Neurodevelopmental challenges are the most prevalent comorbidity associated with a diagnosis of critical CHD, and there is a high incidence of gross and fine motor delays noted in early infancy. The frequency of motor delays in hospitalised infants with critical CHD requires close monitoring from developmental therapies (physical therapists, occupational therapists, and speech-language pathologists) to optimise motor development. Currently, minimal literature defines developmental therapists’ role in caring for infants with critical CHD in intensive or acute care hospital units.

This article describes typical infant motor skill development, how the hospital environment and events surrounding early cardiac surgical interventions impact those skills, and how developmental therapists support motor skill acquisition in infants with critical CHD. Recommendations for healthcare professionals and those who provide medical or developmental support in promotion of optimal motor skill development in hospitalised infants with critical CHD are discussed.

Infants with critical CHD requiring neonatal surgical intervention experience interrupted motor skill interactions and developmental trajectories. As part of the interdisciplinary team working in intensive and acute care settings, developmental therapists assess, guide motor intervention, promote optimal motor skill acquisition, and support the infant’s overall development.

Clinical research staff play a critical role in recruiting families for pediatric research, but their views are not well described. We aimed to describe how pediatric research staff build trusting research relationships with patients and their families.

We interviewed research staff at one pediatric research institution and its affiliated academic medical center between November 2020 and February 2021. Staff were eligible if they conducted participant recruitment, consent, and/or enrollment for clinical research. We developed our semi-structured interview guide based on a framework for trusting researcher-community partnerships.

We interviewed 28 research staff, with a median age of 28 years (range 22–50) and a median of 5 years of experience (range 1–29). Interviewees identified factors relevant to relationship building across three levels: the individual staff member, the relational interaction with the family, and the institutional or other structural backdrop. Individual factors included how staff developed recruitment skills, their perceived roles, and their personal motivations. Relational factors spanned four stages of recruitment: before the approach, forming an initial connection with a family, building the connection, and following up. Structural factors were related to access and diversity, clinical interactions, and the COVID-19 pandemic.

Research staff discussed tensions and supports with various actors, challenges with the integration of research and clinical care, the importance of voluntariness for building trust, and multiple contributors to inequities in research. These findings reveal the importance of ensuring research staff have a voice in institutional policies and are supported to advocate for patients and families.

To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation.

We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis.

Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission.

Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.

Cognitive impairment is common in individuals with substance use disorders (SUDs), yet no evidence-based guidelines exist regarding the most appropriate screening measure for use in this population. This systematic review aimed to (1) describe different cognitive screening measures used in adults with SUDs, (2) identify substance use populations and contexts these tools are utilised in, (3) review diagnostic accuracy of these screening measures versus an accepted objective reference standard, and (4) evaluate methodology of included studies for risk of bias.

Online databases (PsycINFO, MEDLINE, Embase, and CINAHL) were searched for relevant studies according to pre-determined criteria, and risk of bias and applicability was assessed using the Quality Assessment of Diagnostic Accuracy Studies–2 (QUADAS–2). At each review phase, dual screening, extraction, and quality ratings were performed.

Fourteen studies met inclusion, identifying 10 unique cognitive screening tools. The Montreal Cognitive Assessment (MoCA) was the most common, and two novel screening tools (Brief Evaluation of Alcohol-Related Neuropsychological Impairments [BEARNI] and Brief Executive Function Assessment Tool [BEAT]) were specifically developed for use within SUD populations. Twelve studies reported on classification accuracy and relevant psychometric parameters (e.g., sensitivity and specificity). While several tools yielded acceptable to outstanding classification accuracy, there was poor adherence to the Standards for Reporting Diagnostic Accuracy Studies (STARD) across all studies, with high or unclear risk of methodological bias.

While some screening tools exhibit promise for use within SUD populations, further evaluation with stronger methodological design and reporting is required. Clinical recommendations and future directions for research are discussed.

Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population.

The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials.

The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner.

Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.

Drive-through clinics (DTCs) are a novel type of point of dispensing where participants drive to a designated location and receive prophylaxis while remaining inside their vehicle. The objective of this review was to identify effective practices and recommendations for implementing DTCs for mass prophylaxis dispensing during emergency events.

A systematic review was conducted for articles covering DTCs published between 1990 and 2019. Inclusion criteria were peer-reviewed, written in English, and addressed DTCs sufficiently. Effective practices and recommendations identified in the literature were presented by theme.

A total of 13 articles met inclusion criteria. The themes identified were (1) optimal DTC design and planning via decision support systems and decision support tools; (2) clinic layouts, locations, and design aspects; (3) staffing, training, and DTC communication; (4) throughput time; (5) community outreach methods; (6) DTC equipment; (7) infection prevention and personal protective equipment; and (8) adverse events prevention and traffic management.

DTCs are an essential component of emergency preparedness and must be optimally designed and implemented to successfully dispense mass prophylaxis to a community within 48 hours. The effective practices and recommendations presented can be used for the development, implementation, and improvement of DTCs for their target populations.

Population-based prevalence and incidence studies are essential for understanding the burden of frontotemporal dementia (FTD).

The MEDLINE and EMBASE databases were searched to identify population-based publications from 1985 to 2012, addressing the incidence and/or prevalence of FTD. References of included articles and prior systematic reviews were searched for additional studies. Two reviewers screened all abstracts and full-text reviews, abstracted data and performed quality assessments.

Twenty-six studies were included. Methodological limitations led to wide ranges in the estimates for prevalence (point prevalence 0.01-4.6 per 1000 persons; period prevalence 0.16-31.04 per 1000 persons) and incidence (0.0-0.3 per 1000 person-years). FTD accounted for an average of 2.7% (range 0-9.1%) of all dementia cases among prevalence studies that included subjects 65 and older compared to 10.2% (range 2.8-15.7%) in studies restricted to those aged less than 65. The cumulative numbers of male (373 [52.5%]) and female (338 [47.5%]) cases from studies reporting this information were nearly equal (p=0.18). The behavioural variant FTD (bvFTD) was almost four times as common as the primary progressive aphasias.

Population-based estimates for the epidemiology of FTD varied widely in the included studies. Refinements in the diagnostic process, possibly by the use of validated biomarkers or limiting case ascertainment to specialty services, are needed to obtain more precise estimates of the prevalence and incidence of FTD.

Population-based prevalence and incidence studies are essential for understanding the societal burden of dementia with Lewy bodies (DLB).

The MEDLINE and EMBASE databases were searched to identify publications addressing the incidence and/or prevalence of DLB. References of included articles and prior systematic reviews were searched for additional studies. Two reviewers screened all abstracts and full-text reviews, abstracted data and performed quality assessments.

Twenty-two studies were included. Incidence rates ranged from 0.5 to 1.6 per 1000 person-years. DLB accounted for 3.2-7.1% of all dementia cases in the incidence studies. Point and period prevalence estimates ranged from 0.02 to 63.5 per 1000 persons. Increasing prevalence estimates were reported with increasing age. DLB accounted for from 0.3 to 24.4% of all cases of dementia in the prevalence studies.

DLB becomes more common with increasing age and accounts for about 5% of all dementia cases in older populations.