Neurological involvement in hospitalized children with SARS-CoV-2 infection: a multinational study.

BACKGROUND AND OBJECTIVES: Neurological involvement associated with SARS-CoV-2 infection is increasingly recognized. However, the specific characteristics and prevalence in pediatric patients remain unclear. The objective of this study was to describe the neurological involvement in a multinational cohort of hospitalized pediatric patients with SARS-CoV-2. METHODS: This was a multicenter observational study of children <18 years of age with confirmed SARS-CoV-2 infection or multisystemic inflammatory syndrome (MIS-C) and laboratory evidence of SARS-CoV-2 infection in children, admitted to 15 tertiary hospitals/healthcare centers in Canada, Costa Rica, and Iran February 2020-May 2021. Descriptive statistical analyses were performed and logistic regression was used to identify factors associated with neurological involvement. RESULTS: One-hundred forty-seven (21%) of 697 hospitalized children with SARS-CoV-2 infection had neurological signs/symptoms. Headache (n = 103), encephalopathy (n = 28), and seizures (n = 30) were the most reported. Neurological signs/symptoms were significantly associated with ICU admission (OR: 1.71, 95% CI: 1.15-2.55; p = 0.008), satisfaction of MIS-C criteria (OR: 3.71, 95% CI: 2.46-5.59; p < 0.001), fever during hospitalization (OR: 2.15, 95% CI: 1.46-3.15; p < 0.001), and gastrointestinal involvement (OR: 2.31, 95% CI: 1.58-3.40; p < 0.001). Non-headache neurological manifestations were significantly associated with ICU admission (OR: 1.92, 95% CI: 1.08-3.42; p = 0.026), underlying neurological disorders (OR: 2.98, 95% CI: 1.49-5.97, p = 0.002), and a history of fever prior to hospital admission (OR: 2.76, 95% CI: 1.58-4.82; p < 0.001). DISCUSSION: In this study, approximately 21% of hospitalized children with SARS-CoV-2 infection had neurological signs/symptoms. Future studies should focus on pathogenesis and long-term outcomes in these children.

Experiences of Inpatient Healthcare Services Among Children With Medical Complexity and Their Families: A Scoping Review.

BACKGROUND: Children with medical complexity (CMC) have high healthcare utilization and face unique challenges during hospital admissions. The evidence describing their experiences of inpatient care is distributed across disciplines. The aim of this scoping review was to map the evidence related to the inpatient experience of care for CMC and their families, particularly related to key aspects and methodological approaches, and identify gaps that warrant further study. METHODS: This scoping review was conducted in accordance with JBI methodology and included all studies that reported experiences of acute hospital care for CMC/families. All study designs were included. Databases searched included EMBASE, CINAHL Plus with Full Text, Web of Science, MEDLINE(R) and APA PsycInfo from 2000 to 2022. Details about the participants, concepts, study methods and key findings were abstracted using a data abstraction tool. A thematic analysis was conducted. RESULTS: Forty-nine papers were included: 27 qualitative studies, 10 quantitative studies, six mixed methods studies, two descriptive studies and four reviews. Some quantitative studies used validated instruments to measure experience of care, but many used non-validated surveys. There were a few interventional studies with a small sample size. Results of thematic analysis described the importance of negotiating care roles, shared decision-making, common goal setting, relationship-building, communication, sharing expertise and the hospital setting itself. CONCLUSION: CMC and families value relational elements of care and partnering through sharing expertise, decision-making and collaborative goal-setting when admitted to hospital. PATIENT OR PUBLIC CONTRIBUTION: This review was conducted in alignment with the principles of patient and family engagement. The review was conceptualized, co-designed and conducted with the full engagement of the project's parent-partner. This team member was involved in all stages from constructing the review question, to developing the protocol, screening articles and drafting this manuscript.

SARS-CoV-2 infection in technology-dependent children: a multicenter case series.

PURPOSE: The objective of this study was to describe the clinical course and outcomes in children with technology dependence (TD) hospitalized with SARS-CoV-2 infection. METHODS: Seventeen pediatric hospitals (15 Canadian and one each in Iran and Costa Rica) included children up to 17 years of age admitted February 1, 2020, through May 31, 2021, with detection of SARS-CoV-2. For those with TD, data were collected on demographics, clinical course and outcome. RESULTS: Of 691 children entered in the database, 42 (6%) had TD of which 22 had feeding tube dependence only, 9 were on supplemental oxygen only, 3 had feeding tube dependence and were on supplemental oxygen, 2 had a tracheostomy but were not ventilated, 4 were on non-invasive ventilation, and 2 were on mechanical ventilation prior to admission. Three of 42 had incidental SARS-CoV-2 infection. Two with end-stage underlying conditions were transitioned to comfort care and died. Sixteen (43%) of the remaining 37 cases required increased respiratory support from baseline due to COVID-19 while 21 (57%) did not. All survivors were discharged home. CONCLUSION: Children with TD appear to have an increased risk of COVID-19 hospitalization. However, in the absence of end-stage chronic conditions, all survived to discharge.

Routine Elective Gastrojejunostomy Tube Changes Are Associated With Reduced Tube Complications and Radiation Exposure.

Gastrojejunostomy tubes (GJTs) can be a long-term solution for patients with intragastric feeding intolerance. Our retrospective study of 101 patients correlates the frequency of routine and urgent GJT changes, as well as complications and radiation exposure. Over a 2.75-year median duration, 60%, 33%, and 28% of patients had >1 episodes of a tube dislodgement/malpositioning, blockage, or leakage, respectively. Aspiration pneumonia hospital admission was required for 23% of patients. Patients with <1 routine tube change/year had more urgent changes/year (3.0) compared to patients with 1-2 (1.2) or >2 (0.8) routine yearly change. These patients required more frequent sedation for tube placement (21% vs 4.7%, P = 0.03) and experienced greater annual radiation exposure (9599 vs 304.5 and 69.1 µGym 2 , P = 0.01 and 0.008, respectively). Overall, aiming for a routine tube change at least every 6-12 months is associated with fewer urgent changes and complications as well as reduced radiation exposure and sedation requirements.

Care coordination of children with neurodevelopmental disabilities and medical complexity during the COVID-19 pandemic: Caregiver experiences.

BACKGROUND: The COVID-19 pandemic and subsequent public health restrictions created significant challenges for children with neurodevelopmental disabilities with medical complexity and their caregivers including restrictions in care coordination for children and their families. Care coordination enhances families' skills in accessing and coordinating medical, education and disability care across sectors and systems. OBJECTIVE: This study examined the implications of pandemic restrictions on care coordination from caregiver perspectives. These experiences can inform emergency preparedness planning and recovery strategies. METHOD: A qualitative descriptive design was utilized to explore and describe the experience of caregivers of children with neurodevelopmental disabilities and medical complexity. Nineteen caregivers were interviewed about their experience with care coordination during the pandemic. FINDINGS: Caregiver experiences of care coordination during the pandemic highlighted the importance of care coordination during a public health emergency. Two themes emerged: (1) disruptions to care coordination from initial COVID-19 restrictions leading to lack of access to supports and services, increasing level of need, and impacts of disruption for caregivers and children; and (2) adaptation and responsiveness to COVID-19 restrictions by advocating for families and managing uncertainties. RECOMMENDATIONS: Recommendations include recognition of care coordination as a protective factor, designation as an essential service and sustained or increased funding for care coordination during emergencies. Families should be engaged in identifying care needs during care coordination, including during public health emergencies.

Experiences of medical traumatic stress in parents of children with medical complexity.

BACKGROUND AND OBJECTIVES: Parents of children with medical complexity (CMC) experience high levels of stress and adverse mental health outcomes. Pediatric medical traumatic stress (PMTS) could be an important contributor that has not yet been explored. PMTS describes parents' reactions to their child's illness and medical treatment and can lead to post-traumatic stress symptoms. This is the first study to describe the experiences and impact of PMTS among parents of CMC. METHODS: We conducted semi-structured interviews with 22 parents of CMC. Reflexive thematic analysis was used to generate themes that described the experiences of PMTS and potential contributing factors in the healthcare setting. Themes were validated by study participants. RESULTS: Parents experienced a spectrum of events and circumstances that impacted PMTS. These corresponded to three major themes: (a) the distinctive context of being the parent of a CMC, (b) interactions with healthcare providers that can hurt or heal and (c) system factors that set the stage for trauma. The consequences of repeated PMTS were a common point of emphasis among all the themes. Parents identified numerous changes that could mitigate PMTS such as acknowledgement of trauma and provision of proactive mental health support. CONCLUSIONS: Our study highlights the issue of PMTS among parents of CMC and presents opportunities to mitigate their traumatic experiences. Supporting the integration of trauma-informed care practices, increasing awareness of PMTS and advocating for parental mental health services could better support parents and families.

Predictors of severe illness in children with multisystem inflammatory syndrome after SARS-CoV-2 infection: a multicentre cohort study.

BACKGROUND: SARS-CoV-2 infection can lead to multisystem inflammatory syndrome in children (MIS-C). We sought to investigate risk factors for admission to the intensive care unit (ICU) and explored changes in disease severity over time. METHODS: We obtained data from chart reviews of children younger than 18 years with confirmed or probable MIS-C who were admitted to 15 hospitals in Canada, Iran and Costa Rica between Mar. 1, 2020, and Mar. 7, 2021. Using multivariable analyses, we evaluated whether admission date and other characteristics were associated with ICU admission or cardiac involvement. RESULTS: Of 232 children with MIS-C (median age 5.8 yr), 130 (56.0%) were male and 50 (21.6%) had comorbidities. Seventy-three (31.5%) patients were admitted to the ICU but none died. We observed an increased risk of ICU admission among children aged 13-17 years (adjusted risk difference 27.7%, 95% confidence interval [CI] 8.3% to 47.2%), those aged 6-12 years (adjusted risk difference 25.2%, 95% CI 13.6% to 36.9%) or those with initial ferritin levels greater than 500 µg/L (adjusted risk difference 18.4%, 95% CI 5.6% to 31.3%). Children admitted to hospital after Oct. 31, 2020, had numerically higher rates of ICU admission (adjusted risk difference 12.3%, 95% CI -0.3% to 25.0%) and significantly higher rates of cardiac involvement (adjusted risk difference 30.9%, 95% CI 17.3% to 44.4%). At Canadian sites, the risk of ICU admission was significantly higher for children admitted to hospital between December 2020 and March 2021 than those admitted between March and May 2020 (adjusted risk difference 25.3%, 95% CI 6.5% to 44.0%). INTERPRETATION: We observed that age and higher ferritin levels were associated with more severe MIS-C. We observed greater severity of MIS-C later in the study period. Whether emerging SARS-CoV-2 variants pose different risks of severe MIS-C needs to be determined.

Infants hospitalized for acute COVID-19: disease severity in a multicenter cohort study.

Age is the most important determinant of COVID-19 severity. Infectious disease severity by age is typically J-shaped, with infants and the elderly carrying a high burden of disease. We report on the comparative disease severity between infants and older children in a multicenter retrospective cohort study of children 0 to 17 years old admitted for acute COVID-19 from February 2020 through May 2021 in 17 pediatric hospitals. We compare clinical and laboratory characteristics and estimate the association between age group and disease severity using ordinal logistic regression. We found that infants comprised one-third of cases, but were admitted for a shorter period (median 3 days IQR 2-5 versus 4 days IQR 2-7), had a lower likelihood to have an increased C-reactive protein, and had half the odds of older children of having severe or critical disease (OR 0.50 (95% confidence interval 0.32-0.78)).    Conclusion: When compared to older children, there appeared to be a lower threshold to admit infants but their length of stay was shorter and they had lower odds than older children of progressing to severe or critical disease. What is Known: • A small proportion of children infected with SARS-CoV-2 require hospitalization for acute COVID-19 with a subgroup needing specialized intensive care to treat more severe disease. • For most infectious diseases including viral respiratory tract infections, disease severity by age is J-shaped, with infants having more severe disease compared to older children. What is New: • One-third of admitted children for acute COVID-19 during the first 14 months of the pandemic were infants. • Infants had half the odds of older children of having severe or critical disease.

The impact of the COVID-19 pandemic on children with medical complexity.

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.

Exome Sequencing and the Management of Neurometabolic Disorders.

BACKGROUND: Whole-exome sequencing has transformed gene discovery and diagnosis in rare diseases. Translation into disease-modifying treatments is challenging, particularly for intellectual developmental disorder. However, the exception is inborn errors of metabolism, since many of these disorders are responsive to therapy that targets pathophysiological features at the molecular or cellular level. METHODS: To uncover the genetic basis of potentially treatable inborn errors of metabolism, we combined deep clinical phenotyping (the comprehensive characterization of the discrete components of a patient's clinical and biochemical phenotype) with whole-exome sequencing analysis through a semiautomated bioinformatics pipeline in consecutively enrolled patients with intellectual developmental disorder and unexplained metabolic phenotypes. RESULTS: We performed whole-exome sequencing on samples obtained from 47 probands. Of these patients, 6 were excluded, including 1 who withdrew from the study. The remaining 41 probands had been born to predominantly nonconsanguineous parents of European descent. In 37 probands, we identified variants in 2 genes newly implicated in disease, 9 candidate genes, 22 known genes with newly identified phenotypes, and 9 genes with expected phenotypes; in most of the genes, the variants were classified as either pathogenic or probably pathogenic. Complex phenotypes of patients in five families were explained by coexisting monogenic conditions. We obtained a diagnosis in 28 of 41 probands (68%) who were evaluated. A test of a targeted intervention was performed in 18 patients (44%). CONCLUSIONS: Deep phenotyping and whole-exome sequencing in 41 probands with intellectual developmental disorder and unexplained metabolic abnormalities led to a diagnosis in 68%, the identification of 11 candidate genes newly implicated in neurometabolic disease, and a change in treatment beyond genetic counseling in 44%. (Funded by BC Children's Hospital Foundation and others.).

Antireflux Procedures in Children With Neurologic Impairment: A National Survey of Physician Perspectives.

OBJECTIVE: Decision-making about antireflux procedures (ARPs) to treat gastroesophageal reflux disease in children with neurologic impairment and gastrostomy tubes is challenging and likely influenced by physicians' experience and perspectives. This study will explore physician attitudes about ARPs and determine if there are relationships to clinical practice and personal characteristics. METHODS: This is a national observational cross-sectional study that used an electronic questionnaire addressing reported practice, attitudes regarding the ARPs, and responses to clinical vignettes. Participants were physicians in Canadian tertiary-care pediatric settings. Descriptive statistics were used to analyze physician attitudes. Multivariable logistic regression modeling was used to determine associations between physician and practice characteristics and likelihood to consider ARP. RESULTS: Eighty three respondents represented 12 institutions, with a majority from general or complex care pediatrics. There was a wide disparity between likelihood to consider ARP in each clinical scenario. Likelihood to consider ARP ranged from to 19% to 78% depending on the scenario. Two scenarios were equally split in whether the respondent would offer an ARP. None of the demographic characteristics were significantly associated with likelihood to consider ARP. Often, gastrojejunostomy tubes alone were considered (56% to 68%). CONCLUSIONS: There is considerable variability in physician attitudes toward and recommendations regarding ARPs to treat gastroesophageal reflux disease. We did not find a significant association with clinical experience or location of practice. More research is needed to define indications and outcomes for ARPs. This is a scenario where shared decision-making, bringing together physician and family knowledge and expertise, is likely the best course of action.

Trust of inpatient physicians among parents of children with medical complexity: a qualitative study.

Background: Trust is a foundation of the therapeutic relationship and is associated with important patient outcomes. Building trust between parents of children with medical complexity (CMC) and physicians during inpatient care is complicated by lack of relational continuity, cumulative (sometimes negative) parent experiences and the need to adjust roles and expectations to accommodate parental expertise. This study's objective was to describe how parents of CMC conceptualize trust with physicians within the pediatric inpatient setting and to provide recommendations for building trust in these relationships. Methods: Interviews with 16 parents of CMC were completed and analyzed using interpretive description methodology. Results: The research team identified one overarching meta theme regarding factors that influence trust development: situational awareness is needed to inform personalized care of children and families. There were also six major themes: (1) ensuring that the focus is on the child and family, (2) respecting both parent and physician expertise, (3) collaborating effectively, (4) maintaining a flow of communication, (5) acknowledging the impact of personal attributes, and (6) recognizing issues related to the healthcare system. Discussion: Many elements that facilitated trust development were also components of patient- and family-centered care. Parents in this study approached trust with inpatient physicians as something that needs to be earned and reciprocated. To gain the trust of parents of CMC, inpatient physicians should personalize medical care to address the needs of each child and should explore the perceptions, expertise, and previous experiences of their parents.

Learning to Trust Yourself: Decision-Making Skills Among Parents of Children With Medical Complexity.

CONTEXT: Children with medical complexity have substantial medical needs and their caregivers must make many challenging decisions about their care. Caregivers often become more involved in decisions over time, but it is unclear what skills they develop that facilitate this engagement. OBJECTIVES: To describe the skills that caregivers developed as they gained experience making medical decisions. METHODS: Eligible caregivers had a child who met referral criteria for their centre's Complex Care program for >1 year, were adults responsible for their child's medical decisions, and spoke English or a language with an available interpreter. We followed a semistructured interview guide to ask caregivers to describe and reflect on two challenging medical decisions that they made for their child-one early and one recent. Guided by interpretive description, we identified and refined themes in an iterative process. RESULTS: We conducted 15 interviews with 16 parents (14 [88%] women, two [13%] men) of a child with medical complexity (aged 1-17 years). Parents described 1) becoming more adept at managing decisional information, 2) recognizing the influence of the decision's context, 3) building stronger relationships with providers, and 4) becoming more effective at guiding their child's care as a decision-maker. As parents built these skills, they developed a greater sense of agency and confidence as decision-makers. CONCLUSION: Parents of children with medical complexity change how they approach decision making over time as they acquire relevant skills. These findings can inform the development of interventions to support skill-building among new caregivers.

A systematic review of self and observer assessment of pain and related functioning in youth with brain-based developmental disabilities.

ABSTRACT: Pain experiences of youth with brain-based developmental disabilities are often overlooked and/or misinterpreted, increasing the risk for poor or inadequate pain assessment and management. Ample measures exist to assess acute and chronic pain, yet their utility and frequency of use in youth with brain-based developmental disabilities is unclear and available measures do not have strong measurement properties for this diverse group. This systematic review identified the scope of self-reported and observer-reported pain assessment in studies of youth (aged 3-24 years) with brain-based developmental disabilities (phase 1) and summarized other measures of pain-related functioning for acute and chronic pain (ie, physical, emotional, social, sleep, and quality of life, within the subset of quantitative studies focused primarily on pain, phase 2). A comprehensive search for English-language studies was conducted in August 2022 in Web of Science, CINAHL, MEDLINE, Cochrane CENTRAL, EMBASE, and APA PsychINFO (PROSPERO registration: CRD42021237444). A total of 17,029 unique records were screened. Of the 707 articles included in phase 1, most assessed chronic pain (n = 314; 62.0%) and primarily used observer-report (n = 155; 31%) over self-report (n = 67; 13%). Of the 137 articles included in phase 2, other outcomes assessed alongside pain intensity included motor ability (16.8%), adaptive functioning (11%), quality of life (8%), pain interference (6.6%), mental health (5.8%), and communication ability (2.9%). Cerebral palsy was the most common population in both phase 1 (n = 343; 48.5%) and phase 2 (n = 83; 59.7%). This review provides a foundational understanding of pain assessment in brain-based developmental disabilities and highlights continued inequities in holistic pain assessment for this population.

Needs of children with neurodevelopmental disorders and medical complexity: Caregiver perspectives.

BACKGROUND: Caregivers of children with neurodevelopmental disorders and medical complexities (NDD-MC) coordinate care across complex multisectoral systems. Often NDD-MC children have complex behaviors, however there is limited information on coordination needs for families. AIM: The objective of this mixed methods study was to understand the needs of families with NDD-MC children. METHODS AND PROCEDURES:  This sequential exploratory study obtained the perspectives of 67 caregivers of NDD-MC children using semi-structured interviews and validated questionnaires to measure family quality of life, care integration, and resource use. An adapted model using Maslow's hierarchy of needs was integrated, to understand the impacts of caregiving on quality of life. OUTCOMES AND RESULTS: Lack of support in caring for NDD-MC children negatively impacted family quality of life, resulting in various unmet needs, including caregiver burden, behavioural challenges, financial losses, and mental health issues. Caregivers dealt with a fragmented healthcare system that offered limited support to address coordination challenges. CONCLUSIONS AND IMPLICATIONS: The implementation of a needs-based care coordination program is recommended to consider the health, educational, and social needs of NDD-MC children and their families. Effective care for children with complex needs should tailor support for families using Maslow's hierarchy to enhance quality of life.

Children with medical complexity in Canada.

The burden of chronic disease is placing pressure on the Canadian health care system. A small but important chronic disease population is children with medical complexity, defined as individuals with: high family-identified needs; complex chronic disease necessitating specialized care; functional disability; and high health care utilization. These patients present a challenge to community providers who are expected to provide holistic care and manage complex issues, often with a paucity of services and supports. Alternative models of care may address the complex needs of this population. In addition, strategies can be implemented in community practices that may assist with the care of children with medical complexity such as collaborative care, engagement of key workers, focus on goal-directed care and use of care plans. The paediatric community should engage in health care reform discussions focused on chronic disease to ensure that the complex needs of these children are met.

Le fardeau des maladies chroniques accable le système de santé canadien. Les enfants présentant des complexités médicales constituent une population, petite mais importante, définie comme composée de personnes dont la famille a des besoins considérables, dont la maladie chronique complexe exige des soins spécialisés, qui souffrent d'une incapacité fonctionnelle et qui utilisent beaucoup les soins de santé. Ces patients représentent un défi pour les dispensateurs de soins communautaires, qui doivent fournir des soins globaux et prendre en charge des problèmes complexes, souvent malgré un manque de services et de soutien. D'autres modèles de soins peuvent répondre aux besoins complexes de cette population. En outre, des stratégies peuvent être mises en œuvre dans les pratiques communautaires afin de contribuer aux soins des enfants présentant des complexités médicales, telles que les soins coopératifs, la participation de travailleurs clés, une orientation vers des soins axés sur des objectifs et l'utilisation de plans de soins. Le milieu de la pédiatrie devrait entamer des discussions en vue d'une réforme de la santé qui répondra aux besoins complexes de ces enfants.

Measure what matters: considerations for outcome measurement of care coordination for children with neurodevelopmental disabilities and medical complexity.

Introduction: Care Coordination (CC) is a significant intervention to enhance family's capacity in caring for children with neurodevelopmental disability and medical complexity (NDD-MC). CC assists with integration of medical and behavioral care and services, partnerships with medical and community-based supports, and access to medical, behavioral, and educational supports and services. Although there is some consensus on the principles that characterize optimal CC for children with NDD-MC, challenges remain in measuring and quantifying the impacts of CC related to these principles. Two key challenges include: (1) identification of measures that capture CC impacts from the medical system, care provider, and family perspectives; and (2) recognition of the important community context outside of a hospital or clinical setting. Methods: This study used a multilevel model variant of the triangulation mixed methods design to assess the impact of a CC project implemented in Alberta, Canada, on family quality of life, resource use, and care integration at the broader environmental and household levels. At the broader environmental level, we used linked administrative data. At the household level we used quantitative pre-post survey datasets, and aggregate findings from qualitative interviews to measure group-level impacts and an embedded multiple-case design to draw comparisons, capture the nuances of children with NDD-MC and their families, and expand on factors driving the high variability in outcome measures. Three theoretical propositions formed the basis of the analytical strategy for our case study evidence to explore factors affecting the high variability in outcome measures. Discussion: This study expanded on the factors used to measure the outcomes of CC and adds to our understanding of how CC as an intervention impacts resource use, quality of life, and care integration of children with NDD-MC and their families. Given the heterogeneous nature of this population, evaluation studies that account for the variable and multi-level impacts of CC interventions are critical to inform practice, implementation, and policy of CC for children with NDD-MC.

Gastro-oesophageal reflux disease in children with neurological impairment: a retrospective cohort study.

OBJECTIVES: To determine the incidence and prevalence of gastro-oesophageal reflux disease (GERD) diagnosis and treatment in children with neurological impairment (NI) along with relationship to key variables. DESIGN: This is a population-based retrospective cohort study. SETTING: This study takes place in Alberta, Canada. PATIENTS: Children with NI were identified by hospital-based International Classification of Diseases (ICD) codes from 2006 to 2018. MAIN OUTCOME MEASURES: Incidence and prevalence of a GERD diagnosis identified by: (1) hospital-based ICD-10 codes; (2) specialist claims; (3) dispensation of acid-suppressing medication (ASM). Age, gender, complex chronic conditions (CCC) and technology assistance were covariates. RESULTS: Among 10 309 children with NI, 2772 (26.9%) met the GERD definition. The unadjusted incidence rate was 52.1 per 1000 person-years (50.2-54.1). Increasing numbers of CCCs were associated with a higher risk of GERD. The HR for GERD associated with a gastrostomy tube was 4.56 (95% CI 4.15 to 5.00). Overall, 2486 (24.1%) of the children were treated with ASMs of which 1535 (61.7%) met no other GERD criteria. The incidence rate was 16.9 dispensations per year (95% CI 16.73 to 17.07). The prevalence of gastrojejunostomy tubes was 1.1% (n=121), surgical jejunostomy tubes was 0.7% (n=79) and fundoplication was 3.4% (n=351). CONCLUSIONS: The incidence of GERD in children with NI greatly exceeds that of the general paediatric population. Similarly, incidence rate of medication dispensations was closer to the rates seen in adults particularly in children with multiple CCCs and gastrostomy tubes. Further research is needed to determine the appropriate use of ASMs balancing the potential for adverse effects in this population.

Risk factors for severe PCR-positive SARS-CoV-2 infection in hospitalised children.

OBJECTIVE: To identify risk factors for severe disease in children hospitalised for SARS-CoV-2 infection. DESIGN: Multicentre retrospective cohort study. SETTING: 18 hospitals in Canada, Iran and Costa Rica from 1 February 2020 to 31 May 2021. PATIENTS: Children<18 years of age hospitalised for symptomatic PCR-positive SARS-CoV-2 infection, including PCR-positive multisystem inflammatory syndrome in children (MIS-C). MAIN OUTCOME MEASURE: Severity on the WHO COVID-19 Clinical Progression Scale was used for ordinal logistic regression analyses. RESULTS: We identified 403 hospitalisations. Median age was 3.78 years (IQR 0.53-10.77). At least one comorbidity was present in 46.4% (187/403) and multiple comorbidities in 18.6% (75/403). Eighty-one children (20.1%) met WHO criteria for PCR-positive MIS-C. Progression to WHO clinical scale score ≥6 occurred in 25.3% (102/403). In multivariable ordinal logistic regression analyses adjusted for age, chest imaging findings, laboratory-confirmed bacterial and/or viral coinfection, and MIS-C diagnosis, presence of a single (adjusted OR (aOR) 1.90, 95% CI 1.13 to 3.20) or multiple chronic comorbidities (aOR 2.12, 95% CI 1.19 to 3.79), obesity (aOR 3.42, 95% CI 1.76 to 6.66) and chromosomal disorders (aOR 4.47, 95% CI 1.25 to 16.01) were independent risk factors for severity. Age was not an independent risk factor, but different age-specific comorbidities were associated with more severe disease in age-stratified adjusted analyses: cardiac (aOR 2.90, 95% CI 1.11 to 7.56) and non-asthma pulmonary disorders (aOR 3.07, 95% CI 1.26 to 7.49) in children<12 years old and obesity (aOR 3.69, 1.45-9.40) in adolescents≥12 years old. Among infants<1 year old, neurological (aOR 10.72, 95% CI 1.01 to 113.35) and cardiac disorders (aOR 10.13, 95% CI 1.69 to 60.54) were independent predictors of severe disease. CONCLUSION: We identified risk factors for disease severity among children hospitalised for PCR-positive SARS-CoV-2 infection. Comorbidities predisposing children to more severe disease may vary by age. These findings can potentially guide vaccination programmes and treatment approaches in children.

Rehabilitation therapies in Rett syndrome across the lifespan: A scoping review of human and animal studies.

PURPOSE: To perform a scoping review of the evidence for therapeutic interventions to manage functional impairments associated with Rett syndrome (RTT) throughout the lifespan. METHODS: MEDLINE, EMBASE, PsycINFO, CENTRAL, CINAHL, Scopus and Index to Chiropractic Literature were searched systematically up to December 2019. Two investigators independently reviewed all search results and extracted those that met the inclusion criteria. Human and animal model studies pertaining to therapies that increase functional ability or treat RTT-associated symptoms in all age groups were included. Relevant studies were grouped into intervention categories and rated using the Oxford Centre of Evidence Based Medicine Levels of Evidence. Demographics of participants, interventions, and outcomes were summarized. RESULTS: Ninety-one articles representing 88 studies met the inclusion criteria, of which 80 were human clinical studies and eight were studies using animal models. Study designs were primarily case series and only six studies involved participants above the age of 40. CONCLUSION: A small number of rigorously studied rehabilitation interventions have been published. Published studies aim to address a wide variety of functional impairments. Research regarding implementation of therapies for older patients with RTT is lacking and requires further exploration.