Lung Volume Recruitment and Quality of Life in Duchenne Muscular Dystrophy: Secondary Analysis of the STEADFAST Randomized Controlled Trial.

Lung volume recruitment (LVR) is prescribed for children with Duchenne muscular dystrophy (DMD) to maintain chest wall compliance and assist airway clearance. We aimed to determine if twice-daily LVR compared with standard treatment in children with DMD improved health-related quality of life (HRQOL) over 2 years. A multicenter, assessor-blinded randomized controlled trial was conducted among boys aged 6-16 years with DMD with forced vital capacity >30% predicted to undergo conventional treatment or conventional treatment and manual LVR twice daily for 2 years.

A mixed-methods exploration of the Real-Time Virtual Support pathway Child Health Advice in Real-Time Electronically in Northwestern BC.

Objectives: To explore the implementation of a provincial virtual paediatric consulting service, Child Health Advice in Real-Time Electronically (CHARLiE), integrated into the paediatric on-call schedule in Northwestern British Columbia.

Methods: Healthcare providers in Northwestern British Columbia responded to a survey (n = 72) and participated in focus groups (n = 35) and key informant interviews (n = 4) to share their experiences engaging in a healthcare model that incorporated virtual paediatric consultants in lieu of in-person local paediatrician coverage over a 28-month period. Survey data was analyzed using descriptive statistics.

Understanding patient characteristics and medication prescriptions in children with mental health and neurodevelopmental disorders referred to a sleep clinic-A quality improvement/quality assurance analysis.

Introduction: Motivated by challenges faced in outpatient sleep services for mental health and neurodevelopmental disorders (MHNDD) during the COVID-19 clinical shutdown, a pan-Canadian/international working group of clinicians and social scientists developed a concept for capturing challenging sleep and wake behaviours already at the referral stage in the community setting.

Methods: In a quality improvement/quality assurance (QIQA) project, a visual logic model was the framework for identifying the multiple causes and possible interventions for sleep disturbances. Intake forms informed clinicians about situational experiences, goals/concerns, in addition to the questions from the Sleep Disturbances Scale for Children (SDSC), the ADHD Rating Scale-IV and medication history.

Chronic tracheostomy care of ventilator-dependent and -independent children: Clinical practice patterns of pediatric respirologists in a publicly funded (Canadian) healthcare system.

Objectives: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation.

Methods: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up.

Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial.

Background: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care guidelines but is not well studied.

Death and Dying in Hospitalized Pediatric Patients: A Prospective Multicenter, Multinational Study.

For hospitalized children admitted outside of a critical care unit, the location, mode of death, "do-not-resuscitate" order (DNR) use, and involvement of palliative care teams have not been described across high-income countries. To describe location of death, patient and terminal care plan characteristics of pediatric inpatient deaths inside and outside the pediatric intensive care unit (PICU). Secondary analysis of inpatient deaths in the Evaluating Processes of Care and Outcomes of Children in Hospital (EPOCH) randomized controlled trial.

Socioeconomic inequalities in pediatric obstructive sleep apnea.

Study Objectives: To examine the association between multiple measures of socioeconomic status (SES) and objectively measured obstructive sleep apnea (OSA) in a Canadian pediatric population.

Methods: This was a cross-sectional study of 188 children (4-17 years, mean age 9.3 ± 3.

Trametinib therapy for children with neurofibromatosis type 1 and life-threatening plexiform neurofibroma or treatment-refractory low-grade glioma.

Purpose: To describe a series of children with extensive PNF or treatment refractory PLGG treated on a compassionate basis with trametinib.

Methods: We report on six patients with NF-1 treated with trametinib on a compassionate basis at British Columbia Children's Hospital since 2017. Data were collected retrospectively from the patient record.

Implementation study of a 5-component pediatric early warning system (PEWS) in an emergency department in British Columbia, Canada, to inform provincial scale up.

Background: The rapid identification of deterioration in the pediatric population is complex, particularly in the emergency department (ED). A comprehensive multi-faceted Pediatric Early Warning System (PEWS) might maximize early recognition of clinical deterioration and provide a structured process for the reassessment and escalation of care. The objective of the study was to evaluate the implementation fidelity, effectiveness, and utility of a 5-component PEWS implemented in the ED of an urban public general hospital in British Columbia, Canada, and to guide provincial scale up.

Pediatric pulse oximetry-based OSA screening at different thresholds of the apnea-hypopnea index with an expression of uncertainty for inconclusive classifications.

Background: Assessments of pediatric obstructive sleep apnea (OSA) are underutilized across Canada due to a lack of resources. Polysomnography (PSG) measures OSA severity through the average number of apnea/hypopnea events per hour (AHI), but is resource intensive and requires a specialized sleep laboratory, which results in long waitlists and delays in OSA detection. Prompt diagnosis and treatment of OSA are crucial for children, as untreated OSA is linked to behavioral deficits, growth failure, and negative cardiovascular consequences.

Clinician-Driven Design of -An Intelligent Monitoring and Communication Device to Improve Patient Safety in the Intensive Care Unit.

The pediatric intensive care unit (ICU) is a complex environment, in which a multidisciplinary team of clinicians (registered nurses, respiratory therapists, and physicians) continually observe and evaluate patient information. Data are provided by multiple, and often physically separated sources, cognitive workload is high, and team communication can be challenging. Our aim is to combine information from multiple monitoring and therapeutic devices in a mobile application, the , to improve the efficiency of clinical decision-making, communication, and thereby patient safety.

Effect of a Pediatric Early Warning System on All-Cause Mortality in Hospitalized Pediatric Patients: The EPOCH Randomized Clinical Trial.

Importance: There is limited evidence that the use of severity of illness scores in pediatric patients can facilitate timely admission to the intensive care unit or improve patient outcomes.

Objective: To determine the effect of the Bedside Paediatric Early Warning System (BedsidePEWS) on all-cause hospital mortality and late admission to the intensive care unit (ICU), cardiac arrest, and ICU resource use.

Design, Setting, And Participants: A multicenter cluster randomized trial of 21 hospitals located in 7 countries (Belgium, Canada, England, Ireland, Italy, New Zealand, and the Netherlands) that provided inpatient pediatric care for infants (gestational age ≥37 weeks) to teenagers (aged ≤18 years).

Comparison of Consent Models in a Randomized Trial of Corticosteroids in Pediatric Septic Shock.

Objectives: To describe the use of deferred and prior informed consent models in the context of a low additional risk to standard of care, placebo-controlled randomized controlled trial of corticosteroids in pediatric septic shock.

Design: An observational substudy of consent processes in a randomized controlled trial of hydrocortisone versus placebo.

Setting: Seven tertiary level PICUs in Canada.

A Randomized Controlled Trial of Corticosteroids in Pediatric Septic Shock: A Pilot Feasibility Study.

Objective: To determine the feasibility of conducting a randomized controlled trial of corticosteroids in pediatric septic shock.

Design: Randomized, double-blind, placebo controlled trial.

Setting: Seven tertiary level PICUs in Canada.

Phenotypic expansion of TBX4 mutations to include acinar dysplasia of the lungs.

Mutations in the T-box transcription factor TBX4 gene have been reported in patients with Ischiocoxopodopatellar syndrome (MIM# 147891) and childhood-onset pulmonary arterial hypertension. Whole exome sequencing of DNA from a 1 day old deceased newborn, with severe diffuse developmental lung disorder exhibiting features of acinar dysplasia, and her unaffected parents identified a de novo TBX4 missense mutation p.E86Q (c.

Steroids in fluid and/or vasoactive infusion dependent pediatric shock: study protocol for a randomized controlled trial.

Background: Physicians often administer corticosteroids for the treatment of fluid and vasoactive infusion dependent pediatric shock. This use of corticosteroids is controversial, however, and has never been studied in a pediatric randomized controlled trial (RCT). This pilot trial will determine the feasibility of a larger RCT on the role of corticosteroids in pediatric shock.

The quality of life of home-ventilated children and their primary caregivers plus the associated social and economic burdens: a prospective study.

Background: Despite the obvious challenges faced by families caring for children on home ventilation, there is surprisingly little research into the details of their daily lives. In particular, little is known about the quality of life of the child and caregiver plus the associated social and economic burdens of care.

Methods: We prospectively studied 90 families enrolled in a paediatric home ventilation service in British Columbia.

Pulse oximetry recorded from the Phone Oximeter for detection of obstructive sleep apnea events with and without oxygen desaturation in children.

Obstructive sleep apnea (OSA) disrupts normal ventilation during sleep and can lead to serious health problems in children if left untreated. Polysomnography, the gold standard for OSA diagnosis, is resource intensive and requires a specialized laboratory. Thus, we proposed to use the Phone Oximeter™, a portable device integrating pulse oximetry with a smartphone, to detect OSA events.

Evaluation of cardiac modulation in children in response to apnea/hypopnea using the Phone Oximeter(™).

Individuals with sleep disordered breathing (SDB) can experience changes in automatic cardiac regulation as a result of frequent sleep fragmentation and disturbance in normal respiration and oxygenation that accompany most apnea/hypopnea events. In adults, these changes are reflected in enhanced sympathetic and reduced parasympathetic activity. In this study, we examined the autonomic cardiac regulation in children with and without SDB, through spectral and detrended fluctuation analysis (DFA) of pulse rate variability (PRV).

Evaluating processes of care and outcomes of children in hospital (EPOCH): study protocol for a randomized controlled trial.

Background: The prevention of near and actual cardiopulmonary arrest in hospitalized children is a patient safety imperative. Prevention is contingent upon the timely identification, referral and treatment of children who are deteriorating clinically. We designed and validated a documentation-based system of care to permit identification and referral as well as facilitate provision of timely treatment.