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Background: In type 1 diabetes, carbohydrate counting is the standard of care to determine prandial insulin needs, but it can negatively affect quality of life. We developed a novel insulin-and-pramlintide closed-loop system that replaces carbohydrate counting with simple meal announcements., Methods: We performed a randomised crossover trial assessing 14 days of (1) insulin-and-pramlintide closed-loop system with simple meal announcements, (2) insulin-and-placebo closed-loop system with carbohydrate counting, and (3) insulin-and-placebo closed-loop system with simple meal announcements. Participants were recruited at McGill University Health Centre (Montreal, QC, Canada). Eligible participants were adults (aged ≥18 years) and adolescents (aged 12-17 years) with type 1 diabetes for at least 1 year. Participants were randomly assigned in a 1:1:1:1:1:1 ratio to a sequence of the three interventions, with faster insulin aspart used in all interventions. Each intervention was separated by a 14-45-day wash-out period, during which participants reverted to their usual insulin. During simple meal announcement interventions, participants triggered a prandial bolus at mealtimes based on a programmed fixed meal size, whereas during carbohydrate counting interventions, participants manually entered the carbohydrate content of the meal and an algorithm calculated the prandial bolus based on insulin-to-carbohydrate ratio. Two primary comparisons were predefined: the percentage of time in range (glucose 3·9-10·0 mmol/L) with a non-inferiority margin of 6·25% (non-inferiority comparison); and the mean Emotional Burden subscale score of the Diabetes Distress Scale (superiority comparison), comparing the insulin-and-placebo system with carbohydrate counting minus the insulin-and-pramlintide system with simple meal announcements. Analyses were performed on a modified intention-to-treat basis, excluding participants who did not complete all interventions. Serious adverse events were assessed in all participants. This trial is registered on ClinicalTrials.gov, NCT04163874., Findings: 32 participants were enrolled between Feb 14, 2020, and Oct 5, 2021; two participants withdrew before study completion. 30 participants were analysed, including 15 adults (nine female, mean age 39·4 years [SD 13·8]) and 15 adolescents (eight female, mean age 15·7 years [1·3]). Non-inferiority of the insulin-and-pramlintide system with simple meal announcements relative to the insulin-and-placebo system with carbohydrate counting was reached (difference -5% [95% CI -9·0 to -0·7], non-inferiority p<0·0001). No statistically significant difference was found in the mean Emotional Burden score between the insulin-and-pramlintide system with simple meal announcements and the insulin-and-placebo system with carbohydrate counting (difference 0·01 [SD 0·82], p=0·93). With the insulin-and-pramlintide system with simple meal announcements, 14 (47%) participants reported mild gastrointestinal symptoms and two (7%) reported moderate symptoms, compared with two (7%) participants reporting mild gastrointestinal symptoms on the insulin-and-placebo system with carbohydrate counting. No serious adverse events occurred., Interpretation: The insulin-and-pramlintide system with simple meal announcements alleviated carbohydrate counting without degrading glucose control, although quality of life as measured by the Emotional Burden score was not improved. Longer and larger studies with this novel approach are warranted., Funding: Juvenile Diabetes Research Foundation., Competing Interests: Declaration of interests MAT received speaker honoraria from AstraZeneca, Boehringer Ingelheim, Eli Lilly, Janssen, Novo Nordisk, and Sanofi. LL received research support from AstraZeneca and Merck; consultant fees from Abbott, Dexcom, Insulet, and Novo Nordisk; payment or honoraria and support for attending meetings or travel from Novo Nordisk; and participated on a data safety monitoring board and advisory board for the National Institutes of Health artificial pancreas programme. MV received research support from Abbott, Bausch Health, and Novo Nordisk; consultant fees from AbbVie, Abbott, Bausch Health, Boehringer Ingelheim, Lifescan, Lyceum, Novo Nordisk, Roche, and Sanofi; and speaker honoraria from Abbott, AbbVie, Bausch Health, Lifescan, Lilly, Merck, Novo Nordisk, Pfizer, Roche, and Sanofi. J-FY received research support from Bayer, Novo Nordisk, Novartis, and Sanofi; consultant fees from Abbott, Bayer, Boehringer Ingelheim, Dexcom, Eli Lilly, Janssen, Merck, Mylan, Novo Nordisk, and Sanofi; and speaker honoraria from Novo Nordisk and Eli Lilly. NG-P is employed by Tandem Diabetes Care. AH received research support from Adocia, Dexcom, Eli Lilly, and Tandem Diabetes Care; equipment, materials, drugs, medical writing, gifts, or other services from Dexcom, Ypsomed, and Tandem Diabetes Care; consulting fees from Eli Lilly; and intellectual property acquisition fees from Eli Lilly and Bigfoot. All other authors declare no competing interests., (Copyright © 2024 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY-NC 4.0 license. Published by Elsevier Ltd.. All rights reserved.)

Background and Objectives: Children with medical complexity (CMC) comprise a subgroup of children with severe chronic diseases. A conceptual definition for CMC has been formulated, but there is no agreement on criteria to fulfill each of the 4 proposed domains: diagnostic conditions, functional limitations, health care use, and family-identified needs. Our objective with this study was to identify a standardized definition of CMC., Methods: Through a scoping review of the CMC literature, we identified potential criteria to fulfill each domain. These were incorporated into an electronic survey that was completed by a geographic and professionally varied panel of 81 American and Canadian respondents with expertise in managing CMC (response rate 70%) as part of a 4-iteration Delphi procedure. Respondents were asked to vote for the inclusion of each criterion in the definition, and for those with quantitative components (eg, hospitalization rates), to generate a consensus threshold value for meeting that criterion. The final criteria were analyzed by a committee and collapsed when situations of redundancy arose., Results: Of 1411 studies considered, 132 informed 55 criteria for the initial survey, which was presented to 81 respondents. Consensus for inclusion was reached on 48 criteria and for exclusion on 1 criterion. The committee collapsed those 48 criteria into 39 final criteria, 1 for diagnostic conditions, 2 for functional limitations, 13 for health care use, and 23 for family needs., Conclusions: These results represent the first consensus-based, standardized definition of CMC. Standardized identification is needed to advance understanding of their epidemiology and outcomes, as well as to rigorously study treatment strategies and care models., (Copyright © 2024 by the American Academy of Pediatrics.)

Introduction: Children and youth who are uninsured or underinsured in Canada and the USA have limited options where they can receive healthcare. In both countries, community health centres (CHCs) have been established as a solution to provide quality care to children without adequate insurance, including those who are newcomers or refugees. However, little is known about how well these models deliver paediatric care. Cross-country analysis provides an important viewpoint to identify areas of success and growth. The purpose of this scoping review is to compare quality of care for uninsured and underinsured children through CHCs in the USA and Canada., Methods: This scoping review follows the methodological guidelines from the Joanna Briggs Institute Evidence synthesis. The protocol has been registered with the Open Science Framework Registries and can be accessed online. A search will be conducted in electronic databases of peer-reviewed literature (Ovid MEDLINE ALL, CINAHL Complete via EbscoHost, Scopus; Health Business Elite via EbscoHost and Sociological Abstracts via ProQuest) as well as the grey literature. Two reviewers will review all titles and abstracts for inclusion in full-text review. Studies that meet inclusion criteria will be included in full-text review. Data will be extracted into Covidence, using the Donabedian model as a conceptual framework. Findings will be synthesised in a narrative format., Ethics and Dissemination: As this study only uses publicly available data, ethics approval is not required. Findings will be shared at national and international conferences and published in a peer-reviewed journal. In addition, findings will be prepared into a policy brief or white paper to be shared with relevant policy stakeholders to advocate for a better model of care for marginalised children and youth., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Importance: Severe respiratory disease declined during the COVID-19 pandemic, partially due to decreased circulation of respiratory pathogens. However, the outcomes of children with higher risk have not been described using population-based data., Objective: To compare respiratory-related hospitalizations, intensive care unit (ICU) admissions, and mortality during the pandemic vs prepandemic, among children with medical complexity (CMC) and without medical complexity (non-CMC)., Design, Setting, and Participants: This population-based repeated cross-sectional study used Canadian health administrative data of children aged younger than 18 years in community and pediatric hospitals during a pandemic period (April 1, 2020, to February 28, 2022) compared with a 3-year prepandemic period (April 1, 2017, to March 31, 2020). The pandemic period was analyzed separately for year 1 (April 1, 2020, to March 31, 2021) and year 2 (April 1, 2021, to February 28, 2022). Statistical analysis was performed from October 2022 to April 2023., Main Outcomes and Measures: Respiratory-related hospitalizations, ICU admissions, and mortality before and during the pandemic among CMC and non-CMC., Results: A total of 139 078 respiratory hospitalizations (29 461 respiratory hospitalizations for CMC and 109 617 for non-CMC) occurred during the study period. Among CMC, there were fewer respiratory hospitalizations in both 2020 (rate ratio [RR], 0.44 [95% CI, 0.42-0.46]) and 2021 (RR, 0.55 [95% CI, 0.51-0.62]) compared with the prepandemic period. Among non-CMC, there was an even larger relative reduction in respiratory hospitalizations in 2020 (RR, 0.18 [95% CI, 0.17-0.19]) and a similar reduction in 2021 (RR, 0.55 [95% CI, 0.54-0.56]), compared with the prepandemic period. Reductions in ICU admissions for respiratory illness followed a similar pattern for CMC (2020: RR, 0.56 [95% CI, 0.53-0.59]; 2021: RR, 0.66 [95% CI, 0.63-0.70]) and non-CMC (2020: RR, 0.22 [95% CI, 0.20-0.24]; RR, 0.65 [95% CI, 0.61-0.69]). In-hospital mortality for these conditions decreased among CMC in both 2020 (RR, 0.63 [95% CI, 0.51-0.77]) and 2021 (RR, 0.72 [95% CI, 0.59-0.87])., Conclusions and Relevance: This cross-sectional study found a substantial decrease in severe respiratory disease resulting in hospitalizations, ICU admissions, and mortality during the first 2 years of the pandemic compared with the 3 prepandemic years. These findings suggest that future evaluations of the effect of public health interventions aimed at reducing circulating respiratory pathogens during nonpandemic periods of increased respiratory illness may be warranted.

Objective/background: Moderate-to-severe obstructive sleep apnea (OSA) is highly prevalent in children with obesity and/or underlying medical complexity. The first line of therapy, adenotonsillectomy (AT), does not cure OSA in more than 50% of these children. Consequently, continuous positive airway pressure (CPAP) is the main therapeutic option but adherence is often poor. A potential alternative which may be associated with greater adherence is heated high-flow nasal cannula (HFNC) therapy; however, its efficacy in children with OSA has not been systematically investigated. The study aimed to compare the efficacy of HFNC with CPAP to treat moderate-to-severe OSA with the primary outcome measuring the change from baseline in the mean obstructive apnea/hypopnea index (OAHI)., Participants/methods: This was a single-blinded randomized, two period crossover trial conducted from March 2019 to December 2021 at a Canadian pediatric quaternary care hospital. Children aged 2-18 years with obesity and medical complexity diagnosed with moderate-to-severe OSA via overnight polysomnography and recommended CPAP therapy were included in the study. Following diagnostic polysomnography, each participant completed two further sleep studies; a HFNC titration study and a CPAP titration study (9 received HFNC first, and 9 received CPAP first) in a random 1:1 allocation order., Results: Eighteen participants with a mean ± SD age of 11.9 ± 3.8 years and OAHI 23.1 ± 21.7 events/hour completed the study. The mean [95% CI] reductions in OAHI (-19.8[-29.2, -10.5] vs. -18.8 [-28.2, -9.4] events/hour, p = 0.9), nadir oxygen saturation (7.1[2.2, 11.9] vs. 8.4[3.5, 13.2], p = 0.8), oxygen desaturation index (-11.6[-21.0, -2.3] vs. -16.0[-25.3, -6.6], p = 0.5) and sleep efficiency (3.5[-4.8, 11.8] vs. 9.2[0.9, 15.5], p = 0.2) with HFNC and CPAP therapy were comparable between conditions., Conclusion: HFNC and CPAP therapy yield similar reductions in polysomnography quantified measures of OSA severity among children with obesity and medical complexities., Trial Registration: NCT05354401 ClinicalTrials.gov., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objectives: Decisions to pause all non-essential paediatric hospital activities during the initial phase of the COVID-19 pandemic may have led to significant delays, deferrals and disruptions in medical care. This study explores clinical cases where the care of children was perceived by hospital clinicians to have been negatively impacted because of the changes in healthcare delivery attributing to the restrictions placed resulting from the COVID-19 pandemic., Design and Setting: This study used a mixed-methods approach using the following: (1) a quantitative analysis of overall descriptive hospital activity between May and August 2020, and utilisation of data during the study period was performed, and (2) a qualitative multiple-case study design with descriptive thematic analysis of clinician-reported consequences of the COVID-19 pandemic on care provided at a tertiary children's hospital., Results: Hospital-level utilisation and activity patterns revealed a substantial change to hospital activity including an initial reduction in emergency department attendance by 38% and an increase in ambulatory virtual care from 4% before COVID-19 to 67% between May and August 2020. Two hundred and twelve clinicians reported a total of 116 unique cases. Themes including (1) timeliness of care, (2) disruption of patient-centred care, (3) new pressures in the provision of safe and efficient care and (4) inequity in the experience of the COVID-19 pandemic emerged, each impacting patients, their families and healthcare providers., Conclusion: Being aware of the breadth of the impact of the COVID-19 pandemic across all of the identified themes is important to enable the delivery of timely, safe, high-quality, family-centred paediatric care moving forward., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Descriptions of the COVID-19 pandemic's indirect consequences on children are emerging. We aimed to describe the impacts of the pandemic on children with medical complexity (CMC) and their families., Methods: A one-time survey of Canadian paediatricians using the Canadian Paediatric Surveillance Program (CPSP) was conducted in Spring 2021., Results: A total of 784 paediatricians responded to the survey, with 70% (n = 540) providing care to CMC. Sixty-seven (12.4%) reported an adverse health outcome due to a COVID-19 pandemic-related disruption in healthcare delivery. Disruption of the supply of medication and equipment was reported by 11.9% of respondents (n = 64). Respondents reported an interruption in family caregiving (47.5%, n = 252) and homecare delivery (40.8%, n = 218). Almost 47% of respondents (n = 253) observed a benefit to CMC due to COVID-19 related changes in healthcare delivery, including increased availability of virtual care and reduction in respiratory illness. Some (14.4%) reported that CMC were excluded from in-person learning when their peers without medical complexity were not., Conclusion: Canadian paediatricians reported that CMC experienced adverse health outcomes during the COVID-19 pandemic, including disruptions to family caregiving and community supports. They also describe benefits related to the pandemic including the expansion of virtual care. These results highlight the need for healthcare, community and education policymakers to collaborate with families to optimize their health., (© 2022. The Author(s).)

Objectives: Family income is an important determinant of child and parental health. In Canada, cash transfer programs to families with children have existed since 1945. This systematic review aimed to examine the association between cash transfer programs to families with children and health outcomes in Canadian children (ages 0 to 18) as well as family economic outcomes., Methods: We reviewed academic and grey literature published up to November 2021. Additional studies were identified through reference review. We included any study that examined children 0-18 years old and/or their parents, took place in Canada and reported Canada-specific data, and reported child, youth and/or parental health outcomes, as well as family economic outcomes. Risk of bias was assessed by two reviewers using a modified Newcastle-Ottawa Scale., Synthesis: Our search yielded 23 studies meeting the inclusion criteria out of 7052 identified. Eight studies in total measured child health outcomes, including birth outcomes, child overall health, and developmental and behavioural outcomes, and four directly addressed parental health, including mental health, injuries, and obesity. Most studies reported generally positive associations, though some findings were specific to certain subgroups. Some studies also examined fertility and labour force participation outcomes, which described varying effects., Conclusion: Cash transfer programs to families with children in Canada are associated with better child and parental health outcomes. Additional research is needed to evaluate the mechanisms of effects, and to identify which types and levels of government transfers are most effective, and target populations, to optimize the positive effects of these benefits., (© 2022. The Author(s) under exclusive license to The Canadian Public Health Association.)

Children with medical complexity may be at elevated risk of experiencing child maltreatment and child welfare system involvement, though empirical data are limited. This study examined the extent of child welfare system involvement among children with medical complexity and investigated associated health and social factors. A retrospective chart review of children with medical complexity (N = 208) followed at a pediatric hospital-based complex care program in Canada was conducted. Descriptive statistics and odds ratios using logistic regression were computed. Results showed that nearly one-quarter (23.6%) had documented contact with the child welfare system, most commonly for neglect; of those, more than one-third (38.8%) were placed in care. Caregiver reported history of mental health problems (aOR = 3.19, 95%CI = 1.55-6.56), chronic medical conditions (aOR = 2.86, 95%CI = 1.09-7.47), and interpersonal violence or trauma (aOR = 17.58, 95%CI = 5.43-56.98) were associated with increased likelihood of child welfare system involvement, while caregiver married/common-law relationship status (aOR = 0.35, 95%CI = 0.16-0.74) and higher number of medical technology supports (aOR = 0.75, 95%CI = 0.57-0.99) were associated with decreased likelihood. Implications for intervention and prevention of maltreatment in children with high healthcare needs are discussed.

Background and Objectives: Caregivers of children with medical complexity (CMC) face many stressors related to their child's medical condition(s). Financial stress and its impact on housing has been reported to be a challenge among this population. However, unique housing challenges specific to CMC, including disability accommodations in the home and housing space and layout, have yet to be examined in the literature., Methods: We conducted 20 individual semistructured interviews with parents of CMC. Interviews were recorded, coded, and analyzed by using thematic analysis to emphasize, examine, and record patterns of meaning within the data., Results: Eighteen mothers and 2 fathers participated in individual interviews. Two major themes and subthemes (in parentheses) were identified: (1) the impact of health on housing (housing preferences, housing possibilities, and housing outcome as a trade-off) and (2) the impact of housing on health (health of the caregiver and health of the child). Parents had preferences regarding the location and layout of their home specific to their child's illness and medical needs. In addition, parents indicated their child's illness affected their income and home ownership status, which in turn shaped their housing possibilities. The location and layout of the family home was often the result of a trade-off between the caregiver's housing preferences and possibilities., Conclusions: Housing outcomes among CMC are often the result of a trade-off between housing preferences and possibilities, both of which are influenced by the child's health status. Policy changes targeting housing accessibility and affordability are vital to support the health of CMC., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Importance: Children with medical complexity (CMC) represent a growing population in the pediatric health care system, with high resource use and associated health care costs. A genetic diagnosis can inform prognosis, anticipatory care, management, and reproductive planning. Conventional genetic testing strategies for CMC are often costly, time consuming, and ultimately unsuccessful., Objective: To evaluate the analytical and clinical validity of genome sequencing as a comprehensive diagnostic genetic test for CMC., Design, Setting, and Participants: In this cohort study of the prospective use of genome sequencing and comparison with standard-of-care genetic testing, CMC were recruited from May 1, 2017, to November 30, 2018, from a structured complex care program based at a tertiary care pediatric hospital in Toronto, Canada. Recruited CMC had at least 1 chronic condition, technology dependence (child is dependent at least part of each day on mechanical ventilators, and/or child requires prolonged intravenous administration of nutritional substances or drugs, and/or child is expected to have prolonged dependence on other device-based support), multiple subspecialist involvement, and substantial health care use. Review of the care plans for 545 CMC identified 143 suspected of having an undiagnosed genetic condition. Fifty-four families met inclusion criteria and were interested in participating, and 49 completed the study. Probands, similarly affected siblings, and biological parents were eligible for genome sequencing., Exposures: Genome sequencing was performed using blood-derived DNA from probands and family members using established methods and a bioinformatics pipeline for clinical genome annotation., Main Outcomes and Measures: The primary study outcome was the diagnostic yield of genome sequencing (proportion of CMC for whom the test result yielded a new diagnosis)., Results: Genome sequencing was performed for 138 individuals from 49 families of CMC (29 male and 20 female probands; mean [SD] age, 7.0 [4.5] years). Genome sequencing detected all genomic variation previously identified by conventional genetic testing. A total of 15 probands (30.6%; 95% CI 19.5%-44.6%) received a new primary molecular genetic diagnosis after genome sequencing. Three individuals had novel diseases and an additional 9 had either ultrarare genetic conditions or rare genetic conditions with atypical features. At least 11 families received diagnostic information that had clinical management implications beyond genetic and reproductive counseling., Conclusions and Relevance: This study suggests that genome sequencing has high analytical and clinical validity and can result in new diagnoses in CMC even in the setting of extensive prior investigations. This clinical population may be enriched for ultrarare and novel genetic disorders. Genome sequencing is a potentially first-tier genetic test for CMC.

Introduction: The support of families in the care of children with medical complexity (CMC) requires the integration of health care providers' (HCPs') medical knowledge and family experience. Care plans largely represent HCP information, and care maps demonstrate the family experience. Understanding the intersection between a care plan and a care map is critical, as it may provide solutions to the widely recognized tension between HCP-directed care and patient- and family-centered care (PFCC)., Method: This study used qualitative methods to explore the experience and usefulness of care maps. Parents of CMC who already had a care plan, created care maps (n = 15). Subsequent interviews with parents (n = 15) and HCPs (n = 30) of CMC regarding both care maps and care plans were conducted and analyzed using thematic analysis., Results: Data analysis exploring the relationship and utility of care plans and care maps revealed six primary themes related to using care plans and care maps that were grouped into two primary categories: (a) utility of care plans and maps; and (b) intersection of care plans and care maps., Discussion: Care plans and care maps were identified as valuable complementary documents. Their integration offers context about family experience and respects the parents' experiential wisdom in a standard patient care document, thus promoting improved understanding and integration of the family experience into care decision making., (© 2018 John Wiley & Sons Ltd.)

Competing Interests: Competing interests: Nora Fayed reports that the public funder of the health intervention, the Provincial Council for Maternal and Child Health, was a partner in the work and provided in-kind support. The council did not fund Nora Fayed’s program or institution. The authors are arm’s-length evaluators and have not influenced the intervention. No other competing interests were declared.

Background: ERCC6L2-associated disorder has recently been described and only five patients were reported so far. The described phenotype included bone marrow, cerebral, and craniofacial abnormalities. The aim of this study was to further define the genetic and phenotypic spectrum of the disorder by summarizing the five published cases and an additional case that we identified through whole-exome sequencing performed at the University of Toronto., Methods: Clinical data was extracted from the Canadian Inherited Marrow Failure Registry. Whole exome sequencing was performed to identify causative mutations., Results: All six cases had homozygous truncating mutations either at or upstream of the helicase domain of ERCC6L2. All patients displayed bone marrow failure, learning or developmental delay and microcephaly. Our patient was unique in displaying features of cerebellar disease, including ataxia and dysmetria as well as an interval deterioration of the corpus callosum and generalized volume loss on MRI. Another unique feature of our patient was retinal dystrophy with macular involvement. Along with one other patient, our patient displayed craniofacial abnormalities by presenting with low-set prominent ears, a pointed prominent chin, and deep-set eyes. Leukemia is common among patients with inherited bone marrow failure, but thus far, none of the patients have developed this complication., Conclusions: ERCC6L2-associated disorder is a multisystem disorder. The phenotype spectrum includes bone marrow failure, cerebral, and craniofacial abnormalities, as well as cerebellar and retinal abnormalities., (© 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)

Objectives: Family caregivers of children who depend on medical technology (CMT) provide highly skilled care up to 24 hours per day. Sleep disruption places family caregivers at risk for poor health and related outcomes that threaten their long-term caregiving capacity. Few studies exist that have measured sleep in family caregivers, and most have relied entirely on subjective measures., Methods: In a prospective cohort study, family caregivers of CMT (n=42) and caregivers of healthy children (n=43) were recruited. Actigraphy data and a concurrent sleep diary were collected for 6 days/7 nights. Measures of sleep quality, depression, sleepiness, fatigue and quality of life were also administered., Results: Family caregivers of CMT averaged fewer hours of sleep per night (mean (SD)) (6.56 ± 1.4 vs 7.21 ± 0.6, p = 0.02) of poorer quality (7.75 ± 2.9 vs 5.45 ± 2.8, p < 0.01) than the control group. Three times as many family caregivers of CMT scored in the range for significant depressive symptomatology (12(33%) vs 4(10%), p = 0.01) and experienced excessive daytime sleepiness (16(38%) vs 5(12%), p < 0.01). Fatigue was also more problematic among family caregivers of CMT (22.12 ± 9.1 vs 17.44 ± 9.0, p = 0.02)., Conclusions: Family caregivers of CMT are at risk of acute and chronic sleep deprivation, psychological distress and impaired daytime function that may threaten their capacity for sustained caregiving. Family caregivers of CMT may be important targets for screening for sleep disorders and the development of novel sleep-promoting interventions., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Transcatheter aortic valve implantation (TAVI) is a relatively new procedure to treat aortic stenosis in patients at high surgical risk, and it is becoming increasingly available in Canada. Variation exists in the clinical care, program coordination, evaluation, and funding across provinces and centres. As a part of the Canadian Cardiovascular Society (CCS) quality initiative, the TAVI Quality Indicator (QI) Working Group was established in 2014 to develop a set of indicators to measure quality of care for Canadians undergoing TAVI for aortic stenosis. The TAVI QI Working Group is composed of expert clinical and government agency representatives. The group developed consensus agreements for the selection of the first iteration of measurable structure, process, and outcome indicators reflective of the quality of care for patients undergoing TAVI. The objectives of the project are to develop quality indicators with the eventual goal of standardizing TAVI quality reports across Canada and to support local and national quality assurance, as well as engage multiple stakeholders to build a national strategy for the evaluation of quality of care., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background and Objective: Medical education relies heavily on workplace learning where trainees are educated through their clinical experience. Few studies have explored trainees' perceptions of the educational value of these patient care experiences. The aim of this study was to identify pediatric patient characteristics that medical trainees perceive as educationally valuable., Methods: Over 2 months, trainees on pediatric inpatient wards ranked the perceived educational value of patients under their care on a 4-point bipolar Likert scale. Three patient characteristics were examined: complex-chronic and noncomplex-chronic preexisting conditions, difficult social circumstances, and rare diseases. Patient-level predictors of cases perceived as educationally valuable (defined as scores≥3) were examined by using univariate and multivariate analyses., Results: A total of 325 patients were rated by 51 trainees (clinical medical students [45%], first-year residents [29%], third-year residents/fellows [26%]). Rare diseases had a higher educational value score (adjusted odds ratio 1.76, 95% confidence interval 1.08-2.88, P=.02). Complex-chronic and noncomplex-chronic preexisting conditions and difficult social circumstances did not affect the perceived educational value., Conclusions: Trainees attribute the most educational value to caring for patients with rare diseases. Although trainees' perceptions of learning do not necessarily reflect actual learning, they may influence personal interest and limit learning from an educational experience. Knowledge of trainee perceptions of educational experience therefore can direct medical educators' approaches to inpatient education., (Copyright © 2015 by the American Academy of Pediatrics.)

Objectives: To assess use, utility and impact of transition interventions designed to support and empower self-management in youth with chronic health conditions during transition into adult healthcare., Design: A 4-year mixed-method prospective cohort study., Setting: 2 academic paediatric hospitals (13 clinics) in Canada., Participants: 50 adolescents (42% male; mean age 17.9±0.9 years; 20 underlying diagnoses) with transfer to adult care planned within 1 year., Interventions: The Youth KIT (an organisational tool that includes goal setting activities); an online transition mentor., Main Outcome Measures: Frequency of use, utility and impact of the transition interventions; goal achievement; post-transfer qualitative interviews with youth., Results: 50 participants were enrolled during their last year of paediatric care; 36 (72%) were followed into adult care. All participants had access to the transition interventions from enrolment until the end of the study (exposure time: 12-47 months). Most youth (85%) reported using the medical/health section of the Youth KIT at least once; 20 (40%) participants engaged in chats with the mentor. The overall perceived utility of both interventions was modest; the Youth KIT received the highest ratings for 'help with goal setting': (mean (SD): 4.2 (2.3)) on a 7-point Likert scale. 45 (90%) participants set 294 transition goals. Goal achievement performance and satisfaction increased over time (p≤0.001). The qualitative evidence revealed reasons behind the variability in use and utility of the interventions, the interconnectedness of life-course and healthcare transitions, and the need for stronger partnerships between paediatric and adult healthcare systems., Conclusions: Participants' perceptions about the utility of the Youth KIT and the online mentor were modest. Transition supports need to be carefully tailored, timed and integrated into healthcare systems. Individualised goal setting may be an important 'active ingredient' in optimising transition supports and outcomes. Interventions that focus on youth only are insufficient for empowering self-management., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

This position statement addresses issues in revascularization for multivessel coronary artery disease (CAD) from the perspective of both cardiologists and cardiac surgeons. Recommendations are made based on evidence from clinical trials and observational studies, with an emphasis on the increasing number of individuals with significant comorbid disease burden and functional debilitation who are being referred for definitive management of their multivessel CAD in the context of routine clinical practice. These types of individuals have traditionally not been included in the many clinical trials that have been the basis for guidelines and recommendations, and the objective of the proposed medical intervention or revascularization (or both) would not necessarily be to improve prognosis but to improve quality of life. One purpose of this document is to propose practical multidisciplinary approaches to the management of these patients. Recommendations are made for revascularization in acute coronary syndromes and stable CAD, with specific considerations for individuals with left ventricular dysfunction and heart failure, chronic renal failure, and chronic obstructive pulmonary disease. We also consider the use of various risk scores, including the Society of Thoracic Surgeons score, the EuroSCORE, and the SYNTAX II score. The importance of a heart team approach is also emphasized. The complementary role of coronary bypass surgery and percutaneous coronary intervention is highlighted, along with the importance of optimal medical therapy., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Thromboprophylaxis remains often underused in hospitalized patients. In 2001, a cohort study done at our institution, a tertiary care center in Montreal, Canada, showed that 67.7% of VTE cases necessitating thromboprophylaxis were potentially preventable with adequate use of American College of Chest Physicians (ACCP) guidelines. Following implementation of an institution-wide policy in 2005, we assessed the changes in the rate of potentially preventable VTE. We conducted a retrospective cohort study including all hospitalized patients with objectively diagnosed VTE in 2010 at our institution. Each case was classified as preventable (thromboprophylaxis indicated but inadequately administered), non-preventable (thromboprophylaxis indicated and correctly administered), spontaneous (thromboprophylaxis not indicated), and ineligible (contraindication to thromboprophylaxis). The results were compared to those obtained in 2001. Of the 230 cases of VTE, 55 cases were classified as potentially preventable (23.9%), 85 were non-preventable (37.0%), 74 were spontaneous (32.2%) and 16 (7.0%) were ineligible. Of the 140 cases requiring thromboprophylaxis, 39.3% were potentially preventable. The potentially preventable cases were mostly due to omission of thromboprophylaxis (50.9%), occurred during general medical admissions (74.5%), and the most common VTE risk factor was cancer (47.2%). In conclusion, we demonstrate a lower frequency of potentially preventable cases in 2010 compared to 2001 (39.3% vs 67.7%, respectively), partially due to physician education and adoption of an institution-wide policy. However, patients with medical indications for thromboprophylaxis, particularly those with cancer, are more prone to having preventable VTE, indicating an area for potential improvement., (© The Author(s) 2014.)

Purpose: The purpose of this study was to test the feasibility of a parent-briefing intervention for parents of hospitalized children with complex healthcare needs., Design and Methods: A phase I, single-group, posttest study. There were 18 physicians, 25 nurses, and 31 parents who participated in the study. Participants were asked to sit while carrying out the briefings with parents. Parents and clinicians completed a feasibility questionnaire post briefings., Results: Sixty-eight briefings were carried out. Parents and nurses evaluated the briefings in a favorable manner, whereas physicians' ratings were mixed., Practice Implications: Further inquiry is recommended to understand the effects of a structured communication intervention on parent-professional decision-making practices., (© 2014, Wiley Periodicals, Inc.)

Introduction: Care giving for children with chronic diseases can lead to financial strain and compromised family well being. Little is known about whether these stresses lead to changes in residential movement patterns as they relate to income adequacy and proximity to care., Methods: We compared the residential movement patterns and associated changes in neighbourhood income of children with mild to severe chronic diseases compared with those that are healthy. A cohort of infants born from 2002-2007 in Ontario, Canada was followed for 5 years and divided into those with single- or multiple- body system complex chronic conditions (CCCs); low birth weight (LBW); asthma/recurrent wheeze (A/RW) and the control group of otherwise healthy children., Results: Of 598,716 children studied, 15,207 had a single CCC, 3,600 multiple CCCs, 33,206 LBW, 57,137 A/RW and 489,566 were healthy. Lowest income quintile children were most likely to move residence. Compared with healthy controls, chronic disease cohorts, apart from those with asthma, were more likely to be born in the lowest income quintile neighbourhood and to move. Among children who moved, all chronic disease cohorts were significantly more likely to move to a low income quintile neighborhood (adjusted odds ratios for all chronic disease cohorts of 1.1-1.2). There were no differences across cohorts in residential movement close to a children's hospital., Conclusions: Young children with chronic conditions, particularly those born in low income neighbourhoods, are more likely to move residence than other healthy young children. However, it does not seem that proximity to specialized care is driving this movement. Further research is required to determine if these movement patterns impact the ability of children with chronic conditions to secure health services.

Population health intervention research requires stronger definition. There are overlaps and differences between it and established domains such as evaluation, health impact assessment, knowledge translation, health services research, and social and public policy analysis. The value added of this growing field is its potential to draw more resources as well as diverse expertise, methods and ways of knowing under one umbrella at a critical time in history. That is, at a time when actions to reduce health inequities have become paramount.

Background: Limited data are available on adverse events among children admitted to hospital. The Canadian Paediatric Adverse Events Study was done to describe the epidemiology of adverse events among children in hospital in Canada., Methods: We performed a 2-stage medical record review at 8 academic pediatric centres and 14 community hospitals in Canada. We reviewed charts from patients admitted from April 2008 through March 2009, evenly distributed across 4 age groups (0 to 28 d; 29 to 365 d; > 1 to 5 yr and > 5 to 18 yr). In stage 1, nurses and health records personnel who had received training in the use of the Canadian Paediatric Trigger Tool reviewed medical records to detect triggers for possible adverse events. In stage 2, physicians reviewed the charts identified as having triggers and described the adverse events., Results: A total of 3669 children were admitted to hospital during the study period. The weighted rate of adverse events was 9.2%. Adverse events were more frequent in academic pediatric centres than in community hospitals (adjusted odds ratio [OR] 2.98, 95% confidence interval [CI] 1.65-5.39). The incidence of preventable adverse events was not significantly different between types of hospital, but nonpreventable adverse events were more common in academic pediatric centres (adjusted OR 4.39, 95% CI 2.08-9.27). Surgical events predominated overall and occurred more frequently in academic pediatric centres than in community hospitals (37.2% v. 21.5%, relative risk [RR] 1.7, 95% CI 1.0-3.1), whereas events associated with diagnostic errors were significantly less frequent (11.1% v. 23.1%, RR 0.5, 95% CI 0.2-0.9)., Interpretation: More children have adverse events in academic pediatric centres than in community hospitals; however, adverse events in the former are less likely to be preventable. There are many opportunities to reduce harm affecting children in hospital in Canada, particularly related to surgery, intensive care and diagnostic error.

Objective: To examine the effectiveness of hospital-based comprehensive care programs in improving the quality of care for children with special health care needs., Data Sources: A systematic review was conducted using Ovid MEDLINE, CINAHL, EMBASE, PsycINFO, Sociological Abstracts SocioFile, and Web of Science., Study Selection: Evaluations of comprehensive care programs for categorical (those with single disease) and noncategorical groups of children with special health care needs were included. Selected articles were reviewed independently by 2 raters., Data Extraction: Models of care focused on comprehensive care based at least partially in a hospital setting. The main outcome measures were the proportions of studies demonstrating improvement in the Institute of Medicine's quality-of-care domains (effectiveness of care, efficiency of care, patient or family centeredness, patient safety, timeliness of care, and equity of care)., Data Synthesis: Thirty-three unique programs were included, 13 (39%) of which were randomized controlled trials. Improved outcomes most commonly reported were efficiency of care (64% [49 of 76 outcomes]), effectiveness of care (60% [57 of 95 outcomes]), and patient or family centeredness (53% [10 of 19 outcomes). Outcomes less commonly evaluated were patient safety (9% [3 of 33 programs]), timeliness of care (6% [2 of 33 programs]), and equity of care (0%). Randomized controlled trials occurred more frequently in studies evaluating categorical vs noncategorical disease populations (11 of 17 [65%] vs 2 of 16 [17%], P = .008)., Conclusions: Although positive, the evidence supporting comprehensive hospital-based programs for children with special health care needs is restricted primarily to nonexperimental studies of children with categorical diseases and is limited by inadequate outcome measures. Additional high-quality evidence with appropriate comparative groups and broad outcomes is necessary to justify continued development and growth of programs for broad groups of children with special health care needs.

Providing integrated care for children with medical complexity in Canada is challenging as these children are, by definition, in need of coordinated care from disparate providers, organizations and funders across the continuum in order to optimize health outcomes. We describe the development of an inter-organizational team constructed as a unique tripartite partnership of an acute care hospital, a children's rehabilitation hospital and a home/community health organization focused on children who frequently use services across these three organizations. Model building and operationalization within the Canadian healthcare system is emphasized. Key challenges identified to date include communication and policy barriers as well as optimizing interactions with families; critical enablers have been alignment with policy trends in healthcare and inter-organizational commitment to integrate at the point of care. Considerations for policy developments supporting full integration across service sectors are raised. Early indicators of success include the enrolment of 34 clients and patients and the securing of funds to evaluate and expand the model to serve more children.

Background: The REduction of Atherothrombosis for Continued Health (REACH) Registry is an international, prospective cohort of 68,236 patients with established coronary artery, cerebrovascular or peripheral arterial disease, or three or more atherothrombotic risk factors. Baseline data from the 1976 Canadian patients in the REACH Registry provide opportunities to assess atherothrombotic risk and treatment in a real-world Canadian setting., Objectives: To present baseline characteristics of Canadian REACH Registry patients, and to compare cardiovascular risk and treatment among Canadian, United States (USA) and global patients., Methods: Patients 45 years of age or older with established atherosclerotic vascular disease or three or more cardiovascular risk factors were enrolled during 2004. Baseline data were used in analyses of risk factor prevalence and control and medication use. Comparisons between the Canadian and USA populations, Canadian and global populations, and the Canadian regions were conducted., Results: Of the 1976 Canadian REACH patients, 82.5% had documented vascular disease, 12.6% of whom had manifestations in more than one vascular bed (polyvascular disease). A high prevalence of hypercholesterolemia (84.4%), hypertension (76.6%) and diabetes mellitus (43.7%) were noted, and 75.1% of patients were overweight or obese. Of the 1976 Canadian REACH patients, 75.1% were at target cholesterol levels, 67.4% were at target fasting blood glucose levels and 60.6% were at target blood pressure levels. Significant differences existed in the prevalence of risk factors and their management among Canadian, USA and global REACH populations, as well as within Canada., Conclusions: Canada compared favourably with USA and global REACH populations in the use of proven risk-reducing medications.

The 1995 Consensus Conference of the Canadian Cardiovascular Society on "Indications for and Access to Revascularization" recommended that Canadian centres with invasive cardiovascular facilities should participate in a national observational database that monitors the selection of patients, as well as evaluate outcomes. The Canadian Cardiovascular Society, the Heart and Stroke Foundation of Canada, and Health Canada with IBM as a partner, initiated a process to identify factors influencing the development of the Canadian Cardiovascular Information Network. IBM's "Business Discovery Methodology" was adapted for health care. Structured interviews with representatives of health organizations, cardiovascular databases and research institutes were conducted across Canada, followed by a workshop to identify goals, issues and challenges. Participants identified goals for a cardiovascular database (eg, evidence-based decision-making), project related issues (eg, respecting the integrity of existing databases) and health care related issues (eg, cardiac waiting lists). Challenges included initial mistrust between representatives of provincial cardiovascular databases and national agencies, and a lack of sustained funding. A Project Team was formed to address 'cardiac waiting lists'. Analysis of Alberta and Ontario data identified differences in definitions, such as when the waiting time for bypass surgery began, that impeded detailed comparisons. Development of a centralized national database was not feasible at this time for political, technical and financial reasons. However, provincial cardiovascular database representatives agreed to work together and to share aggregate data and analyses. A first step toward developing a national surveillance system for cardiovascular services will be achieving consensus about standardizing data definitions. This process will require sustained funding.

The objective of this study was to examine whether there are international variations in the use of evidence-based medical therapy in patients undergoing percutaneous coronary revascularization. We analyzed the medical therapy of patients in the United States (US) (n = 878), Europe (n = 134), and Canada (n = 274) who underwent percutaneous coronary revascularization in either the Coronary Angioplasty Versus Excisional Atherectomy Trial (CAVEAT-I) (enrollment from August 1991 to April 1992) or the Canadian Coronary Atherectomy Trial (CCAT) (enrollment from July 1991 to August 1992). We found that at the time of hospital admission, Canadian patients had the highest rates of treatment with aspirin (95% vs 57% US and 78% Europe; p = 0.002), calcium antagonists (75% vs 48% US and 43% Europe; p 0.0001), beta blockers (60% vs 32% US and 46% Europe; p = 0.02), and combination anti-ischemic therapy (67% vs 43% US and 56% Europe; p = 0.0001). By discharge, however, Canadian patients had the lowest rates of treatment with nitrates (12% vs 40% US and 44% Europe; p = 0.0001) and combination anti-ischemic therapy (29% vs 53% US and 47% Europe; p < 0.01). At both admission and discharge, rates of treatment with angiotensin-converting enzyme inhibitors and lipid-lowering agents were < 15% in all 3 regions. We conclude that significant international variations exist in the use of evidence-based medical therapy in patients undergoing percutaneous coronary revascularization.

Objective: To assess the procedural success and complication rates of the first 120 directional coronary atherectomy cases performed at two Toronto hospitals., Design and Setting: Case series in tertiary referral centres., Patients: One hundred and thirteen patients in whom 120 atherectomy procedures were attempted between July 1990 and April 1992., Intervention: Directional coronary atherectomy., Main Results: Angiographic success was obtained in 115 of 120 procedures (96%) involving 117 of 123 lesions (95%). Procedural success (angiographic success without death, myocardial infarction or coronary bypass surgery) was obtained in 110 of 120 procedures (92%). Adjunctive balloon angioplasty was required in 20 procedures (17%). There was one death at 36 h in an elderly patient who underwent an emergency procedure while in cardiogenic shock. Periprocedural non-Q wave myocardial infarction occurred in five patients. There were no Q wave myocardial infarctions. Three patients required coronary bypass surgery prior to discharge and vascular complications occurred in five patients., Conclusions: Directional coronary atherectomy can be performed with procedural success and complication rates comparable to conventional balloon angioplasty. Randomized trials are underway to determine if atherectomy results in a lower restenosis rate.