U.S. Physical Activity Para Report Card for Children and Adolescents With Disabilities.

The U.S. Report Card on Physical Activity for Children and Youth has tracked 10 physical activity (PA) indicators common to the Active Healthy Kids Global Matrix since 2014. This article expands on the U.S. report cards by presenting PA indicator assessments among children and adolescents with disabilities. Grades for indicators were assigned based on a search of peer-reviewed articles presenting nationally representative data. The Global Matrix 3.0 benchmarks and grading framework guided the process. Grades for overall PA, sedentary behaviors, organized sports, and school were F, D+, D+, and D, respectively. Insufficient evidence existed to assign grades to the remaining six indicators. There is a need in the United States for targeted PA promotion strategies that are specific to children and adolescents with disabilities. Without a commitment to this effort across sectors and settings, the low grades identified in this para report card are expected to remain.

Multiple mechanisms disrupt the let-7 microRNA family in neuroblastoma.

Poor prognosis in neuroblastoma is associated with genetic amplification of MYCN. MYCN is itself a target of let-7, a tumour suppressor family of microRNAs implicated in numerous cancers. LIN28B, an inhibitor of let-7 biogenesis, is overexpressed in neuroblastoma and has been reported to regulate MYCN. Here we show, however, that LIN28B is dispensable in MYCN-amplified neuroblastoma cell lines, despite de-repression of let-7. We further demonstrate that MYCN messenger RNA levels in amplified disease are exceptionally high and sufficient to sponge let-7, which reconciles the dispensability of LIN28B. We found that genetic loss of let-7 is common in neuroblastoma, inversely associated with MYCN amplification, and independently associated with poor outcomes, providing a rationale for chromosomal loss patterns in neuroblastoma. We propose that let-7 disruption by LIN28B, MYCN sponging, or genetic loss is a unifying mechanism of neuroblastoma development with broad implications for cancer pathogenesis.

Characterization of clinically relevant copy-number variants from exomes of patients with inherited heart disease and unexplained sudden cardiac death.

PURPOSE: Copy-number variant (CNV) analysis is increasingly performed in genetic diagnostics. We leveraged recent gene curation efforts and technical standards for interpretation and reporting of CNVs to characterize clinically relevant CNVs in patients with inherited heart disease and sudden cardiac death. METHODS: Exome sequencing data were analyzed for CNVs using eXome-Hidden Markov Model tool in 48 established disease genes. CNV breakpoint junctions were characterized. CNVs were classified using the American College of Medical Genetics and Genomics technical standards. RESULTS: We identified eight CNVs in 690 unrelated probands (1.2%). Characterization of breakpoint junctions revealed nonhomologous end joining was responsible for four deletions, whereas one duplication was caused by nonallelic homologous recombination between duplicated sequences in MYH6 and MYH7. Identifying the precise breakpoint junctions determined the genomic involvement and proved useful for interpreting the clinical relevance of CNVs. Three large deletions involving TTN, MYBPC3, and KCNH2 were classified as pathogenic in three patients. Haplotype analysis of a deletion in ACTN2, found in two families, suggests the deletion was caused by an ancestral event. CONCLUSION: CNVs infrequently cause inherited heart diseases and should be investigated when standard genetic testing does not reveal a genetic diagnosis.

Physical Activity, Medical Home, and Health Behavior Counseling Among Adolescents with Special Health Care Needs: NSCH 2016-2017.

OBJECTIVES: Aim 1 was to establish updated prevalence estimates for meeting national physical activity (PA) guidelines among adolescents with and without special healthcare needs (SHCN), 12-17 years old. To identify at-risk subgroups, our sub-aim was to compare the distribution of prevalence estimates across PA levels by SHCN subtypes, and in reference to peers without SHCN. Aim 2 was to examine the association between meeting PA guidelines, having a medical home, and receiving positive health behavior counseling in this population. METHODS: Weighted prevalence estimates for meeting the 2018 National PA Guidelines (inactive, insufficiently active, sufficiently active: guidelines met) were calculated from a secondary analysis of the National Survey of Children's Health 2016-2017 ( n = 16,171, 27% SHCN). Adjusted odds ratios and 95% CIs were estimated from logistic regression models to measure the association between PA, medical home, and postive health behavior counseling. RESULTS: Of adolescents with SHCN, 15% were reported to be meeting PA guidelines compared to 19% of peers without SHCN peers. Among adolescents with a medical home, regardless of SHCN status, those receiving positive health behavior counseling had 1.70 times the adjusted odds of meeting PA guidelines compared to peers without counseling. CONCLUSIONS FOR PRACTICE: Adolescents with and without SHCN were more likely to meet PA guidelines if they had a medical home and received positive health behavior counseling, highlighting the value of comprehensive healthcare practices for PA promotion.

A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults.

AIMS: To assess the reported prevalence of left ventricular non-compaction (LVNC) in different adult cohorts, taking in to consideration the role of diagnostic criteria and imaging modalities used. METHODS AND RESULTS: A systematic review and meta-analysis of studies reporting LVNC prevalence in adults. Studies were sourced from Pre-Medline, Medline, and Embase and assessed for eligibility according to inclusion criteria. Eligible studies provided a prevalence of LVNC in adult populations (≥12 years). Studies were assessed, and data extracted by two independent reviewers. Fifty-nine eligible studies documenting LVNC in 67 unique cohorts were included. The majority of studies were assessed as moderate or high risk of bias. The pooled prevalence estimates for LVNC were consistently higher amongst cohorts diagnosed on cardiac magnetic resonance (CMR) imaging (14.79%, n = 26; I2 = 99.45%) compared with echocardiogram (1.28%, n = 36; I2 = 98.17%). This finding was unchanged when analysis was restricted to studies at low or moderate risk of bias. The prevalence of LVNC varied between disease and population representative cohorts. Athletic cohorts demonstrated high pooled prevalence estimates on echocardiogram (3.16%, n = 5; I2 = 97.37%) and CMR imaging (27.29%, n = 2). CONCLUSION: Left ventricular non-compaction in adult populations is a poorly defined entity which likely encompasses both physiological adaptation and pathological disease. There is a higher prevalence with the introduction of newer imaging technologies, specifically CMR imaging, which identify LVNC changes more readily. The clinical significance of these findings remains unclear; however, there is significant potential for overdiagnosis, overtreatment, and unnecessary follow-up.

Exploring the Interaction of Disability Status and Childhood Predictors of Physical Activity and Sport Participation: An Exploratory Decision-Tree Analysis.

A secondary data analysis of 33,093 children and adolescents age 6-17 years (12% with disabilities) from a 2016-2017 National Survey of Children's Health nonrepresentative sample aimed to identify (a) unique clusters of sociodemographic characteristics and (b) the relative importance of disability status in predicting participation in daily physical activity (PA) and sports. Exploratory classification tree analyses identified hierarchical predictors of daily PA and sport participation separately. Disability status was not a primary predictor of daily PA. Instead, it emerged in the fifth level after age, sex, body mass index, and income, highlighting the dynamic intersection of disability with sociodemographic factors influencing PA levels. In comparison, disability status was a second-level predictor for sport participation, suggesting that unique factors influencing PA level are likely experienced by disabled children and adolescents. The authors employ an intersectionality lens to critically discuss implications for research in adapted PA.

Left Ventricular Non-Compaction: Review of the Current Diagnostic Challenges and Consequences in Athletes.

Left ventricular non-compaction (LVNC) is a complex clinical condition with no diagnostic gold standard. At present, there is trepidation about the accuracy of the diagnosis, the correlation to clinical outcomes and the long-term medical management. This article reviews the current imaging criteria, the limitations of echocardiography and cardiac magnetic resonance and the consequences of LV hypertrabeculation in athletes.

Means-end problem solving in infancy: Development, emergence of intentionality, and transfer of knowledge.

Behaviors and performance of 23 typically developing infants were assessed longitudinally at 6, 9, 12, 18, and 24 months in two means-end tasks: pulling a towel or rotating a turntable to obtain a supported object. With age, infants performed more goal-directed behaviors, leading to increased problem-solving success. Intentionality emerged earlier in the towel task than in the turntable task (6.9 vs. 10.8 months). Potential knowledge transfer between the tasks was first observed at 9 months. This study provides insight into the development of means-end learning, the emergence of intentionality, and potential transfer of knowledge in tasks involving a similar concept (support) but requiring different modes of action for success (pulling vs. rotating).

Interferon-α signaling promotes embryonic HSC maturation.

In the developing mouse embryo, the first hematopoietic stem cells (HSCs) arise in the aorta-gonad-mesonephros (AGM) and mature as they transit through the fetal liver (FL). Compared with FL and adult HSCs, AGM HSCs have reduced repopulation potential in irradiated adult transplant recipients but mechanisms underlying this deficiency in AGM HSCs are poorly understood. By co-expression gene network analysis, we deduced that AGM HSCs show lower levels of interferon-α (IFN-α)/Jak-Stat1-associated gene expression than FL HSCs. Treatment of AGM HSCs with IFN-α enhanced long-term hematopoietic engraftment and donor chimerism. Conversely, IFN-α receptor-deficient AGMs (Ifnαr1(-/-)), had significantly reduced donor chimerism. We identify adenine-thymine-rich interactive domain-3a (Arid3a), a factor essential for FL and B lymphopoiesis, as a key transcriptional co-regulator of IFN-α/Stat1 signaling. Arid3a occupies the genomic loci of Stat1 as well as several IFN-α effector genes, acting to regulate their expression. Accordingly, Arid3a(-/-) AGM HSCs had significantly reduced transplant potential, which was rescued by IFN-α treatment. Our results implicate the inflammatory IFN-α/Jak-Stat pathway in the developmental maturation of embryonic HSCs, whose manipulation may lead to increased potency of reprogrammed HSCs for transplantation.

High definition transcranial pink noise stimulation of anterior cingulate cortex on food craving: An explorative study.

Dysfunctional neural activity in the cortical reward system network has been implicated in food addiction. This is the first study exploring the potential therapeutic effects of high definition transcranial pink noise stimulation (HD-tPNS) targeted at the anterior cingulate cortex (ACC) on craving and brain activity in women with obesity who showed features of food addiction (Yale Food Addiction Scale score of ≥3). Sixteen eligible females participated in a randomized, double-blind, parallel group study. Participants received six 20-minute sessions of either 1 mA (n = 8) or sham (n = 8) stimulation with HD-tPNS over two weeks. Anode was placed above the ACC (Fz) with 4 cathodes (F7, T3, F8, and T4). Food craving was assessed using the Food Cravings Questionnaire State (FCQ-S) and brain activity was measured using electroencephalogram (EEG). Assessments were at baseline, and two days, four weeks, and six weeks after stimulation. A 22% decrease (mean decrease of -1.11, 95% CI -2.09, -0.14) was observed on the 5-point 'intense desire to eat' subscale two days after stimulation in the HD-tPNS group compared to sham. Furthermore, whole brain analysis showed a significant decrease in beta 1 activity in the ACC in the stimulation group compared to sham (threshold 0.38, p = 0.04). These preliminary findings suggest HD-tPNS of the ACC transiently inhibits the desire to eat and, thus, warrants further examination as a potential tool in combating food craving.

Patient-Centered Medical Home and Receipt of Part C Early Intervention Among Young CSHCN and Developmental Disabilities Versus Delays: NS-CSHCN 2009-2010.

Objective To determine, among a sample of young CSHCN with developmental conditions, (1) characteristics associated with receipt of both patient-centered medical home (PCMH) and Part C early intervention, (2) the association between each PCMH criterion and receipt of Part C generally, and (3) for CSHCN with disabilities versus delays. Methods Secondary data analysis of the 2009/10 National Survey of CSHCN. Sample included CSHCN (n = 755) birth to 3 years with a developmental disability or delay that affected their function. Adjusted ordinal regression analysis examined characteristics associated with receiving both PCMH and Part C. Stratified adjusted logistic regression examined the association between PCMH criteria and Part C, by disabilities versus delays. Results 19% of our sample received both PCMH and Part C. Black, non-Hispanic children had lower odds [OR 0.44, 95% CI (0.20, 0.97)] and CSHCN with more severe developmental conditions had higher odds [OR 2.13, 95% CI (1.22, 3.17)] of receiving both services. CSHCN with a PCMH were no more likely to be receiving Part C than those without a PCMH [OR 0.85, 95% CI (0.49, 1.49)]. Receiving any one of the PCMH criterion was not associated with receiving Part C, with one exception. Among CSHCN with delays, effective care coordination was associated with lower odds of Part C [OR 0.46, 95% CI (0.21, 0.97)]. Conclusion Concurrent PCMH and Part C access was low for young CSHCN with developmental conditions affecting their function. Given the overlapping mandates for PCMH and Part C, integrated efforts are warranted to identify if lack of concurrent services in fact reflects unmet service needs.

Fundamental motor skills: A systematic review of terminology.

The three aims of this systematic review are to describe: (1) use of the term fundamental motor/movement skills (FMS) in published articles; (2) the quality of definitions; and (3) relative use of process- and product- oriented assessments to measure FMS. The inclusion criteria included: (a) peer-reviewed article, (b) printed in English, (c) published between January 2000 and 31 December 2015, (d) presence of either the term "fundamental motor or movement skill" in the title and/or abstract, and (e) FMS were a measured outcome. There has been an increase in the number of publications on FMS in recent years, with the majority of studies conducted in Australia (n = 41, 33%). Approximately 24% of studies (n = 30) did not provide any explicit definition of FMS. A majority of studies reported the use of process-oriented measures (n = 98, 79%) compared to product-oriented measures (n = 23, 19%), and few studies used both (n = 6, 5%). We recommend that researchers provide: (1) an operational definition of FMS that states FMS are the "building blocks" (or similar terminology) of more advanced, complex movements; (2) specific categories of skills that compose FMS; and (3) at least one specific example of a FMS.

Feasibility of a Modified Ride-on Car Intervention on Play Behaviors during an Inclusive Playgroup.

AIMS: Children with mobility related disabilities often experience limited participation and access to social interactions. An emerging pediatric powered mobility device are modified ride-on cars that provide self-directed mobility experiences to children with disabilities. This study aimed to determine: (1) the feasibility of a modified ride-on car intervention during an inclusive playgroup, (2) the effect of a modified ride-on car intervention on the play behaviors of children with and without mobility related disabilities. METHOD: A single-subject research design was implemented. Thirteen children participated in a weekly inclusive playgroup. The five children with mobility related disabilities were provided modified ride-on cars during the intervention. Children's play behaviors were classified with Howes' Peer Play Scale. Intervention effects were examined using nonoverlap of all pairs (NAP). RESULTS: The intervention was feasible based on participants' good attendance, retention rates, and successful use of modified ride-on cars. Overall children did not experience significant changes in play behaviors, with a few exceptions for decreased solitary, and increased parallel play, and/or direct peer interaction, among children with mobility related disabilities. Future research could examine modified ride-on car use by children with mobility related disabilities focusing on changes in unique play interactions between children with and without disabilities.

Relations of Early Motor Skills on Age and Socialization, Communication, and Daily Living in Young Children With Developmental Disabilities.

Young children with developmental disabilities experience known deficits in salient child behaviors, such as social behaviors, communication, and aspects of daily living, behaviors that generally improve with chronological age. The purpose of this study was to examine the mediating effects of motor skills on relations of age and salient child behaviors in a group of young children with developmental disabilities, thus tapping into the potential influences of motor skills in the development of salient child behaviors. One hundred thirteen young children with developmental disabilities participated in this study. Independent mediation analysis, with gender as a moderator between the mediating and outcome variable, indicated that motor skills meditated relations between age and socialization, communication, and daily living skills in young male children with developmental disabilities, but not female participants. Findings suggest motor skill content needs to be considered in combination with other child behaviors commonly focused on in early intervention.

Associations between 24-h movement guidelines compliance and anxiety and depression among youth receiving special education services in the US.

BACKGROUND: Youth with disabilities are five times more likely to experience anxiety or depression than peers without disabilities. Engagement in sufficient daily physical activity (PA), adequate nightly sleep, and limited daily screen time (collectively known as 24-h movement guidelines) is associated with lower odds of anxiety and depression for peers without disabilities. Extending the investigation of these modifiable behaviors to youth with disabilities is warranted. OBJECTIVE: To estimate the association between meeting 24-h movement guidelines and anxiety and depression among a nationally representative sample of youth with disabilities. METHODS: A cross-sectional secondary analysis of the 2019-2020 NSCH was conducted and included youth 6-17 years old who were currently receiving special education services. Weighted prevalence estimates and logistic regressions were employed to estimate the association between meeting guidelines (separately and combined) and current anxiety or depression status. RESULTS: Compared to youth with disabilities who met the guideline, those not meeting the sleep or screen time guidelines, independently, had significantly higher odds of depression, or anxiety (aOR range 1.53, 2.31 respectively). Comparable odds were observed between those meeting the PA guidelines, alone or in combination, and those meeting none of the 24-h movement guidelines. CONCLUSION: Adequate nightly sleep, and limited screen time, were significantly associated with anxiety and depression among youth with disabilities, a pattern consistent to peers without disabilities. Yet, meeting more than one guideline did not further reduce odds of poor mental health, warranting further investigation of compounding benefits of the 24-h guidelines within this population.