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. We aimed to determine whether twice-daily LVR, compared with standard of care alone, attenuates the decline in FVC at 2 years in boys with DMD. METHODS: In this multicentre, assessor-blinded, randomised controlled trial, boys with DMD, aged 6-16 years with FVC >30% predicted, were randomised to receive conventional treatment or conventional treatment plus manual LVR twice daily for 2 years. The primary outcome was FVC % predicted at 2 years, adjusted for baseline FVC % predicted, age and ambulatory status. Secondary outcomes included change in chest wall distensibility (maximal insufflation capacity minus FVC) and peak cough flow. RESULTS: Sixty-six boys (36 in LVR group, 30 in control) were evaluated (median age (IQR): 11.5 years (9.5-13.5), median baseline FVC (IQR): 85% predicted (73-96)). Adjusted mean difference in FVC between groups at 2 years was 1.9% predicted (95% CI -6.9% to 10.7%; p=0.68) in the direction of treatment benefit. We found no differences in secondary outcomes. CONCLUSION: There was no difference in decline in FVC % predicted with use of twice-daily LVR for boys with DMD and relatively normal lung function. The burden associated with routine LVR may outweigh the benefit. Benefits of LVR to maintain lung health in boys with worse baseline lung function still need to be clarified. TRIAL REGISTRATION NUMBER: NCT01999075.

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. METHODS: We used a before-and-after design to evaluate the implementation fidelity, effectiveness, and utility of a 5-component PEWS (pediatric assessment flowsheet, PEWS score, situational awareness, escalation aid, and communication framework). Sources of data included patient medical records, surveys of direct care staff, and key-informant interviews. Data were analyzed using mixed-methods approaches. RESULTS: The majority of medical records had documented PEWS scores at triage (80%) and first bedside assessment (81%), indicating that the intervention was implemented with high fidelity. The intervention was effective in increasing vital signs documentation, both at first beside assessment (84% increase) and throughout the ED stay (> 100% increase), in improving staff's self-perceived knowledge and confidence in providing pediatric care, and self-reported communication between staff. Satisfaction levels were high with the PEWS scoring system, flowsheet, escalation aid, and to a lesser extent with the situational awareness tool and communication framework. Reasons for dissatisfaction included increased paperwork and incidence of false-positives. Overall, the majority of providers indicated that implementation of PEWS and completing a PEWS score at triage alongside the Canadian Triage and Acuity Scale (CTAS) added value to pediatric care in the ED. Results also suggest that the intervention is aligned with current practice in the ED. CONCLUSION: Our study shows that high-fidelity implementation of PEWS in the ED is feasible. We also show that a multi-component PEWS can be effective in improving pediatric care and be well-accepted by staff. Results and lessons learned from this pilot study are being used to scale up implementation of PEWS in ED settings across the province of British Columbia.

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). Participating hospitals had continuous physician staffing and subspecialized pediatric services. Patient enrollment began on February 28, 2011, and ended on June 21, 2015. Follow-up ended on July 19, 2015. Interventions: The BedsidePEWS intervention (10 hospitals) was compared with usual care (no severity of illness score; 11 hospitals). Main Outcomes and Measures: The primary outcome was all-cause hospital mortality. The secondary outcome was a significant clinical deterioration event, which was defined as a composite outcome reflecting late ICU admission. Regression analyses accounted for hospital-level clustering and baseline rates. Results: Among 144 539 patient discharges at 21 randomized hospitals, there were 559 443 patient-days and 144 539 patients (100%) completed the trial. All-cause hospital mortality was 1.93 per 1000 patient discharges at hospitals with BedsidePEWS and 1.56 per 1000 patient discharges at hospitals with usual care (adjusted between-group rate difference, 0.01 [95% CI, -0.80 to 0.81 per 1000 patient discharges]; adjusted odds ratio, 1.01 [95% CI, 0.61 to 1.69]; P = .96). Significant clinical deterioration events occurred during 0.50 per 1000 patient-days at hospitals with BedsidePEWS vs 0.84 per 1000 patient-days at hospitals with usual care (adjusted between-group rate difference, -0.34 [95% CI, -0.73 to 0.05 per 1000 patient-days]; adjusted rate ratio, 0.77 [95% CI, 0.61 to 0.97]; P = .03). Conclusions and Relevance: Implementation of the Bedside Paediatric Early Warning System compared with usual care did not significantly decrease all-cause mortality among hospitalized pediatric patients. These findings do not support the use of this system to reduce mortality. Trial Registration: clinicaltrials.gov Identifier: NCT01260831.

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. PATIENTS: Children newborn to 17 years inclusive admitted to PICU with suspected septic shock between July 2014 and March 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Information on the number of families approached, consent rates obtained, and spontaneously volunteered reasons for nonparticipation were collected for both deferred and informed consent. The research ethics board of five of seven centers approved a deferred consent model; however, implementation criteria for use of this model varied across sites. The consent rate using deferred versus prior informed consent was significantly higher (83%; 35/42 vs 58%; 15/26; p = 0.02). The mean times from meeting inclusion criteria to randomization (1.8 ± 1.8 vs 3.6 ± 2.1 hr; p = 0.007) and study drug administration (3.4 ± 2.7 hr vs 4.8 ± 2.1 hr; p = 0.05) were significantly shorter with the use of deferred consent versus prior informed consent. No family member or research ethics board expressed concern following use of deferred consent. CONCLUSIONS: Deferred consent was acceptable in time-sensitive critical care research to most research ethics boards, families, and healthcare providers and resulted in higher consent rates and more efficient recruitment. Larger studies on deferred consent and consistency interpreting jurisdictional guidelines are needed to advance pediatric acute care.

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. PATIENTS: Children newborn to 17 years old inclusive with suspected septic shock. INTERVENTION: Administration of IV hydrocortisone versus placebo until hemodynamic stability is achieved or for a maximum of 7 days. MEASUREMENTS AND MAIN RESULTS: One hundred seventy-four patients were potentially eligible of whom 101 patients met eligibility criteria. Fifty-seven patients were randomized, and 49 patients (23 and 26 patients in the hydrocortisone and placebo groups, respectively) were included in the final analysis. The mean time from screening to randomization was 2.4 ± 2.1 hours and from screening to first dose of study drug was 3.8 ± 2.6 hours. Forty-two percent of potentially eligible patients (73/174) received corticosteroids prior to randomization: 38.5% (67/174) were already on corticosteroids for shock at the time of screening, and in 3.4% (6/174), the treating physician wished to administer corticosteroids. Six of 49 randomized patients (12.2%) received open-label steroids, three in each of the hydrocortisone and placebo groups. Time on vasopressors, days on mechanical ventilation, PICU and hospital length of stay, and the rate of adverse events were not statistically different between the two groups. CONCLUSIONS: This study suggests that a large randomized controlled trial on early use of corticosteroids in pediatric septic shock is potentially feasible. However, the frequent use of empiric corticosteroids in otherwise eligible patients remains a significant challenge. Knowledge translation activities, targeted recruitment, and alternative study designs are possible strategies to mitigate this challenge.

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.256G>C) in the DNA-binding T-box domain. We propose phenotypic expansion of the TBX4-related clinical disease spectrum to include acinar dysplasia of the lungs. The reported mutation is the first identified genetic variant causative for acinar dysplasia. © 2016 Wiley Periodicals, Inc.

Orthodontic and Facial Characteristics of Craniofacial Syndromic Children with Obstructive Sleep Apnea.

Introduction: Obstructive sleep apnea (OSA) is a disorder in which ventilation becomes disrupted due to a complete or partial upper airway obstruction Altered craniofacial morphology is one of the most important anatomical factors associated with obstructive sleep apnea (OSA). Studies have assessed craniofacial features in the non-syndromic pediatric population. The aim of this study was to analyze the orthodontic and facial characteristic of craniofacial syndromic children referred for polysomnography (PSG) and to assess the correlation with the apnea-hypopnea index (AHI). Methods: In the current cross-sectional study, consecutive syndromic patients referred for PSG were invited to participate. A systematic clinical examination including extra- and intra-oral orthodontic examination was performed by calibrated orthodontists. Standardized frontal and profile photographs with reference points were taken and analyzed using ImageJ® software to study the craniofacial morphology. PSG data were analyzed for correlation with craniofacial features. STROBE guidelines were strictly adopted during the research presentation. Results: The sample included 52 syndromic patients (50% females, mean age 9.38 ± 3.36 years) diagnosed with 17 different syndromes, of which 24 patients had craniofacial photography analysis carried out. Most of the sample (40%) had severe OSA, while only 5.8% had no OSA. Down's syndrome (DS) was the most common syndrome (40%) followed by Goldenhar syndrome (5%), Pierre Robin Sequence (5%), and other syndromes. The severity of AHI was significantly correlated with decreased midfacial height. increased thyromental angle and cervicomental angle, decreased mandibular angle, and decreased upper facial height. All patients with DS were diagnosed with OSA (57% severe OSA), and their ODI was significantly correlated with increased intercanthal distance. Obesity was not correlated to the severity of AHI for syndromic patients. Conclusions: Decreased midfacial height and obtuse thyromental angle were correlated with increased AHI for syndromic patients. Increased intercanthal distance of DS patients could be a major predictor of OSA severity. Obesity does not seem to play a major role in the severity of OSA for syndromic patients. Further studies with larger samples are necessary to confirm these findings.

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. RESULTS: The response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re-use, and cleaning practices. Most centers require two trained caregivers (14/17) and recommend 24/7 eyes on care and oxygen saturation monitoring. Discharge with an emergency tracheostomy kit was universal (17/17). Considerable heterogeneity was observed in the timing and use of speaking valves and speech-language assessment. Inhaled anti-pseudomonal antibiotics are employed by most centers (16/17) though the indication, agent, and protocol varied by center. Though decannulation practices varied considerably, the requirement of upper airway patency was universally required to proceed with decannulation (17/17) independent of ongoing ventilatory support requirements. CONCLUSION: Considerable variability in pediatric tracheostomy tube care practice exists across Canada. These results will serve as a starting point to standardize and evaluate tracheostomy tube care nationally.

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. Descriptive statistics were used to describe the sample. Results: 66% of the pilot study patients (n = 41) scored in the SDSC red domains (highest scoring) with highest sub-scores for insomnia (falling asleep 73%; staying asleep: 51%) and daytime somnolence (27%). A total of 90% of patients were taking at least one medication; 59% sleep initiation/sleep medications, 41% in combination with further non-stimulant medications, 9% with stimulants, 27% with antidepressants and 18% with antipsychotics. Polypharmacy was observed in 62% of all patients and in 73% of the ones medicated for sleep disturbances. Qualitative information supported individualisation of assessments. Conclusion: Our intake process enabled a comprehensive understanding of patients' sleep and wake profiles prior to assessment, at the referral stage. The high prevalence of insomnia in patients, combined with polypharmacy, requires special attention in the triaging process at the community level.

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 ± 3.5 years) prospectively recruited from two hospital sleep clinics in Canada, using multivariable-adjusted linear and logistic regression of five measures of SES including parental education, income, social class, geographic location, and perceived SES based on the MacArthur Scale of Subjective Social Status, assessed in relation to four polysomnographic OSA variables including apnea-hypopnea index, apnea index, mean oxygen saturation level, and oxygen desaturation index. RESULTS: Overall, low household-level SES appeared to be associated with both frequency (apnea index ≥ 1 events/h) and severity (apnea-hypopnea index ≥ 5 events/h) of OSA in children, with maternal education showing the most consistent and significant associations. Specifically, children with mothers reporting less than high school education had nearly three times the odds of having OSA after controlling factors including body mass index (odds ratio 2.96 [95% confidence interval, 1.05-8.37]), compared to university-educated participants. Consistent associations were also observed for geographic location with less frequency and severity of OSA among nonurban children. Perceived SES was minimally inversely associated with our outcomes. CONCLUSIONS: This cross-sectional, multicenter study demonstrated that SES factors are linked to the occurrence and severity of OSA in children. Results indicated the need to incorporate the screening of SES in the diagnostic process of pediatric OSA to provide more targeted intervention and patient-centered care. CITATION: Park JW, Hamoda MM, Almeida FR, et al. Socioeconomic inequalities in pediatric obstructive sleep apnea. J Clin Sleep Med. 2022;18(2):637-645.

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

Background: 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. Objective: To describe location of death, patient and terminal care plan characteristics of pediatric inpatient deaths inside and outside the pediatric intensive care unit (PICU). Design: Secondary analysis of inpatient deaths in the Evaluating Processes of Care and Outcomes of Children in Hospital (EPOCH) randomized controlled trial. Setting/Subjects: Twenty-one centers from Canada, Belgium, the United Kingdom, Ireland, Italy, the Netherlands, and New Zealand. Measurement: Descriptive statistics were used to compare patient and terminal care plan characteristics. A multivariable generalized estimating equation examined if palliative care consult during hospital admission was associated with location of death. Results: A total of 365 of 144,539 patients enrolled in EPOCH died; 219 (60%) died in PICU and 143 (40%) died on another inpatient unit. Compared with other inpatient wards, patients who died in PICU were less likely to be expected to die, have a DNR or palliative care consult. Hospital palliative care consultation was more common in older children and independently associated with a lower adjusted odds (95% confidence interval) of dying in PICU [0.59 (0.52-0.68)]. Conclusion: Most pediatric inpatient deaths occur in PICU where patients were less likely to have a DNR or palliative care consult. Palliative care consultation could be better integrated into end-of-life care for younger children and those dying in PICU.

Multicentre validation of the bedside paediatric early warning system score: a severity of illness score to detect evolving critical illness in hospitalised children.

INTRODUCTION: The timely provision of critical care to hospitalised patients at risk for cardiopulmonary arrest is contingent upon identification and referral by frontline providers. Current approaches require improvement. In a single-centre study, we developed the Bedside Paediatric Early Warning System (Bedside PEWS) score to identify patients at risk. The objective of this study was to validate the Bedside PEWS score in a large patient population at multiple hospitals. METHODS: We performed an international, multicentre, case-control study of children admitted to hospital inpatient units with no limitations on care. Case patients had experienced a clinical deterioration event involving either an immediate call to a resuscitation team or urgent admission to a paediatric intensive care unit. Control patients had no events. The scores ranged from 0 to 26 and were assessed in the 24 hours prior to the clinical deterioration event. Score performance was assessed using the area under the receiver operating characteristic (AUCROC) curve by comparison with the retrospective rating of nurses and the temporal progression of scores in case patients. RESULTS: A total of 2,074 patients were evaluated at 4 participating hospitals. The median (interquartile range) maximum Bedside PEWS scores for the 12 hours ending 1 hour before the clinical deterioration event were 8 (5 to 12) in case patients and 2 (1 to 4) in control patients (P < 0.0001). The AUCROC curve (95% confidence interval) was 0.87 (0.85 to 0.89). In case patients, mean scores were 5.3 at 20 to 24 hours and 8.4 at 0 to 4 hours before the event (P < 0.0001). The AUCROC curve (95% CI) of the retrospective nurse ratings was 0.83 (0.81 to 0.86). This was significantly lower than that of the Bedside PEWS score (P < 0.0001). CONCLUSIONS: The Bedside PEWS score identified children at risk for cardiopulmonary arrest. Scores were elevated and continued to increase in the 24 hours before the clinical deterioration event. Prospective clinical evaluation is needed to determine whether this score will improve the quality of care and patient outcomes.

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. RAPNO and volumetric criteria were used to evaluate the response of intracranial and extracranial lesions, respectively. RESULTS: Subjects were 21 months to 14 years old at the time of initiation of trametinib therapy and 3/6 subjects are male. Duration of therapy was 4-28 months at the time of this report. All patients had partial response or were stable on analysis. Two patients with life-threatening PNF had a partial radiographic response in tandem with significant clinical improvement and developmental catch up. One subject discontinued therapy after 6 months due to paronychia and inadequate response. The most common adverse effect (AE) was grade 1-2 paronychia or dermatitis in 5/6 patients. There were no grade 3 or 4 AEs. At the time of this report, five patients remain on therapy. CONCLUSION: Trametinib is an effective therapy for advanced PNF and refractory PLGG in patients with NF-1 and is well tolerated in children. Further data and clinical trials are required to assess tolerance, efficacy and durability of response, and length of treatment required in such patients.

Comparison of two red-cell transfusion strategies after pediatric cardiac surgery: a subgroup analysis.

OBJECTIVE: To determine the impact of a restrictive vs. a liberal transfusion strategy on new or progressive multiple organ dysfunction syndrome in children post cardiac surgery. The optimal transfusion threshold after cardiac surgery in children is unknown. DESIGN: Randomized, controlled trial. SETTING: Tertiary pediatric intensive care units. PATIENTS: Participants are a subgroup of pediatric patients post cardiac surgery from the TRIPICU (Transfusion Requirements in Pediatric Intensive Care Units) study. Exclusion criteria specific to the cardiac surgery subgroup included: age <28 days and patients remaining cyanotic. INTERVENTION: Critically ill children with a hemoglobin < or = 95 g/L within 7 days of pediatric intensive care unit admission were randomized to receive prestorage leukocyte-reduced red-cell transfusion if their hemoglobin dropped either <70 g/L (restrictive) or 95 g/L (liberal). MEASUREMENTS AND MAIN RESULTS: Postoperative cardiac patients (n = 125) from seven centers were enrolled. The restrictive (n = 63) and liberal (n = 62) groups were similar at baseline in age (mean +/- standard deviation = 31.4 +/- 38.1 mos vs. 26.4 +/- 39.1 mos), surgical procedure, severity of illness (Pediatric Risk of Mortality score = 3.4 +/- 3.2 vs. 3.2 +/- 3.2), multiple organ dysfunction syndrome (46% vs. 44%), mechanical ventilation (62% vs. 60%), and hemoglobin (83 vs. 80 g/L). Mean hemoglobin remained 21 g/L lower in the restrictive group after randomization. No significant difference was found in new or progressive multiple organ dysfunction syndrome (primary outcome) in the restrictive group vs. liberal group (12.7% vs. 6.5%; p = .36), pediatric intensive care unit length of stay (7.0 +/- 5.0 days vs. 7.4 +/- 6.4 days) or 28-day mortality (3.2% vs. 3.2%). CONCLUSION: In this subgroup analysis of cardiac surgery patients, a restrictive red-cell transfusion strategy, as compared with a liberal one, was not associated with any significant difference in new or progressive multiple organ dysfunction syndrome, but this evidence is not definitive.

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. We aim to assess the performance of a portable pediatric OSA screening tool at different AHI cut-offs using overnight smartphone-based pulse oximetry. MATERIAL AND METHODS: Following ethics approval and informed consent, children referred to British Columbia Children's Hospital for overnight PSG were recruited for two studies including 160 and 75 children, respectively. An additional smartphone-based pulse oximeter sensor was used in both studies to record overnight pulse oximetry [SpO2 and photoplethysmogram (PPG)] alongside the PSG. Features characterizing SpO2 dynamics and heart rate variability from pulse peak intervals of the PPG signal were derived from pulse oximetry recordings. Three multivariate logistic regression screening models, targeted at three different levels of OSA severity (AHI ≥ 1, 5, and 10), were developed using stepwise-selection of features using the Bayesian information criterion (BIC). The "Gray Zone" approach was also implemented for different tolerance values to allow for more precise detection of children with inconclusive classification results. RESULTS: The optimal diagnostic tolerance values defining the "Gray Zone" borders (15, 10, and 5, respectively) were selected to develop the final models to screen for children at AHI cut-offs of 1, 5, and 10. The final models evaluated through cross-validation showed good accuracy (75%, 82% and 89%), sensitivity (80%, 85% and 82%) and specificity (65%, 79% and 91%) values for detecting children with AHI ≥ 1, AHI ≥ 5 and AHI ≥ 10. The percentage of children classified as inconclusive was 28%, 38% and 16% for models detecting AHI ≥ 1, AHI ≥ 5, and AHI ≥ 10, respectively. CONCLUSIONS: The proposed pulse oximetry-based OSA screening tool at different AHI cut-offs may assist clinicians in identifying children at different OSA severity levels. Using this tool at home prior to PSG can help with optimizing the limited resources for PSG screening. Further validation with larger and more heterogeneous datasets is required before introducing in clinical practice.

Iatrogenic hyponatremia in hospitalized children: Can it be avoided?

Iatrogenic hyponatremia in hospitalized children is a common problem. It is usually caused by the administration of free water, either orally or through the prescription of hypotonic intravenous fluids. It can result in cerebral edema and death, and is most commonly reported in healthy children undergoing minor surgery. The current teachings and practical guidelines for maintenance fluid infusions are based on caloric expenditure data in healthy children that were derived and published more than 50 years ago. A re-evaluation of these data and more recent recognition that hospitalized children are vulnerable to hyponatremia, with its resulting morbidity and mortality rates, suggest that changes in paediatricians' approach to fluid administration are necessary. There is no single fluid therapy that is optimal for all hospitalized children. A thorough assessment of the type of fluid, volume of fluid and electrolyte requirements based on individual patient requirements, plus rigorous monitoring, is required in any child receiving intravenous fluids. The present article reviews how hyponatremia occurs and makes recommendations for minimizing the risk of iatrogenic hyponatremia.

Clinician-Driven Design of VitalPAD-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 VitalPAD, to improve the efficiency of clinical decision-making, communication, and thereby patient safety. We observed individual ICU clinicians, multidisciplinary rounds, and handover procedures for 54 h to identify data needs, workflow, and existing cognitive aid use and limitations. A prototype was developed using an iterative participatory design approach; usability testing, including general and task-specific feedback, was obtained from 15 clinicians. Features included map overviews of the ICU showing clinician assignment, patient status, and respiratory support; patient vital signs; a photo-documentation option for arterial blood gas results; and team communication and reminder functions. Clinicians reported the prototype to be an intuitive display of vital parameters and relevant alerts and reminders, as well as a user-friendly communication tool. Future work includes implementation of a prototype, which will be evaluated under simulation and real-world conditions, with the aim of providing ICU staff with a monitoring device that will improve their daily work, communication, and decision-making capacity. Mobile monitoring of vital signs and therapy parameters might help improve patient safety in wards with single-patient rooms and likely has applications in many acute and critical care settings.

Caregiver knowledge and skills to safely care for pediatric tracheostomy ventilation at home.

OBJECTIVE: Caregivers of children using home mechanical ventilation (HMV) via tracheostomy require appropriate knowledge and skills. Existing training curricula are locally developed and content variable. We sought to develop a competency checklist to inform initial training and subsequent assessment of knowledge and skills of family caregivers. METHODS: We used a 2-step process. Candidate items were generated by synthesis of a scoping review, existing checklists, with additional items suggested by an eight member inter-professional group representing pediatric HMV programs across Canada. Following removal of duplicate items, we conducted a three-round Delphi to gain consensus on items for the KidsVent Checklist. RESULTS: The scoping review and checklists from five HMV programs identified 18 domains and 172 items; one additional domain and 83 additional items were identified by our expert group who also classified domains as mandatory or optional. We recruited 95 clinicians representing 12 Canadian paediatric HMV programs to participate in Delphi round 1 (response rate 72%; 84%, and 100% for subsequent rounds). Importance rating of the 255 items reduced them to 246 items. In the final checklist, the 19 domains comprised 14 mandatory (189 mandatory items) and 5 optional domains (57 optional items). CONCLUSIONS: We have developed the KidsVent checklist using rigorous consensus building methods, informed by participants with diverse geographic and inter-professional representation. This checklist represents knowledge and skills required to safely care for children using tracheostomy ventilation at home. Further study is required to explore the impact of this checklist on outcomes of this growing group of technology-dependent children.

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. In the clinic, we recorded demographic information, patient acuity score and quality of life for patient and caregiver using standardised questionnaires. Parents then monitored social and financial costs of care at home over the subsequent 8 weeks. These data were collected by telephone at 1 and 2 months. RESULTS: Most children led rich active lives. Camping trips, wheelchair sports and foreign travel were the norm, not the exception. Over 90% assessed the burden of care as mild or moderate. Government support covers medical expenses and home nursing (median 32 h/week, IQR 0-62.5 h). Monthly unreimbursed family expenses were low (median $87.7, IQR $15.3-$472). Despite this, nearly 25% of primary caregivers assessed burden of care as severe and over 50% had chronic illnesses requiring daily medication (principally depression, anxiety and arthritis). Quality of life for children or caregivers did not correlate with income or education. INTERPRETATION: Home ventilation of complex children is a successful strategy but it places significant strain on the primary caregiver. Specific attention to the physical and mental health of the caregiver should be an integral part of the management of home-ventilated children. TRIAL REGISTRATION NUMBER: NCT01863992.