) and motor proficiency including upper limb coordination, bilateral coordination, balance, running speed and agility, and muscle strength (Bruininks-Oseretsky Test of Motor Proficiency). RESULTS: The intervention led to no change in MVPA (I group, 39.6 vs 38.9 min·d; C group, 40.6 vs 38.3 min·d). The intervention led to improvements in body coordination (22.3%; P < 0.05), as well as strength and agility (13.7%; P < 0.05). Specifically, the I group showed increases in upper limb coordination (19.1%), bilateral coordination (27.8%), and muscle strength (12.9%; P < 0.05 for all) not observed in the C group: -0.2%, 2.5%, and -3.2%, respectively. CONCLUSIONS: This parent-guided PA intervention did not increase PA. However, the intervention led to improvements in gross motor skill competency. Providing families with tools and support can lead to implementation of PA routines that contribute to motor skill proficiency in youth with and without PWS.

Disentangling the Relations between Social Identity and Prosocial and Antisocial Behavior in Competitive Youth Sport.

The social identities formed through membership on extracurricular activity groups may contribute to the frequency with which youth engage in prosocial and antisocial behavior. However, researchers have yet to disentangle the individual- and group-level processes social identification effects operate through; sex and perceived norms may also moderate such effects. Thus, we investigated the hierarchical and conditional relations between three dimensions of social identity (i.e., ingroup ties, cognitive centrality, ingroup affect) and prosocial and antisocial behavior in youth ice hockey players (N = 376; 33% female). Multilevel analyses demonstrated antisocial teammate and opponent behavior were predicted by cognitive centrality at the team level. Further, prosocial teammate behavior was predicted by cognitive centrality and ingroup ties at the individual-level. Also, perceived norms for prosocial teammate behavior moderated the relations between ingroup ties, cognitive centrality, and ingroup affect and prosocial teammate behaviour. Finally, sex moderated the relations between cognitive centrality/ingroup affect and antisocial opponent behavior. This work demonstrates the multilevel and conditional nature of how social identity dimensions relate to youth prosocial and antisocial behavior.

Quality of life in children with Prader Willi Syndrome: Parent and child reports.

PURPOSE: The purpose of this study was to evaluate the use of the Peds QL4.0 instrument to assess quality of life (QL) in children with Prader Willi Syndrome (PWS). This study also sought to compare differences in parent and child report as well as between children with PWS and without PWS. METHODS: Parents and children with PWS (N=44) completed the PedsQL 4.0 instrument. A sub-sample of children completed the Peds QL 4.0 a second time to assess test-retest reliability. A comparison sample of children who were obese but without PWS (N=66) also completed the PedsQL 4.0. RESULTS: PedsQL 4.0 showed acceptable internal consistency for the child report (αs >0.72) and was acceptable for 4 out of the 6 scales for the parent report (αs >0.66). Test-retest reliability coefficients showed support for the reliability of the instrument (ICCs>0.64). Parents perceived lower QL than children with PWS. Children with PWS also showed lower QL than children without PWS. CONCLUSIONS: This study provides support for the use of the PedsQL 4.0 instrument in children with PWS. As observed in other populations, parents perceive a lower QL for their children with PWS than the children themselves.

JAK-STAT6 Pathway Inhibitors Block Eotaxin-3 Secretion by Epithelial Cells and Fibroblasts from Esophageal Eosinophilia Patients: Promising Agents to Improve Inflammation and Prevent Fibrosis in EoE.

BACKGROUND: Although most studies on treatments for eosinophilic esophagitis (EoE) have focused on effects in the epithelium, EoE is a transmural disease. Eosinophils that infiltrate the subepithelial layers of the esophagus lead to fibrosis and the serious complications of EoE, and current therapies have shown minimal effects on this fibrosis. We aimed to elucidate T helper (Th)2 cytokine effects on esophageal fibroblasts and to explore potential fibroblast-targeted therapies for EoE. METHODS: We established telomerase-immortalized fibroblasts from human esophageal biopsies. We stimulated these esophageal fibroblasts with Th2 cytokines, and examined effects of omeprazole and inhibitors of the Janus kinase (JAK)-signal transducer and activator of transcription (STAT6) pathway (AS1517499, leflunomide, and ruxolitinib) on STAT6 phosphorylation, STAT6 nuclear translocation, and eotaxin-3 expression. We also measured the effects of these inhibitors in esophageal epithelial cells stimulated with Th2 cytokines. RESULTS: As in esophageal epithelial cells, Th2 cytokines increased STAT6 phosphorylation, STAT6 nuclear translocation, eotaxin-3 transcription and protein secretion in esophageal fibroblasts. Unlike in epithelial cells, however, omeprazole did not inhibit cytokine-stimulated eotaxin-3 expression in fibroblasts. In contrast, JAK-STAT6 pathway inhibitors decreased cytokine-stimulated eotaxin-3 expression in both fibroblasts and epithelial cells. CONCLUSIONS: Omeprazole does not inhibit Th2 cytokine-stimulated eotaxin-3 expression by esophageal fibroblasts, suggesting that PPIs will have limited impact on subepithelial EoE processes such as fibrosis. JAK-STAT6 pathway inhibitors block Th2 cytokine-stimulated eotaxin-3 expression both in fibroblasts and in epithelial cells, suggesting a potential role for JAK-STAT inhibitors in treating both epithelial inflammation and subepithelial fibrosis in EoE.

Association between physical activity and bone in children with Prader-Willi syndrome.

BACKGROUND: The aim of the study was to determine if physical activity (PA) is associated with bone health in children with Prader-Willi syndrome (PWS). METHODS: Participants included 23 children with PWS (age: 11.0±2.0 years). PA, measured by accelerometry, was categorized into light, moderate, vigorous and moderate plus vigorous intensities. Hip, total body minus the head (body), bone mineral content (BMC), bone mineral density (BMD) and BMD z-score (BMDz) were measured by dual X-ray absorptiometry. Separate hierarchical regression models were completed for all bone parameters, PA intensity and select covariates. RESULTS: Moderate PA and select covariates explained the most variance in hip BMC (84.0%), BMD (61.3%) and BMDz (34.9%; p<0.05 for all). Likewise, for each body parameter, moderate PA and select covariates explained the most variance in body BMC (75.8%), BMD (74.4%) and BMDz (31.8%; p<0.05 for all). CONCLUSIONS: PA of at least moderate intensity appears important for BMC and BMD in children with PWS.

Novel r(2)(p25q31) cytogenetic abnormality in a pediatric patient with acute leukemia of ambiguous lineage.

We describe a case of acute leukemia of ambiguous lineage with a novel cytogenetic abnormality. A 1-year-old boy presented with abnormal complete blood count findings, and was found to have blasts and mild dysgranulopoiesis. The blasts showed immunophenotypic evidence of myeloid and T-lineage differentiation. Subsequent cytogenetic analysis showed r(2)(p25q31) as the sole stem line cytogenetic defect with clonal evolution. While cytogenetic abnormalities can have a critical role in the classification and prognostication of acute lymphoblastic and acute myeloid leukemia, the significance of cytogenetic abnormalities in acute leukemia of ambiguous lineage remains unclear. This finding has not been reported previously to the best of our knowledge.

Patterns of habitual physical activity in youth with and without Prader-Willi Syndrome.

Children classified as overweight or obese and those with disabilities are at a greater risk of not meeting the minimum recommendation of 60 min a day of moderate to vigorous physical activity (PA). Youth with Prader-Willi Syndrome (PWS) appear to participate in less PA compared to nonsyndromal children, likely due to syndrome-related factors. However, description of PA patterns in youth with PWS is lacking. The purpose of this study was to characterize PA in youth with PWS and to compare it to PA in children with nonsyndromal obesity. Twenty-four youth with PWS (ages 8-16 years) and 40 obese children without PWS (OB) (ages 8-11 years) wore accelerometers for eight consecutive days. Data were screened for compliance and classified into PA intensities: sedentary behavior (SED), light (LPA), moderate (MPA), vigorous (VPA) and moderate plus vigorous (MVPA). Youth with PWS spent 19.4% less time in weekly LPA (p=0.007) and 29.8% less time in weekly VPA compared to OB controls (p=0.036). All other intensities were similar between groups. In addition, PWS participated in less LPA and VPA during the weekends compared to OB, and less LPA on weekdays when compared to OB. There was also a trend towards PWS participating in less MVPA during the weekends and less VPA during the weekends than OB controls. There was a trend towards PWS participating in less VPA on weekends compared to weekdays, while OB participated similarly in VPA on weekdays and weekend days. On average, neither PWS nor OB children met minimum MVPA recommendations. The results suggest there is a need to design exercise programs for PWS youth that focus on integrating vigorous intensity activities, especially during the weekends when structured PA may not be available.

Rationale and design of active play @ home: a parent-led physical activity program for children with and without disability.

BACKGROUND: Compared to other children, those with disability have additional challenges to being physically active. Prader-Willi Syndrome is a genetic form of childhood obesity that is characterized by hypotonia, growth hormone deficiency, behavioral, and cognitive disability. In children, the low prevalence of this syndrome (1 in 10,000 to 15,000 live births) makes group-based physical activity interventions difficult. In contrast, the home environment presents a natural venue to establish a physical activity routine for this population. This manuscript describes the design of a parent-led physical activity intervention incorporating playground and interactive console-based games to increase physical activity participation in youth with and without Prader-Willi Syndrome. METHODS/DESIGN: The study participants will be 115 youth ages 8-15 y (45 with the syndrome and 70 without the syndrome but categorized as obese). The study will use a parallel design with the control group receiving the intervention after serving as control. Participants will be expected to complete a physical activity curriculum 4 days a week for 6 months including playground games 2 days a week and interactive console games 2 days a week. Parents will be trained at baseline and then provided with a curriculum and equipment to guide their implementation of the program. Tips related to scheduling and coping with barriers to daily program implementation will be provided. Throughout, parents will be contacted by phone once a week (weeks 1-4) and then every other week to receive support in between visits. Measurements of children and parents will be obtained at baseline, 12 weeks, and at the end (week 24) of the intervention. Children main outcomes include physical activity (accelerometry), body composition (dual x-ray absorptiometry), motor proficiency (Bruininks-Oseretsky Test of Motor Proficiency), quality of life and physical activity self-efficacy (questionnaires). Intervention compliance will be monitored using mail-in daily self-report checklists. DISCUSSION: This parent-guided physical activity intervention aims to increase physical activity by using a curriculum that builds physical activity related self-confidence through the development and/or enhancement of motor skill competency. Ultimately, helping children develop these skills as well as joy in being physically active will translate into sustained behavior change. TRIAL REGISTRATION: Current Controlled Trial: NCT02058342.