Exome and genome sequencing for rare genetic disease diagnosis: A scoping review and critical appraisal of clinical guidance documents produced by genetics professional organizations.

PURPOSE: Exome and genome sequencing have rapidly transitioned from research methods to widely used clinical tests for diagnosing rare genetic diseases. We sought to synthesize the topics covered and appraise the development processes of clinical guidance documents generated by genetics professional organizations. METHODS: We conducted a scoping review of guidance documents published since 2010, systematically identified in peer-reviewed and gray literature, using established methods and reporting guidelines. We coded verbatim recommendations by topic using content analysis and critically appraised documents using the Appraisal of Guidelines Research and Evaluation (AGREE) II tool. RESULTS: We identified 30 guidance documents produced by 8 organizations (2012-2022), yielding 611 recommendations covering 21 topics. The most common topic related to findings beyond the primary testing indication. Mean AGREE II scores were low across all 6 quality domains; scores for items related to rigor of development were among the lowest. More recently published documents generally received higher scores. CONCLUSION: Guidance documents included a broad range of recommendations but were of low quality, particularly in their rigor of development. Developers should consider using tools such as AGREE II and basing recommendations on living knowledge syntheses to improve guidance development in this evolving space.

Searching for evidence in public health emergencies: a white paper of best practices.

Objectives: Information professionals have supported medical providers, administrators and decision-makers, and guideline creators in the COVID-19 response. Searching COVID-19 literature presented new challenges, including the volume and heterogeneity of literature and the proliferation of new information sources, and exposed existing issues in metadata and publishing. An expert panel developed best practices, including recommendations, elaborations, and examples, for searching during public health emergencies. Methods: Project directors and advisors developed core elements from experience and literature. Experts, identified by affiliation with evidence synthesis groups, COVID-19 search experience, and nomination, responded to an online survey to reach consensus on core elements. Expert participants provided written responses to guiding questions. A synthesis of responses provided the foundation for focus group discussions. A writing group then drafted the best practices into a statement. Experts reviewed the statement prior to dissemination. Results: Twelve information professionals contributed to best practice recommendations on six elements: core resources, search strategies, publication types, transparency and reproducibility, collaboration, and conducting research. Underlying principles across recommendations include timeliness, openness, balance, preparedness, and responsiveness. Conclusions: The authors and experts anticipate the recommendations for searching for evidence during public health emergencies will help information specialists, librarians, evidence synthesis groups, researchers, and decision-makers respond to future public health emergencies, including but not limited to disease outbreaks. The recommendations complement existing guidance by addressing concerns specific to emergency response. The statement is intended as a living document. Future revisions should solicit input from a broader community and reflect conclusions of meta-research on COVID-19 and health emergencies.

Balanced Versus Unbalanced Fluid in Critically Ill Children: Systematic Review and Meta-Analysis.

OBJECTIVES: The ideal crystalloid fluid bolus therapy for fluid resuscitation in children remains unclear, but pediatric data are limited. Administration of 0.9% saline has been associated with hyperchloremic metabolic acidosis and acute kidney injury. The primary objective of this systematic review was to compare the effect of balanced versus unbalanced fluid bolus therapy on the mean change in serum bicarbonate or pH within 24 hours in critically ill children. DATA SOURCES: We searched MEDLINE including Epub Ahead of Print, In-Process & Other Non-Indexed Citations, Embase, CENTRAL Trials Registry of the Cochrane Collaboration, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform. STUDY SELECTION: Using the Preferred Reporting Items for Systematic Review and Meta-analysis Protocols guidelines, we retrieved all controlled trials and observational cohort studies comparing balanced and unbalanced resuscitative fluids in critically ill children. The primary outcome was the change in serum bicarbonate or blood pH. Secondary outcomes included the prevalence of hyperchloremia, acute kidney injury, renal replacement therapy, and mortality. DATA EXTRACTION: Study screening, inclusion, data extraction, and risk of bias assessments were performed independently by two authors. DATA SYNTHESIS: Among 481 references identified, 13 met inclusion criteria. In the meta-analysis of three randomized controlled trials with a population of 162 patients, we found a greater mean change in serum bicarbonate level (pooled estimate 1.60 mmol/L; 95% CI, 0.04-3.16; p = 0.04) and pH level (pooled mean difference 0.03; 95% CI, 0.00-0.06; p = 0.03) after 4-12 hours of rehydration with balanced versus unbalanced fluids. No differences were found in chloride serum level, acute kidney injury, renal replacement therapy, or mortality. CONCLUSIONS: Our systematic review found some evidence of improvement in blood pH and bicarbonate values in critically ill children after 4-12 hours of fluid bolus therapy with balanced fluid compared with the unbalanced fluid. However, a randomized controlled trial is needed to establish whether these findings have an impact on clinical outcomes before recommendations can be generated.

Consent models in Canadian critical care randomized controlled trials: a scoping review.

PURPOSE: Our primary objective was to describe consent models used in Canadian-led adult and pediatric intensive care unit (ICU/PICU) randomized controlled trials (RCTs). Our secondary objectives were to determine the consent rate of ICU/PICU RCTs that did and did not use an alternate consent model to describe consent procedures. SOURCE: Using scoping review methodology, we searched MEDLINE, Embase, and CENTRAL databases (from 1998 to June 2019) for trials published in English or French. We included Canadian-led RCTs that reported on the effects of an intervention on ICU/PICU patients or their families. Two independent reviewers assessed eligibility, abstracted data, and achieved consensus. PRINCIPAL FINDINGS: We identified 48 RCTs of 17,558 patients. Included RCTs had ethics approval to use prior informed consent (43/48; 90%), deferred consent (13/48; 27%), waived consent (5/48; 10%), and verbal consent (1/48; 2%) models. Fifteen RCTs (15/48; 31%) had ethics approval to use more than one consent model. Twice as many trials used alternate consent between 2010 and 2019 (13/19) than between 2000 and 2009 (6/19). The consent rate for RCTs using only prior informed consent ranged from 54 to 91% (ICU) and 43 to 94% (PICU) and from 78 to 100% (ICU) and 74 to 87% (PICU) in trials using an alternate/hybrid consent model. CONCLUSION: Alternate consent models were used in the minority of Canadian-led ICU/PICU RCTs but have been used more frequently over the last decade. This suggests that Canadian ethics boards and research communities are becoming more accepting of alternate consent models in ICU/PICU trials.

RéSUMé: OBJECTIF: Notre objectif principal était de décrire les modèles de consentement utilisés dans les études randomisées contrôlées (ERC) menées par des chercheurs canadiens dans les unités de soins intensifs adultes et pédiatriques (USI/USIP). Nos objectifs secondaires étaient de déterminer le taux de consentement aux ERC à l'USI et l'USIP qui utilisaient et n'utilisaient pas un autre modèle de consentement pour décrire les processus de consentement. SOURCES: À l'aide d'une méthodologie d'étude de portée, nous avons effectué des recherches dans les bases de données MEDLINE, Embase et CENTRAL (de 1998 à juin 2019) pour en tirer les études publiées en anglais ou en français. Nous avons inclus des ERC dirigées par des chercheurs canadiens qui rapportaient les effets d'une intervention sur les patients à l'USI/USIP ou leurs familles. Deux examinateurs indépendants ont évalué l'admissibilité, résumé les données et atteint un consensus. RéSULTATS PRINCIPAUX: Nous avons identifié 48 ERC portant sur 17 558 patients. Les ERC incluses avaient obtenu l'approbation du comité d'éthique pour l'utilisation de modèles de consentement éclairé préalable (43/48; 90 %), de consentement différé (13/48; 27 %), de renoncement au consentement (5/48; 10 %) et de consentement verbal (1/48; 2 %). Quinze ERC (15/48; 31 %) avaient reçu l'approbation du comité d'éthique pour utiliser plus d'un modèle de consentement. Deux fois plus d'études ont utilisé un autre type de consentement entre 2010 et 2019 (13/19) qu'entre 2000 et 2009 (6/19). Le taux de consentement pour les ERC utilisant uniquement un consentement éclairé préalable variait de 54 à 91 % (USI) et de 43 à 94 % (USIP), contre 78 à 100 % (USI) et 74 à 87 % (USIP) pour les études utilisant un modèle de consentement alternatif/hybride. CONCLUSION: Des modèles de consentement alternatif ont été utilisés dans une minorité des ERC en USI/USIP dirigées par des chercheurs canadiens, mais ils ont été utilisés plus fréquemment au cours de la dernière décennie. Cela donne à penser que les comités d'éthique et les communautés de recherche canadiens acceptent de plus en plus les modèles de consentement alternatifs dans les études réalisées en USI et en USIP.

Evaluating the relationship between citation set size, team size and screening methods used in systematic reviews: a cross-sectional study.

BACKGROUND: Standard practice for conducting systematic reviews (SRs) is time consuming and involves the study team screening hundreds or thousands of citations. As the volume of medical literature grows, the citation set sizes and corresponding screening efforts increase. While larger team size and alternate screening methods have the potential to reduce workload and decrease SR completion times, it is unknown whether investigators adapt team size or methods in response to citation set sizes. Using a cross-sectional design, we sought to understand how citation set size impacts (1) the total number of authors or individuals contributing to screening and (2) screening methods. METHODS: MEDLINE was searched in April 2019 for SRs on any health topic. A total of 1880 unique publications were identified and sorted into five citation set size categories (after deduplication): < 1,000, 1,001-2,500, 2,501-5,000, 5,001-10,000, and > 10,000. A random sample of 259 SRs were selected (~ 50 per category) for data extraction and analysis. RESULTS: With the exception of the pairwise t test comparing the under 1000 and over 10,000 categories (median 5 vs. 6, p = 0.049) no statistically significant relationship was evident between author number and citation set size. While visual inspection was suggestive, statistical testing did not consistently identify a relationship between citation set size and number of screeners (title-abstract, full text) or data extractors. However, logistic regression identified investigators were significantly more likely to deviate from gold-standard screening methods (i.e. independent duplicate screening) with larger citation sets. For every doubling of citation size, the odds of using gold-standard screening decreased by 15 and 20% at title-abstract and full text review, respectively. Finally, few SRs reported using crowdsourcing (n = 2) or computer-assisted screening (n = 1). CONCLUSIONS: Large citation set sizes present a challenge to SR teams, especially when faced with time-sensitive health policy questions. Our study suggests that with increasing citation set size, authors are less likely to adhere to gold-standard screening methods. It is possible that adjunct screening methods, such as crowdsourcing (large team) and computer-assisted technologies, may provide a viable solution for authors to complete their SRs in a timely manner.

Patient-reported Outcome Measures in Pediatric Non-Malignant Hematology: A Systematic Review.

Patient-reported outcome measures (PROMs) are questionnaires completed by patients or caregivers without influence by health care professionals. As such, PROMs show subjective health experiences, enhance the clinical information available to providers, and inform clinical action. The objective of this systematic review is to identify and list which validated PROMs have been used to monitor health-related quality of life in pediatric patients with nonmalignant hematology (hemophilia, immune thrombocytopenia, sickle cell disease, and thalassemia). Databases (MEDLINE, Embase, HaPI, CINAHL, and PsycTESTS) were searched to identify publications that validated or used PROMs as an outcome measure in the 4 disease groups. Overall, 209 articles met the inclusion criteria, identifying 113 PROMs. Of the 113 identified PROMs, 95 are generic and can be used in multiple disease groups. The Pediatric Quality of Life Generic Core Scales was the most frequently used generic PROM (68 studies). The 18 remaining PROMs were disease specific. The results of this review, together with the COSMIN tool for selecting outcome measures, will allow clinicians to evaluate the PROMs that are best suited to their patient population. In addition, the focus groups are currently being conducted with patients, parents, and clinicians to determine the optimal use of PROMs in the clinical environment.

What is the risk of recurrent concussion in children and adolescents aged 5-18 years? A systematic review and meta-analysis.

OBJECTIVE: We aimed to examine the risk of concussion in children with a previous history of concussion. DESIGN: Systematic review and meta-analysis. The primary outcome was number of children with and without a previous lifetime history of concussion who sustained a diagnosed concussion within each study period. Risk of bias was assessed using the Newcastle-Ottawa Scale. A random effects model was used to estimate a pooled risk ratio (RR) with corresponding 95% CIs; results were summarised in forest plots. DATA SOURCES: Four electronic databases (MEDLINE, Embase, CINAHL, SPORTDiscus) and selected reference lists were searched (PROSPERO registration No CRD42019135462). ELIGIBILITY CRITERIA: Original English language peer-reviewed publications that compared concussion risk in children aged 5-18 years with and without a previous concussion history in which risk estimates were reported or able to be calculated. RESULTS: Of 732 identified studies, 7 studies representing 23 411 children (risk of bias range, 7-9; maximum possible score=9) were included for meta-analysis. Pooled risk of sustaining a concussion was more than three times greater in children with a previous concussion compared with those with no previous concussion (RR=3.64; 95% CI: 2.68 to 4.96; p<0.0001; I 2=90.55%). Unreported sex-stratified data precluded direct comparison of concussion risk in male versus female athletes. CONCLUSION: Previously concussed children have four times the risk of sustaining a concussion compared with those with no previous concussion history. This should be a consideration for clinicians in return to sport decision-making. Future studies examining subsequent recurrent concussion in youth sports must consider sex differences.

Combinations of physical activity, sedentary time, and sleep duration and their associations with depressive symptoms and other mental health problems in children and adolescents: a systematic review.

BACKGROUND: For optimal health benefits, the Canadian 24-Hour Movement Guidelines for Children and Youth (aged 5-17 years) recommend an achievement of high levels of physical activity (≥60 min of moderate-to-vigorous physical activity), low levels of sedentary behaviour (≤2 h of recreational screen time), and sufficient sleep (9-11 h for children or 8-10 h for adolescents) each day. The objective of this systematic review was to examine how combinations of physical activity, sedentary time, and sleep duration relate to depressive symptoms and other mental health indicators among children and adolescents. METHODS: Literature was obtained through searching Medline, EMBASE, PsycINFO, and SportDiscus up to September 30, 2019. Peer-reviewed studies published in English or French were included if they met the following criteria: population (apparently healthy children and adolescents with a mean age of 5-17 years), intervention/exposure (combinations of physical activity, sedentary time, and sleep duration), and outcomes (depressive symptoms and other mental health indicators). A risk of bias assessment was completed for all included studies using the methods described in the Cochrane Handbook. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) framework was used to assess the quality of evidence for each health indicator. Narrative syntheses were employed to describe the results due to high levels of heterogeneity across studies. RESULTS: A total of 13 cross-sectional studies comprised in 10 papers met inclusion criteria. Data across studies involved 115,540 children and adolescents from 12 countries. Overall, the findings indicated favourable associations between meeting all 3 recommendations and better mental health indicators among children and adolescents when compared with meeting none of the recommendations. There was evidence of a dose-response gradient between an increasing number of recommendations met and better mental health indicators. Meeting the screen time and sleep duration recommendations appeared to be associated with more mental health benefits than meeting the physical activity recommendation. The quality of evidence reviewed was "very low" according to GRADE. CONCLUSIONS: The findings indicate favourable associations between meeting all 3 movement behaviour recommendations in the 24-h guidelines and better mental health indicators among children and adolescents. There is a clear need for high-quality studies that use robust measures of all movement behaviours and validated measures of mental health to increase our understanding in this topic area.

Creating enriched training sets of eligible studies for large systematic reviews: the utility of PubMed's Best Match algorithm.

INTRODUCTION: Solutions like crowd screening and machine learning can assist systematic reviewers with heavy screening burdens but require training sets containing a mix of eligible and ineligible studies. This study explores using PubMed's Best Match algorithm to create small training sets containing at least five relevant studies. METHODS: Six systematic reviews were examined retrospectively. MEDLINE searches were converted and run in PubMed. The ranking of included studies was studied under both Best Match and Most Recent sort conditions. RESULTS: Retrieval sizes for the systematic reviews ranged from 151 to 5,406 records and the numbers of relevant records ranged from 8 to 763. The median ranking of relevant records was higher in Best Match for all six reviews, when compared with Most Recent sort. Best Match placed a total of thirty relevant records in the first fifty, at least one for each systematic review. Most Recent sorting placed only ten relevant records in the first fifty. Best Match sorting outperformed Most Recent in all cases and placed five or more relevant records in the first fifty in three of six cases. DISCUSSION: Using a predetermined set size such as fifty may not provide enough true positives for an effective systematic review training set. However, screening PubMed records ranked by Best Match and continuing until the desired number of true positives are identified is efficient and effective. CONCLUSIONS: The Best Match sort in PubMed improves the ranking and increases the proportion of relevant records in the first fifty records relative to sorting by recency.

Crowdsourcing the Citation Screening Process for Systematic Reviews: Validation Study.

BACKGROUND: Systematic reviews (SRs) are often cited as the highest level of evidence available as they involve the identification and synthesis of published studies on a topic. Unfortunately, it is increasingly challenging for small teams to complete SR procedures in a reasonable time period, given the exponential rise in the volume of primary literature. Crowdsourcing has been postulated as a potential solution. OBJECTIVE: The feasibility objective of this study was to determine whether a crowd would be willing to perform and complete abstract and full text screening. The validation objective was to assess the quality of the crowd's work, including retention of eligible citations (sensitivity) and work performed for the investigative team, defined as the percentage of citations excluded by the crowd. METHODS: We performed a prospective study evaluating crowdsourcing essential components of an SR, including abstract screening, document retrieval, and full text assessment. Using CrowdScreenSR citation screening software, 2323 articles from 6 SRs were available to an online crowd. Citations excluded by less than or equal to 75% of the crowd were moved forward for full text assessment. For the validation component, performance of the crowd was compared with citation review through the accepted, gold standard, trained expert approach. RESULTS: Of 312 potential crowd members, 117 (37.5%) commenced abstract screening and 71 (22.8%) completed the minimum requirement of 50 citation assessments. The majority of participants were undergraduate or medical students (192/312, 61.5%). The crowd screened 16,988 abstracts (median: 8 per citation; interquartile range [IQR] 7-8), and all citations achieved the minimum of 4 assessments after a median of 42 days (IQR 26-67). Crowd members retrieved 83.5% (774/927) of the articles that progressed to the full text phase. A total of 7604 full text assessments were completed (median: 7 per citation; IQR 3-11). Citations from all but 1 review achieved the minimum of 4 assessments after a median of 36 days (IQR 24-70), with 1 review remaining incomplete after 3 months. When complete crowd member agreement at both levels was required for exclusion, sensitivity was 100% (95% CI 97.9-100) and work performed was calculated at 68.3% (95% CI 66.4-70.1). Using the predefined alternative 75% exclusion threshold, sensitivity remained 100% and work performed increased to 72.9% (95% CI 71.0-74.6; P<.001). Finally, when a simple majority threshold was considered, sensitivity decreased marginally to 98.9% (95% CI 96.0-99.7; P=.25) and work performed increased substantially to 80.4% (95% CI 78.7-82.0; P<.001). CONCLUSIONS: Crowdsourcing of citation screening for SRs is feasible and has reasonable sensitivity and specificity. By expediting the screening process, crowdsourcing could permit the investigative team to focus on more complex SR tasks. Future directions should focus on developing a user-friendly online platform that allows research teams to crowdsource their reviews.

Antenatal Consultations at Extreme Prematurity: A Systematic Review of Parent Communication Needs.

OBJECTIVE: To synthesize and describe parental expectations on how healthcare professionals should interact with them during a peripartum, antenatal consultation for extremely preterm infants. STUDY DESIGN: For this systematic literature review with textual narrative synthesis, we included studies that explored parental perspectives regarding the antenatal consultation for an extremely preterm infant. Electronic searches of Medline, CINAHL, PsycInfo, and Embase were conducted, along with a search of the grey literature. Quality appraisal was conducted using the guide by Walsh and Downe. Two independent reviewers reviewed 783 titles, of which 130 abstracts then 40 full-text articles were reviewed. Final data abstraction includes 19 studies. We predetermined 6 topics of interest (setting, timing, preferred healthcare professional, information, resources, and parents-physician interaction) to facilitate thematic analysis. RESULTS: In consideration of the variability of parents' specific desires, six predetermined topics and additional overarching themes such as perception of support, degree of understanding, hope, spirituality, and decision-making influences emerged. Studies suggest the quality of the antenatal consultation is not purely about information content, but also the manner in which it is provided. Limitations include thematic analysis that can potentially lead to the exclusion of important nuances. Relevant studies may have been missed if published outside the healthcare literature. CONCLUSIONS: The findings may inform clinical practice guidelines. This paper includes suggested strategies related to parents' perspectives that may facilitate communication during antenatal consultation for an extremely preterm infant. These strategies may also support parental engagement and satisfaction.

The mental health of adolescents and pre-adolescents living with inherited arrhythmia syndromes: a systematic review of the literature.

Potentially fatal arrhythmias add to the mental health challenges of adolescence. This systematic review sought to summarise current knowledge regarding the mental health of adolescents and pre-adolescents diagnosed with inherited arrhythmia syndromes. Searches combining psychological problems with inherited cardiac arrhythmia diagnoses identified 16 studies with paediatric (<18 years) inherited arrhythmia patients. All studies were cross-sectional; 8/16 required an implantable cardioverter defibrillator. Methods were quantitative (n=11), qualitative (n=4), or mixed (n=1), with 14-100% of participants having an inherited arrhythmia syndrome. Mean/median age in 13/16 studies was 12-16 years. Patients and parents reported lower quality of life, particularly in relation to physical function, social relationships, restriction of peer activities, bodily pain, and mental and emotional health. Self-perceptions and behaviour were similar to healthy populations. Rates of anxiety and depression (15-33% of these patients) were not increased in these studies where patients were assessed 2+ years after diagnosis. Higher mental health risk occurred among patients who have a diagnosed sibling, those with cardiomyopathy, and those who report decreased quality of life. Mental health research among youth with inherited arrhythmias is extremely limited and of low quality. Data, primarily from patients 2-4 years after diagnosis or treatment with an implantable cardioverter defibrillator, indicate that quality of life may be decreased and 15-33% experience mental health issues. Future research is required to examine the mental health and quality of life of paediatric patients with inherited arrhythmia syndromes, whether or not they have an implantable cardioverter defibrillator, from time of diagnosis.

Vitamin D deficiency in critically ill children: a systematic review and meta-analysis.

BACKGROUND: Vitamin D deficiency (VDD) has been hypothesized not only to be common but also to represent a potentially modifiable risk factor for greater illness severity and clinical outcome during critical illness. The objective of this systematic review was to determine the frequency of VDD in pediatric critical illness and its association with clinical outcomes. METHODS: MEDLINE, Embase, and CENTRAL were searched through December 12, 2016, with no date or language restrictions. The primary objective was to estimate the prevalence of VDD in the pediatric intensive care unit (PICU) and compare vitamin D status with healthy control populations. Secondary objectives were to evaluate whether VDD is associated with mortality, increased illness severity, PICU interventions, and patient clinical course. Random effects meta-analysis was used to calculate pooled VDD event rate, compare levels with those of control subjects, and evaluate for associations between VDD and clinical outcome. RESULTS: Among 2700 citations, 17 studies meeting study eligibility were identified. The studies reported a total of 2783 critically ill children and had a median sample size of 120 (range 12-511). The majority of studies used a 25-hydroxyvitamin D [25(OH)D] level less than 50 nmol/L to define VDD, and the pooled VDD prevalence was 54.8 (95% CI 45.4-63.9). Average 25(OH)D levels were significantly lower in PICU patients than in healthy control subjects (pooled difference -17.3 nmol/L, 95% CI -14.0 to -20.6). In a meta-analysis calculation, we found that VDD was associated with increased mortality (OR 1.62, 95% CI 1.11-2.36), illness severity, and need for PICU interventions. CONCLUSIONS: Approximately 50% of critically ill children have VDD at the time of PICU admission, defined as a blood total 25(OH)D concentration under 50 nmol/L. VDD was further determined to be associated with greater illness severity, multiple organ dysfunction, and mortality in the PICU setting. Clinical trials are required to determine if optimization of vitamin D status improves patient outcome. TRIAL REGISTRATION: PROSPERO, CRD42016026617 . Registered on 11 January 2016.

Systematic review of the relationships between sleep duration and health indicators in the early years (0-4 years).

BACKGROUND: The objective of this systematic review was to examine for the first time the associations between sleep duration and a broad range of health indicators in children aged 0 to 4 years. METHODS: Electronic databases were searched with no limits on date or study design. Included studies (published in English or French) were peer-reviewed and met the a priori determined population (apparently healthy children aged 1 month to 4.99 years), intervention/exposure/comparator (various sleep durations), and outcome criteria (adiposity, emotional regulation, cognitive development, motor development, growth, cardiometabolic health, sedentary behaviour, physical activity, quality of life/well-being, and risks/injuries). The quality of evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) framework. Due to high levels of heterogeneity across studies, narrative syntheses were employed. RESULTS: A total of 69 articles/studies (62 unique samples) met inclusion criteria. Data across studies included 148,524 unique participants from 23 countries. The study designs were randomized trials (n = 3), non-randomized interventions (n = 1), longitudinal studies (n = 16), cross-sectional studies (n = 42), or longitudinal studies that also reported cross-sectional analyses (n = 7). Sleep duration was assessed by parental report in 70% of studies (n = 48) and was measured objectively (or both objectively and subjectively) in 30% of studies (n = 21). Overall, shorter sleep duration was associated with higher adiposity (20/31 studies), poorer emotional regulation (13/25 studies), impaired growth (2/2 studies), more screen time (5/5 studies), and higher risk of injuries (2/3 studies). The evidence related to cognitive development, motor development, physical activity, and quality of life/well-being was less clear, with no indicator showing consistent associations. No studies examined the association between sleep duration and cardiometabolic biomarkers in children aged 0 to 4 years. The quality of evidence ranged from "very low" to "high" across study designs and health indicators. CONCLUSIONS: Despite important limitations in the available evidence, longer sleep duration was generally associated with better body composition, emotional regulation, and growth in children aged 0 to 4 years. Shorter sleep duration was also associated with longer screen time use and more injuries. Better-quality studies with stronger research designs that can provide information on dose-response relationships are needed to inform contemporary sleep duration recommendations.

Systematic review of the relationships between sedentary behaviour and health indicators in the early years (0-4 years).

BACKGROUND: The purpose of this systematic review was to examine the relationships between sedentary behaviour (SB) and health indicators in children aged 0 to 4 years, and to determine what doses of SB (i.e., duration, patterns [frequency, interruptions], and type) were associated with health indicators. METHODS: Online databases were searched for peer-reviewed studies that met the a priori inclusion criteria: population (apparently healthy, 1 month to 4.99 years), intervention/exposure and comparator (durations, patterns, and types of SB), and outcome/health indicator (critical: adiposity, motor development, psychosocial health, cognitive development; important: bone and skeletal health, cardiometabolic health, fitness, risks/harm). The quality of the evidence was assessed by study design and outcome using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) framework. RESULTS: Due to heterogeneity, meta-analyses were not possible; instead, narrative syntheses were conducted, structured around the health indicator and type of SB. A total of 96 studies were included (195,430 participants from 33 countries). Study designs were: randomized controlled trial (n = 1), case-control (n = 3), longitudinal (n = 25), longitudinal with additional cross-sectional analyses (n = 5), and cross-sectional (n = 62). Evidence quality ranged from "very low" to "moderate". Associations between objectively measured total sedentary time and indicators of adiposity and motor development were predominantly null. Associations between screen time and indicators of adiposity, motor or cognitive development, and psychosocial health were primarily unfavourable or null. Associations between reading/storytelling and indicators of cognitive development were favourable or null. Associations between time spent seated (e.g., in car seats or strollers) or in the supine position, and indicators of adiposity and motor development, were primarily unfavourable or null. Data were scarce for other outcomes. CONCLUSIONS: These findings continue to support the importance of minimizing screen time for disease prevention and health promotion in the early years, but also highlight the potential cognitive benefits of interactive non-screen-based sedentary behaviours such as reading and storytelling. Additional high-quality research using valid and reliable measures is needed to more definitively establish the relationships between durations, patterns, and types of SB and health indicators, and to provide insight into the appropriate dose of SB for optimal health in the early years.

Correction to: Systematic review of the relationships between physical activity and health indicators in the early years (0-4 years).

CORRECTION: After publication of the article [1], it has been brought to our attention that an incorrect reference has been used in this article, both in the main body and additional file 2. The reference in question is #105 in the main body and #74 in additional file 2. Here it is cited as "Lindsay H, Brussoni M. Injuries and helmet use related to non-motorized wheeled activities among pediatric patients. Chronic Dis Inj Canada. 2014;34(2-3):74-81".

Systematic review of the relationships between physical activity and health indicators in the early years (0-4 years).

BACKGROUND: Given the rapid development during the early years (0-4 years), an understanding of the health implications of physical activity is needed. The purpose of this systematic review was to examine the relationships between objectively and subjectively measured physical activity and health indicators in the early years. METHODS: Electronic databases were originally searched in April, 2016. Included studies needed to be peer-reviewed, written in English or French, and meet a priori study criteria. The population was apparently healthy children aged 1 month to 59.99 months/4.99 years. The intervention/exposure was objectively and subjectively measured physical activity. The comparator was various volumes, durations, frequencies, patterns, types, and intensities of physical activity. The outcomes were health indicators ranked as critical (adiposity, motor development, psychosocial health, cognitive development, fitness) and important (bone and skeletal health, cardiometabolic health, and risks/harm). The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) framework was used to assess the quality of evidence for each health indicator by each study design. RESULTS: Ninety-six studies representing 71,291 unique participants from 36 countries were included. Physical activity interventions were consistently (>60% of studies) associated with improved motor and cognitive development, and psychosocial and cardiometabolic health. Across observational studies, physical activity was consistently associated with favourable motor development, fitness, and bone and skeletal health. For intensity, light- and moderate-intensity physical activity were not consistently associated with any health indicators, whereas moderate- to vigorous-intensity, vigorous-intensity, and total physical activity were consistently favourably associated with multiple health indicators. Across study designs, consistent favourable associations with health indicators were observed for a variety of types of physical activity, including active play, aerobic, dance, prone position (infants; ≤1 year), and structured/organized. Apart from ≥30 min/day of the prone position for infants, the most favourable frequency and duration of physical activity was unclear. However, more physical activity appeared better for health. Evidence ranged from "very low" to "high" quality. CONCLUSIONS: Specific types of physical activity, total physical activity, and physical activity of at least moderate- to vigorous-intensity were consistently favourably associated with multiple health indicators. The majority of evidence was in preschool-aged children (3-4 years). Findings will inform evidence-based guidelines.

Canadian 24-Hour Movement Guidelines for the Early Years (0-4 years): An Integration of Physical Activity, Sedentary Behaviour, and Sleep.

BACKGROUND: The Canadian Society for Exercise Physiology convened representatives of national organizations, research experts, methodologists, stakeholders, and end-users who followed rigorous and transparent guideline development procedures to create the Canadian 24-Hour Movement Guidelines for the Early Years (0-4 years): An Integration of Physical Activity, Sedentary Behaviour, and Sleep. These novel guidelines for children of the early years embrace the natural and intuitive integration of movement behaviours across the whole day (24-h period). METHODS: The development process was guided by the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. Four systematic reviews (physical activity, sedentary behaviour, sleep, combined behaviours) examining the relationships within and among movement behaviours and several health indicators were completed and interpreted by a Guideline Development Panel. The systematic reviews that were conducted to inform the development of the guidelines, and the framework that was applied to develop the recommendations, followed the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. Complementary compositional analyses were performed using data from the Canadian Health Measures Survey to examine the relationships between movement behaviours and indicators of adiposity. A review of the evidence on the cost effectiveness and resource use associated with the implementation of the proposed guidelines was also undertaken. A stakeholder survey (n = 546), 10 key informant interviews, and 14 focus groups (n = 92 participants) were completed to gather feedback on draft guidelines and their dissemination. RESULTS: The guidelines provide evidence-informed recommendations as to the combinations of light-, moderate- and vigorous-intensity physical activity, sedentary behaviours, and sleep that infants (<1 year), toddlers (1-2 years) and preschoolers (3-4 years) should achieve for a healthy day (24 h). Proactive dissemination, promotion, implementation, and evaluation plans were prepared to optimize uptake and activation of the new guidelines. CONCLUSIONS: These guidelines represent a sensible evolution of public health guidelines whereby optimal health is framed within the balance of movement behaviours across the whole day, while respecting preferences of end-users. Future research should consider the integrated relationships among movement behaviours, and similar integrated guidelines for other age groups should be developed.

The Single-Case Reporting Guideline In BEhavioural Interventions (SCRIBE) 2016 Statement.

We developed a reporting guideline to provide authors with guidance about what should be reported when writing a paper for publication in a scientific journal using a particular type of research design: the single-case experimental design. This report describes the methods used to develop the Single-Case Reporting guideline In BEhavioural interventions (SCRIBE) 2016. As a result of 2 online surveys and a 2-day meeting of experts, the SCRIBE 2016 checklist was developed, which is a set of 26 items that authors need to address when writing about single-case research. This article complements the more detailed SCRIBE 2016 Explanation and Elaboration article (Tate et al., 2016 ) that provides a rationale for each of the items and examples of adequate reporting from the literature. Both these resources will assist authors to prepare reports of single-case research with clarity, completeness, accuracy, and transparency. They will also provide journal reviewers and editors with a practical checklist against which such reports may be critically evaluated. We recommend that the SCRIBE 2016 is used by authors preparing manuscripts describing single-case research for publication, as well as journal reviewers and editors who are evaluating such manuscripts. SCIENTIFIC ABSTRACT Reporting guidelines, such as the Consolidated Standards of Reporting Trials (CONSORT) Statement, improve the reporting of research in the medical literature (Turner et al., 2012 ). Many such guidelines exist and the CONSORT Extension to Nonpharmacological Trials (Boutron et al., 2008 ) provides suitable guidance for reporting between-groups intervention studies in the behavioural sciences. The CONSORT Extension for N-of-1 Trials (CENT 2015) was developed for multiple crossover trials with single individuals in the medical sciences (Shamseer et al., 2015 ; Vohra et al., 2015 ), but there is no reporting guideline in the CONSORT tradition for single-case research used in the behavioural sciences. We developed the Single-Case Reporting guideline In BEhavioural interventions (SCRIBE) 2016 to meet this need. This Statement article describes the methodology of the development of the SCRIBE 2016, along with the outcome of 2 Delphi surveys and a consensus meeting of experts. We present the resulting 26-item SCRIBE 2016 checklist. The article complements the more detailed SCRIBE 2016 Explanation and Elaboration article (Tate et al., 2016 ) that provides a rationale for each of the items and examples of adequate reporting from the literature. Both these resources will assist authors to prepare reports of single-case research with clarity, completeness, accuracy, and transparency. They will also provide journal reviewers and editors with a practical checklist against which such reports may be critically evaluated.

Pain Management During Newborn Screening: Using YouTube to Disseminate Effective Pain Management Strategies.

To assess the reach, acceptability, and effect of the BSweet2Babies video showing breast-feeding, skin-to-skin care, and sucrose during blood sampling on intention to recommend the video or advocate for use of the interventions. In July 2014, the video and an electronic survey were produced and posted. After 1 year, the online viewer survey responses and YouTube analytics were analyzed. One year after posting, the BSweet2Babies video had 10 879 views from 125 countries and 187 (1.7%) viewers completed the survey. Most respondents were aware of the analgesic effects of breast-feeding, skin-to-skin care, and sucrose. Nearly all respondents (n = 158, 92%) found the BSweet2Babies video to be a helpful resource and 146 (84%) answered that they would recommend the video to others. After viewing the video, 183 (98%) respondents answered that they would advocate for 1 or more of the interventions. The BSweet2Babies video showing effective pain treatment during blood sampling had a large reach but a very small response rate for the survey. Therefore, analysis of acceptability and effect on intention to recommend the video and advocate for the interventions depicted are limited. Further research is warranted to explore how to best evaluate videos delivered through social media and to determine the effect of the video to promote knowledge translation into clinical practice.