IWK Health Centre

Assessing autistic traits alongside co‐occurring emotional/behavioral concerns (EBCs) is challenging due to their overlap in clinical presentations, which can vary by age and sex. This study aimed to investigate domain‐specific associations between autistic traits and EBCs–including anxiety, affective, attention‐deficit/hyperactivity, and oppositional‐defiant problems–across childhood in autistic boys and girls. We prospectively followed 389 children (84% male) diagnosed with autism at ages 2–5 years, using the Social Responsiveness Scale (SRS) and Child Behavior Checklist (CBCL) across eight timepoints until age 12. Moderated nonlinear factor analysis was used to identify and adjust for measurement non‐invariance of SRS items by age, sex, and EBCs. The adjusted scores were then used for sex‐moderated time‐varying modeling of associations between autistic traits and EBCs. Several SRS items in the domains of social‐interaction difficulties and repetitive mannerisms showed significant intercept bias by age and level of co‐occurring anxiety and ADHD (effect size r > 0.20). In autistic boys, strong associations were observed between social‐communication difficulties and EBCs around ages 7–9, which tended to diminish in late childhood. In contrast, autistic girls showed stable or intensifying associations, particularly with anxiety, into late childhood. Results revealed significant associations between autistic traits and EBCs after addressing item‐level measurement biases. The varying associations over time highlight the importance of continuous monitoring to promptly address autistic children's sex‐differential mental health needs. These findings emphasize the benefits of refining behavioral constructs and adopting a nuanced developmental approach to identify critical periods of symptom coupling/decoupling for informing evaluation and service provision.

Objectives: We aimed to develop a comprehensive list of patient care components performed by pediatric emergency department (PED) physicians that could be individually scored on their subjective workload using the National Aeronautics and Space Administration Task Load Index (NASA-TLX). These “care components,” alongside patient and environmental factors that influence workload (“modifiers”), will form the basis of the Standardized Workload Assessment Metric for Pediatric Emergency Departments (SWAMPED). We sought to obtain preliminary workload scores for each care component and assess the face validity of the NASA-TLX-derived workload tool. Methods: After establishing a working list of “care elements” and modifiers, we convened an expert panel during a 3-day workshop to curate a comprehensive list of PED patient care components and modifiers affecting physician workload using a modified Delphi process. Experts completed a pilot version of the NASA-TLX-derived workload survey for each care component. A virtual follow-up was held 5 months after the initial meeting to finalize the list of modifiers and care components. Results: Of the 93 initial care elements and 75 modifiers, 46 care components were retained, alongside 6 final modifiers. Preliminary workload scores showed “high acuity, low occurrence procedures (cricothyroidotomy, thoracotomy, pericardiocentesis, burr hole, etc.),” with the highest median workload score of 106, while “immobilization device simple (prefabricated)” had the lowest median workload score of 22. Conclusions: The SWAMPED, derived through expert consensus, holds promise as a standardized assessment tool for PED physician workload. Validation studies involving larger cohorts are crucial for refining the SWAMPED and allowing widespread adoption of this novel quantitative workload metric.

Purpose of review Pasteurized donor human milk (PDHM) is the recommended supplement when there is inadequate volume of mother's own milk (MOM) for very low birth weight (<1500 g, VLBW) infants. Differences in the composition of these milks may impact growth, morbidities or long-term development of infants. The aim of this review is to highlight current trends in understanding compositional differences between MOM and PDHM, technological advances in processing PDHM, and infant outcomes when VLBW infants are fed these milks. Recent findings Reported differences in the composition between MOM and PDHM are due to several factors including when and how milk is collected, sampled for analysis, and processed. Systematic reviews and primary research studies demonstrate that PDHM reduces the risk of necrotizing enterocolitis in VLBW infants but is also associated with slower postnatal growth. Work is ongoing to determine if alternative approaches to processing PDHM can improve milk composition and thereby infant growth and neurodevelopment and reduce morbidity. Summary PDHM is a key component of feeding VLBW infants when there is inadequate volume of MOM. Recent developments aim to optimize this source of nutrition and bioactive compounds for VLBW infants while further understanding limitations of its use.

Co-occurring autism and attention-deficit/hyperactivity disorder (ADHD) have been associated with poorer social skills. Most studies examining the association of ADHD symptoms and social skills in autism employ categorical and cross-sectional designs, which provide a narrow view of the development of ADHD symptoms. Using group-based trajectory modeling, we identified five trajectories of caregiver-reported attention problems in an inception cohort of autistic children ( N = 393) followed from age 2–5 years (T1) to age 10.5–11 years (T8): Low-Stable (LS; 15.5% of participants), Low-Decreasing (LD; 25.2%), Low-Increasing (LI; 19.2%), Moderate-Decreasing (MD; 32.9%), and High-Stable (HS; 7.2%). Child FSIQ and caregiver age at baseline were lower and caregiver depression at baseline was higher for participants in the MD group than the LS group. Psychotropic medication use was associated with higher attention problems. The MD and HS groups had similar mean Vineland Adaptive Behavior Scales, Second Edition (VABS-II) Socialization standard scores at T8, which were lower than other groups. The LI group had lower Socialization scores than the LS group. Results support that a decline in caregiver-reported attention problems is common but not universal in autistic children and that even moderate/subclinical attention problems may relate to social skills outcomes in autism.

The human placenta is the composite of multiple cell types, each which contributes uniquely to placental function. Small non-coding RNAs (sncRNAs) are regulators of gene expression and can be cell-specific. The sncRNA transcriptome of individual placental cell types has not yet been investigated due to difficulties in their procurement and isolation. Using a custom sequencing method, we explored the expression of seven sncRNA species (miRNA, piRNA, rRNA, scaRNA, snRNA, snoRNA, tRNA) from whole chorionic villi and four major sample-matched FACS-sorted cell type (cytotrophoblast, stromal, endothelial, Hofbauer) samples from 9 first trimester and 17 term placentas. After normalization for technical variables, samples clustered primarily by cell type lineage. No sncRNAs were uniquely expressed by cell type, however, mean expression differed by cell type for 115 sncRNAs. Known placentally-expressed sncRNAs showed differing expression by cell type and trimester. Expression of few sncRNAs varied by sex. Lastly, sample-matched sncRNA expression and DNA methylation correlation was not significant, although high correlation (> R² ± 0.6) was observed for some sncRNA-CpG pairs. This study represents the first exploration of the sncRNA transcriptome of bulk placental villi and placental cell types, informing about the expression and regulatory patterns underlying human placental development.

Background Digital pathology facilitates remote pathology consultations. Pediatric pathologists in Canada formed a nationwide digital pathology consultation network, mostly for second opinion review of pediatric cancer cases. Validation of such a large network for clinical use is challenging. Here we report our unique validation process of this digital pathology network. Method This study was designed in keeping with the College of American Pathologist (CAP) guidelines, and included 14 pathologists from 9 hospitals across Canada. All cases are pediatric pathology cases. Each pathologist reviewed multiple digital cases and the corresponding glass slide cases. For each review, intra-observer concordance (diagnosis on digital case versus diagnosis on glass slide case) was recorded, creating a data point. Result The study generated 269 valid diagnostic data points. Out of the 269 data points, 257 were concordant (95.5% concordance), exceeding the CAP recommendation of 95% concordance. Thus, the network was successfully validated. Conclusion This is a unique validation study for a large nationwide digital pediatric pathology network. The study involved all pathologists/hospitals in the network, closely emulating real world clinical process. The network was successfully validated.

Purpose: To investigate the gene variant spectrum in patients with familial exudative vitreoretinopathy (FEVR). Methods: Probands clinically diagnosed with FEVR and their relatives were enrolled and clinical information and DNA collected. An expanded FEVR panel was used, including six recognized FEVR genes (FZD4, NDP, LRP5, TSPAN12, ZNF408, and CTNNB1) and 19 genes previously associated with ocular features overlapping FEVR (FEVR-associated genes). Variants identified using targeted next-generation sequencing and/or Sanger sequencing were analyzed and classified using the American College of Medical Genetics and Clinical Genome Resource Sequence Variant Interpretation (ClinGen SVI) working group recommendations to detect disease-causing variants (DCVs). Results: Analyses of data from a cohort of 94 probands provided a molecular diagnosis for 39 (41.5%) probands: 34 (87.2%) had a single DCV, whereas 5 (12.8%) harbored more than 1 DCV. Of 41 total DCVs in solved probands, 33 (80.5%) were in 4 of the 6 recognized genes, LRP5, FZD4, TSPAN12, and NDP, whereas 8 were found in FEVR-associated genes, 6 in KIF11, and 2 (LAMA1 and DOCK6) each in association with a KIF11 DCV. Reanalyzing variants using the latest criteria impacted the variant classification in five probands (5.3%), changing variants that were once deemed likely pathogenic to variants of uncertain significance. Conclusions: The expanded FEVR gene panel detected DCVs in nearly one-half of our cohort. Including the criteria used in classification will improve transparency of variant calls as more data become available. Four FEVR genes account for most cases, and the role of rare FEVR genes and candidate genes requires further study.

Background There is increasing demand for health systems to reduce greenhouse gas emissions and invest in climate‐resilient health care. Coordinating organizational structures and processes for reducing health system emissions presents challenges. Learning health systems, defined as systems that seek to continuously generate and apply evidence, innovation, quality, and value in health care, can guide health systems with planning organizational structures and processes to advance environmentally sustainable healthcare. The purpose of this research is to gather in‐depth insight from key health system leaders and healthcare professionals to identify challenges and recommendations for planning environmentally sustainable learning health systems. Methods Environmental scan methods were used, comprising jurisdictional literature review and informal discussions with key informants at one tertiary care center in Nova Scotia, Canada. Key informants were asked to describe challenges of coordinating environmentally sustainable health system structures and processes, and recommendations to advance planning for environmentally sustainable learning health systems. Deductive thematic analysis was used to categorize challenges and recommendations into seven characteristics of a learning health system framework. Results Informal discussions with 16 key informants provide detailed descriptions of 7 challenges and recommendations for planning and coordinating organizational structures and processes to advance environmentally sustainable learning health systems. Health system challenges include limited patient and community engagement, no systematic approach to measuring and monitoring emissions data, and limited knowledge of sustainability co‐benefits and strategies for mobilizing sustainable organizational change. Recommendations include engaging patients and communities in co‐creation of sustainable healthcare, monitoring of emissions data identifying high‐impact areas for action, and well‐coordinated leadership supporting sustainable policies, procedures, and decision‐making in practice. Conclusion Learning health systems provide structure for establishing critical processes to adapt to routinely collected data through rapid cycle improvements, and operationalization of value‐based health care that prioritizes health outcomes, reduction of costs, and mitigating environmental impacts.

Prior cross-sectional research established that four distinct responses to sexual rejection are associated with sexual and relationship well-being among couples affected by Sexual Interest/Arousal Disorder (SIAD). Examining these associations daily and prospectively will provide insight into within-person variations, temporality, and directionality. Women and gender-diverse individuals diagnosed with SIAD and their partners (N = 232 couples) completed a baseline survey, 56-day diary, and 6-month follow-up survey, assessing responses to sexual rejection, sexual satisfaction, dyadic sexual desire, sexual distress, and relationship satisfaction. Post hoc exploratory analyses controlled for non-physical sexual coercion and sexual rejection frequency. On days when individuals with SIAD perceived or their partners reported greater understanding responses than usual, they both reported higher relationship satisfaction, but also greater sexual distress. Daily perceived or reported greater resentful or insecure responses were associated with lower sexual and relationship satisfaction, and greater sexual distress. Enticing responses showed mixed results. For partners, all daily self-reported response types were positively associated with their own dyadic sexual desire. Prospectively for individuals with SIAD perceiving greater understanding and lower resentful and enticing responses at baseline predicted their own greater sexual and relationship satisfaction and perceiving greater insecure responses predicted their partners’ greater sexual satisfaction, six months later. For partners, greater insecure responses at baseline predicted their own lower sexual satisfaction and greater sexual distress six months later. Results suggest that partner responses to sexual rejection may be useful intervention targets aimed at promoting the sexual well-being and relationship satisfaction of couples coping with SIAD.

Molecular imaging is used in clinical and research settings. Since tools to study viral pathogenesis longitudinally and systemically are limited, molecular imaging is an attractive and largely unexplored tool. This review discusses molecular imaging probes and techniques for studying viruses, particularly those currently used in oncology that are applicable to virology. Expanding the repertoire of probes to better detect viral disease may make imaging even more valuable in (pre-)clinical settings.

Background With early detection and improvements in systemic and local therapies, millions of people are surviving cancer, but for some at a high cost. In some cancer types, cardiovascular disease now competes with recurrent cancer as the cause of death. Traditional care models, in which the cardiologist or oncologist assess patients individually, do not address complex cancer and cardiovascular needs. Nursing disciplines should be an integral part of holistic assessment in cardio-oncology care. To learn what educational needs nurses perceive important for provision of competent cardio-oncology nursing care, we undertook an international survey, aiming to understand their learning needs and preferred learning modalities. Methods A cross-sectional survey was developed by members of the International Cardio-Oncology Society (IC-OS) Nursing Research group. The survey was in English and consisted of 23 questions which include demographic information, clinical specialty (oncology, cardiology, or cardio-oncology), multiple-choice questions related to clinical topics that nurses might be interested in learning, and preferred methods of instruction. Results Three hundred and twenty-nine responses were received. The majority expressed interest in learning more about cardio-oncology related topics, primarily via pre-recorded webinars (n = 206, 67%) and live virtual meetings (n = 192, 63%). Formal programs leading to certification were highly endorsed (n = 247, 80%). In relation to specific cardio-oncology topics, there was a strong interest in learning more about specific cardiovascular toxicities, and their monitoring and management (n = 205, 66%). Conclusion Cardio-oncology is a new field of expertise requiring competent nurses with current knowledge incorporating both specialties. The survey we conducted described the sample’s characteristics, identified cardio-oncology learning needs and preferred methods of delivery. A cardio-oncology core curriculum based on the survey responses can offer convenient, accessible and learner-directed education for nurses worldwide. Ultimately, development of cardio-oncology nursing expertise will benefit cancer patients and survivors worldwide.

Objective We aimed to study the disease course, outcomes, and predictors of outcome in pediatric‐onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) affecting the kidneys. Methods Patients eligible for this study had a diagnosis of granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or ANCA‐positive pauci‐immune glomerulonephritis, were 18 years or younger at diagnosis, had renal disease defined by biopsy or dialysis dependence, and had clinical data at diagnosis and at either 12 or 24 months. Ambispective data from A Registry for Children with Vasculitis/Pediatric Vasculitis Initiative Registry was used. The primary outcome was inactive renal disease (pediatric vasculitis activity score = 0 or 1) at 12 months. Secondary outcomes included rates of improved renal function and damage within 24 months. Renal function, defined by estimated glomerular filtration rate, was categorized into Kidney Disease Improving Global Outcomes (KDIGO) stages at diagnosis and tested as a predictor of outcome using a proportional‐odds logistic regression model. Results A total of 145 patients were included: 68% were female, and 78% had GPA. At 12 months, 83% of patients achieved inactive renal disease; however, 42% had evidence of permanent renal damage. Compared with patients with normal renal function at diagnosis, patients with moderate to severely reduced renal function, or kidney failure at diagnosis, had an odds ratio of 8.62 (P = 0.002; 95% confidence interval [CI] 2.31–32.1) and 26.3 (P < 0.001; 95% CI 6.32–109), respectively, for being in a non‐normal KDIGO category at 12 months. Conclusion The majority of patients with pediatric AAV achieve inactive renal disease by 12 months; however, almost half have evidence of damage. Renal function at diagnosis is a strong predictor of renal function at 12 months.

Background Childhood obesity can result in adverse health outcomes. The objectives of this study were to describe the prevalence of obesity and determine the association between obesity at cancer diagnosis and event‐free survival (EFS) and overall survival (OS) in children diagnosed with cancer in Canada. Methods The authors conducted a retrospective cohort study using the Cancer in Young People in Canada database, including all children with newly diagnosed cancer aged 2–18 years across Canada from 2001 to 2020. Obesity was defined as age‐adjusted and sex‐adjusted body mass index greater than or equal to the 95th percentile. Univariate and multivariable Cox proportional hazards models compared EFS and OS between patients with and without obesity at diagnosis. Results In total, 11,291 patients were included, of whom 10.5% were obese at diagnosis. In multivariable models controlling for age, sex, ethnicity, neighborhood income quintile, treatment era, and cancer categories, obesity at diagnosis was independently associated with inferior EFS (adjusted hazard ratio [aHR], 1.16; 95% confidence interval [CI], 1.02–1.32; p = .02) and OS (aHR, 1.29; 95% CI, 1.11–1.49; p = .001). The adverse prognostic impact of obesity was particularly notable for acute lymphoblastic leukemia (ALL) and central nervous system (CNS) tumors. In children with ALL (n = 3458), obesity remained associated with inferior EFS (aHR, 1.55; p = .002) and OS (aHR, 1.75; p = .002) in multivariable analysis. In patients with CNS tumors (n = 2458), obesity was also associated with inferior EFS (aHR, 1.38; p = .008) and OS (aHR, 1.47; p = .004). Conclusions In this population‐based study, obesity at cancer diagnosis was independently associated with inferior survival across the entire cohort, and prominently in children with ALL and CNS tumors.

Background/Objectives: Caregivers of children with chronic illnesses, including chronic pain, experience high levels of distress, which impacts their own mental and physical health as well as child outcomes. Virtual care solutions offer opportunities to provide accessible support, yet most overlook caregivers’ needs. We conducted a scoping review to create an interactive Evidence and Gap Map (EGM) of virtual care solutions across a stepped care continuum (i.e., from self-directed to specialized care) for caregivers of youth with chronic illnesses. Methods: The review methodology was co-designed with four caregivers. Data sources were the peer-reviewed scientific literature and a call for innovations. Records were independently coded and assessed for quality. Results: Overall, 73 studies were included. Most virtual care solutions targeted caregivers of children with cancer, neurological disorders, and complex chronic illnesses. Over half were noted at lower levels of stepped care (i.e., self-guided apps and websites), with psychological strategies being predominant (84%). However, very few addressed caregivers’ physical health (15%) or provided family counseling (19%) or practical support (1%). Significant gaps were noted in interventions for managing caregiver chronic pain, despite its high prevalence and impact on child outcomes. Conclusions: Evidence and Gap Maps are innovative visual tools for knowledge synthesis, facilitating rapid, evidence-informed decision-making for patients, families, health professionals, and policymakers. This EGM highlighted high-quality virtual care solutions ready for immediate scaling and identified critical evidence gaps requiring prioritization. To address the complexities of pediatric chronic illnesses, including chronic pain, virtual care initiatives must prioritize family-centered, accessible, and equitable approaches. Engaging caregivers as partners is critical to ensure interventions align with their needs and priorities.

Background: Cause of death analysis is fundamental to forensic pathology. We present the case of a 9½-year-old girl with a genetically confirmed diagnosis of Dravet syndrome who died in her sleep with no evidence of motor seizure. She also had a lifelong history of recurrent pneumonias and, along with her family, had tested positive for COVID-19 10 days before death. Methods: Long-term clinical history of Dravet Syndrome and respiratory infections were obtained from patient’s medical charts and radiology reports. A Rapid-Antigen Test was used to confirm SARS-CoV2 infection days prior to death. At autopsy, brain, heart and lung tissues were obtained. Paraffin-embedded tissues were double-stained with H&E, and immunohistochemically stained using various antibodies. Results: Autopsy revealed evidence of previous seizure activity in the brain and cellular interstitial thickening in the lung. The brain showed edema and fibrillary gliosis without neuronal loss in neocortex and hippocampus. The lung showed inflammatory interstitial thickening with histiocytes, megakaryocytes, B-lymphocytes, and T-lymphocytes, including helper/suppressor cells and cytotoxic T-lymphocytes. Diffuse alveolar damage was observed as alveolar flooding with proteinaceous fluid. Conclusions: The cause of death may be attributed to Sudden Unexpected Death in Epilepsy (SUDEP) in Dravet syndrome, sudden death in viral pneumonia, or some combination of the two. When two independent risk factors for sudden unexpected death are identified due to co-pathology, it may not be possible to determine a single cause of death beyond a reasonable doubt.

Objective From 2002 to 2023, palivizumab was the only intervention to reduce respiratory syncytial virus (RSV)-associated hospitalizations in high-risk infants in Canada but advances in RSV prevention are drastically changing this landscape. Eligibility criteria for this monoclonal antibody for preterm infants varied over time across each of 10 Canadian provinces and 3 territories. The National Professional Pediatric Association (Canadian Pediatric Society) revised its eligibility recommendations in 2015, removing access for preterm infants 30 to 32 weeks gestation (WG). The province of Nova Scotia followed these recommendations the next season. This study aimed to determine if the removal of access to palivizumab in these previously eligible infants was associated with a change in hospital admissions, deaths, or ambulatory visits associated with RSV. Study Design We identified a retrospective cohort of Nova Scotia infants born between 30 and 32 WG, without other risk factors for RSV-H, from April 2012 to September 2019 by linking six population-based provincial databases, and followed each infant through the first year of life. Episodes of RSV-associated hospitalization (RSV-H), ambulatory visits (RSV-A), or death were identified by the International Statistical Classification of Diseases and Related Health Disorders (ICD) RSV-associated diagnostic codes. Results Of 4,835 infants born during the study period, 250 were 30 to 32 WG and eligible for the cohort. RSV-H increased approximately 10-fold following restricted access to palivizumab (from no RSV-H (0/123) to 9.4%; 95% CI 5.0, 15.9; risk difference 9.4), but no RSV-associated deaths occurred. RSV-A also increased from 5.7 to 17.3% (risk difference 11.6). Conclusion Discontinuation of access to a prophylactic anti-RSV monoclonal antibody in very preterm infants was associated with a higher risk of RSV-H and RSV-A. Evaluation of health care policy change on patient well-being is essential to assess the impact and guide future decision-making at the population level. Key Points

Introduction Macroglossia is a frequent clinical feature of Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth disorder. Macroglossia can lead to abnormal breathing, feeding, speech, and dentoskeletal development. Partial glossectomy is a common intervention aimed at reducing these abnormalities. The optimal timing of partial glossectomy remains controversial due to the potential need for secondary surgery and the ongoing growth of the tongue in early childhood. Materials and Methods After PRISMA-ScR and PRISMA-S reporting standards, this systematic review included English language studies of patients with BWS who underwent partial glossectomy. Data were extracted, including patient age, clinical outcomes, and follow-up. Study evidence levels were categorized based on a recognized hierarchy, and bias was assessed using the MINORS criteria. Results Early tongue reduction surgery (<24 mo) was associated with a lower incidence of class 3 occlusion and anterior open bite compared with later surgery. Improvements in speech intelligibility, tongue mobility, and breathing outcomes, including a reduction in obstructive sleep apnea, were observed, especially in early surgical groups. Feeding and drooling outcomes improved across both early and late surgical interventions, although no direct comparisons were made between the 2. Overall, tongue reduction surgery demonstrated benefits in functional outcomes, whereas dentoskeletal improvements remained variable. Conclusion Although there is a lack of consensus to the optimal age for the procedure, overall tongue reduction surgery in BWS seems to have functional benefits, including in speech, feeding, and breathing. Dentoskeletal outcomes are more variable. Variability in macroglossia severity, surgical technique, and surgeon experience may account for differences in reported outcomes across studies.

We aimed to understand how experiences with vaccine-related information and communication challenges during the COVID-19 pandemic impacted motivations and behaviors among Canadian adults regarding future vaccines. Semi-structured interviews were conducted with participants purposively selected to ensure diversity in age, sex at birth, self-identified gender, and region. Data were analyzed using thematic analysis; findings were mapped to the Information-Motivation-Behavioral Skills Model focusing on factors affecting vaccine hesitancy and uptake. Of 62 interviews completed, most were with woman (n = 32, 51.6%) and residents of Ontario (n = 36, 58.1%); the median age was 43.5 yr (interquartile range 23.3 yr). Themes included: 1) accessibility of information, 2) ability to assess information accuracy and validity, 3) trust in communications from practitioners and decision-makers, and 4) information seeking behaviors. Participants expressed various concerns about vaccines, including fears about potential side effects, particularly regarding the long-term effects of novel vaccinations. These concerns may reflect broader societal anxieties, which have been intensified by widespread misinformation and an overload of vaccine information. Moreover, participants highlighted a lack of trust in the information provided by government agencies and pharmaceutical companies, primarily driven by concerns regarding their underlying motives. Concerns about COVID-19 vaccine safety and effectiveness negatively impacted future vaccine attitudes and behaviors. Vaccine hesitancy studies should consider how individuals receive, perceive, and seek information within social contexts and risk profiles.

Obesity factors into hypertrophic cardiomyopathy (HCM)-related risk as a disease modifying environmental factor. Behaviours such as diet and sleep are seldom reported upon in children with HCM. It was our aim to report on these factors in this population. A multicenter cross-sectional study recruited children and teens with HCM aged 10–19 years old from 10 Canadian pediatric care sites. Patient demographics were obtained from review of medical charts. Participants completed the Healthy Hearts Survey, involving 92 questions related to family health history, personal health, smoking, nutrition, and physical or sedentary activity. A cohort of n = 56 patients with median (IQR) age of 15.5 (13.8–16.8) years were enrolled. Over half (53.6%) were classified as overweight or obese based on BMI, with 75% under activity restriction. Consumption of sugar-rich and starchy foods alongside skipping of meals (43.6%) and snacking (87.5%) were reported. A desire to lose weight was prevalent (48.2%) despite general perceptions of good health. Participants slept a median duration of 8 (7.4–9.0) hours on weekdays, however, sleep disturbances were widely reported. A majority (96.4%) reported less than 20 min of exercise per day over the course of the week. Unhealthy patterns regarding diet, sleep, and physical or sedentary activities that associate with cardiovascular risk are present within children with HCM. These habits may worsen disease burden in children with HCM. It is unclear how activity restriction factors into such patterns. There are modifiable lifestyle factors in children with HCM which, if addressed, could positively impact cardiovascular risk.