An algorithm for discontinuing mechanical ventilation in boys with x-linked myotubular myopathy after positive response to gene therapy: the ASPIRO experience.

X-linked myotubular myopathy (XLMTM) is a rare, life-threatening congenital myopathy. Most (80%) children with XLMTM have profound muscle weakness and hypotonia at birth resulting in severe respiratory insufficiency, the inability to sit up, stand or walk, and early mortality. At birth, 85-90% of children with XLMTM require mechanical ventilation, with more than half requiring invasive ventilator support. Historically, ventilator-dependent children with neuromuscular-derived respiratory failure of this degree and nature, static or progressive, are not expected to achieve complete independence from mechanical ventilator support. In the ASPIRO clinical trial (NCT03199469), participants receiving a single intravenous dose of an investigational gene therapy (resamirigene bilparvovec) started showing significant improvements in daily hours of ventilation support compared with controls by 24 weeks post-dosing, and 16 of 24 dosed participants achieved ventilator independence between 14 and 97 weeks after dosing. At the time, there was no precedent or published guidance for weaning chronically ventilated children with congenital neuromuscular diseases off mechanical ventilation. When the first ASPIRO participants started showing dramatically improved respiratory function, the investigators initiated efforts to safely wean them off ventilator support, in parallel with primary protocol respiratory outcome measures. A group of experts in respiratory care and physiology and management of children with XLMTM developed an algorithm to safely wean children in the ASPIRO trial off mechanical ventilation as their respiratory muscle strength increased. The algorithm developed for this trial provides recommendations for assessing weaning readiness, a stepwise approach to weaning, and monitoring of children during and after the weaning process.

Family caregivers of children transitioning hospital to home receiving nasogastric feeding: descriptive qualitative study.

BACKGROUND: Nasogastric tube (NGT) feeding is used for children unable to tolerate adequate nutrition orally to sustain growth and development. This vulnerable population is at risk of gaps in care because they often lack a medical home due to the transitional nature of the technology. This study explores perspectives and challenges of family caregivers (FCs) of children requiring NGTs transitioning from hospital to home. METHODS: Semi-structured qualitative interviews were conducted with FCs at the Hospital for Sick Children and Children's Hospital of Eastern Ontario. Research ethics approval was obtained (SK REB# 1000064641, CHEO REB# 19/133X). Written informed consent was obtained. RESULTS: Thirteen interviews revealed FCs feeling overwhelmed and uncomfortable with first communication of the NGT but learned to manage NGTs with training and virtual care support over time. Initial transition home was described as challenging due to physical, emotional, and financial strain associated with constant management of NGTs. CONCLUSIONS: Our study describes the importance of emotional support and additional time for decision making during initial communication to FCs of their child's NGT need, and access to specialized healthcare professionals after transitioning home. Future programs should focus on personalized education and psychosocial support for FCs of children with NGTs at home. IMPACT: This study delves into the challenges faced by family caregivers (FCs) of children requiring nasogastric tube (NGT) feeding when returning home from the hospital. There is a pressing need for more time for FC decision-making and emotional support during the initial communication of the need for an NGT for their child. In addition, FCs require ongoing 24/7 support including access to healthcare professionals specialized in NGT care after the initial transition home. The study highlights the need for personalized education and psychosocial supports for FCs of children with NGTs to improve their experiences at home.

Cardioneuroablation for the treatment of reflex syncope and functional bradyarrhythmias: A Scientific Statement of the European Heart Rhythm Association (EHRA) of the ESC, the Heart Rhythm Society (HRS), the Asia Pacific Heart Rhythm Society (APHRS) and the Latin American Heart Rhythm Society (LAHRS).

Cardioneuroablation has emerged as a potential alternative to cardiac pacing in selected cases with vasovagal reflex syncope, extrinsic vagally induced sinus bradycardia-arrest or atrioventricular block. The technique was first introduced decades ago, and its use has risen over the past decade. However, as with any intervention, proper patient selection and technique are a prerequisite for a safe and effective use of cardioneuroablation therapy. This document aims to review and interpret available scientific evidence and provide a summary position on the topic.

Perioperative clinical practice recommendations for pediatric tonsillectomy: a systematic review.

PURPOSE: Tonsillectomy is one of the most common surgical procedures performed in children. Since most clinical practice guidelines (CPGs) are designed to support surgical decisions, none are specifically designed for the perioperative management of children undergoing tonsillectomy. We aimed to identify and analyze the existing CPGs with recommendations for the perioperative management of children undergoing tonsillectomy by conducting a systematic review. SOURCE: We searched Embase, MEDLINE, MEDLINE ePub Ahead of Print, and CINAHL for relevant articles published from inception to 3 August 2022. The inclusion criteria were: 1) CPG of perioperative recommendations for tonsillectomy under general anesthesia in children, 2) CPG that include at least one evidence-based recommendation, 3) peer-reviewed CPG published in English after 2000. We extracted data on baseline characteristics of each CPG and general recommendations for perioperative interventions or complications. PRINCIPAL FINDINGS: Out of five eligible CPGs, AGREE II and REX confirmed that two CPGs were high quality while only one of the two was recommended for implementation without modifications. Most of the recommendations were for pain management. Acetaminophen was the only medication recommended in all five CPG. Except for the oldest CPG, the CPG all supported of the use of nonsteroidal anti-inflammatory drugs and steroids as a pain adjunct. CONCLUSIONS: Acetaminophen, nonsteroidal anti-inflammatory drugs, and steroids are recommended in the perioperative management of pediatric tonsillectomy. Future CPG should further clarify the safe use of opioids based on severity of obstructive sleep apnea and in the context of opioid-sparing techniques, such as dexmedetomidine, high-dose dexamethasone, and gabapentinoids. STUDY REGISTRATION: PROSPERO (CRD42021253374); first submitted 18 June 2021.

RéSUMé: OBJECTIF: L'amygdalectomie est l'une des interventions chirurgicales les plus courantes pratiquées chez les enfants. Étant donné que la plupart des lignes directrices de pratique clinique sont conçues pour soutenir les décisions chirurgicales, aucune n'est spécifiquement conçue pour la prise en charge périopératoire des enfants bénéficiant d'une amygdalectomie. Notre objectif était d'identifier et d'analyser les lignes directrices de pratique clinique existantes comportant des recommandations pour la prise en charge périopératoire des enfants bénéficiant d'une amygdalectomie en réalisant une revue systématique. SOURCES: Nous avons recherché des articles pertinents dans Embase, MEDLINE, MEDLINE ePub Ahead of Print et CINAHL, publiés depuis la création de ces bases de données jusqu'au 3 août 2022. Les critères d'inclusion étaient les suivants : 1) lignes directrices de pratique clinique comportant des recommandations périopératoires pour l'amygdalectomie sous anesthésie générale chez les enfants, 2) lignes directrices de pratique clinique incluant au moins une recommandation fondée sur des données probantes, et 3) lignes directrices de pratique clinique évaluées par des pairs et publiées en anglais après 2000. Nous avons extrait des données sur les caractéristiques de base de chacune des lignes directrices de pratique clinique et des recommandations générales pour les interventions périopératoires ou les complications. CONSTATATIONS PRINCIPALES: Sur les cinq lignes directrices de pratique clinique admissibles, AGREE II et REX ont confirmé que deux lignes directrices de pratique clinique étaient de haute qualité, tandis qu'une seule des deux a été recommandée pour une mise en œuvre sans modifications. La plupart des recommandations portaient sur la prise en charge de la douleur. L'acétaminophène était le seul médicament recommandé dans les cinq lignes directrices de pratique clinique. À l'exception des lignes directrices de pratique clinique les plus anciennes, les autres ont toutes soutenu l'utilisation d'agents anti-inflammatoires non stéroïdiens et de stéroïdes comme adjuvants pour la douleur. CONCLUSION: L'acétaminophène, les agents anti-inflammatoires non stéroïdiens et les stéroïdes sont recommandés pour la prise en charge périopératoire de l'amygdalectomie pédiatrique. À l'avenir, les lignes directrices de pratique clinique devraient clarifier davantage l'utilisation sécuritaire des opioïdes en fonction de la gravité de l'apnée obstructive du sommeil et dans le contexte des techniques d'épargne des opioïdes, telles que la dexmédétomidine, la dexaméthasone à forte dose et les gabapentinoïdes. ENREGISTREMENT DE L'éTUDE: PROSPERO (CRD42021253374); soumise pour la première fois le 18 juin 2021.

Evaluation of Stain Removal Efficacy and Color Stability of Three Different Dentifrices on Artificially Stained Enamel Surface-An In Vitro Study.

AIM: The aim of the present study was to assess the stain removal ability and color stability of three distinct dentifrices on artificially stained enamel surface. MATERIALS AND METHODS: This study included 75 intact, healthy premolars free of dental caries that were extracted during orthodontic therapy. The samples were allowed to dry for 6 hours after being submerged in the prepared tea solution for roughly 18 hours every day. Then this procedure was repeated for seven successive days. All samples were randomly divided into three experimental groups with 25 samples in each group. Group I: control dentifrice, group II: dentifrice containing hydrogen peroxide, group III: dentifrice containing papain and bromelain. A specially designed toothbrushing simulator was used to brush every sample in the relevant group. Using a spectrophotometer and a measurement program, color measurement was evaluated after staining process after 4 weeks and 8 weeks of teeth cleaning. Using a profilometer, the surface roughness values (Ra) were assessed. RESULTS: After 8 weeks of brushing of stained samples, the color stability was better in dentifrice containing hydrogen peroxide (1.14 ± 0.11) followed by dentifrice containing papain and bromelain (1.22 ± 0.08) and control group (1.30 ± 0.09). And after 8 weeks of brushing of stained samples, the surface roughness was more in dentifrice containing hydrogen peroxide (0.237 ± 0.02) followed by dentifrice containing papain and bromelain (0.229 ± 0.13) and control group (0.207 ± 0.05). CONCLUSION: The present study concluded that the dentifrice containing hydrogen peroxide showed a superior whitening effect on the stained enamel surface than dentifrice containing papain and bromelain and control dentifrice. CLINICAL SIGNIFICANCE: The development of various dentifrice products has been greatly aided by the increased demand for an improved esthetic appearance. Teeth's natural color and any external stains that could accumulate on the tooth surface combine to determine a tooth's color. Additionally, the use of whitening dental pastes to remove external stains has grown in favor. With the development of these whitening toothpastes, dentifrices' ability to lessen or eliminate extrinsic dental stains has increased. How to cite this article: Mishra D, Kamath DG, Alagla M, et al. Evaluation of Stain Removal Efficacy and Color Stability of Three Different Dentifrices on Artificially Stained Enamel Surface-An In Vitro Study. J Contemp Dent Pract 2024;25(1):68-71.

Non-invasive ventilation usage and adherence in children and adults with Duchenne muscular dystrophy: A multicenter analysis.

INTRODUCTION/AIMS: Non-invasive ventilation (NIV) is routinely prescribed to support the respiratory system in Duchenne muscular dystrophy (DMD) patients; however, factors improving NIV usage are unclear. We aimed to identify predictors of NIV adherence in DMD patients. METHODS: This was a multicenter retrospective analysis of DMD patients prescribed NIV and followed at (1) The Hospital for Sick Children, Canada; (2) Rady Children's Hospital San Diego, USA; and (3) University of California San Diego Health, USA, between February 2016 and October 2020. The primary and secondary outcomes were 90-day period NIV adherence and clinical and socioeconomic predictors of NIV adherence. RESULTS: We identified 59 DMD patients prescribed NIV (mean ± SD age = 20.1 ± 6.7 y). Overall, percentage of nights used, and average nightly usage, were 79.9 ± 31.1% and 7.23 ± 4.12 h, respectively. Compared with children, adults had higher percentage of nights used (92.9 ± 16.9% vs. 70.4 ± 36.9%; P < .05), and average nightly usage (9.5 ± 4.7 h vs. 5.3 ± 3.7 h; P < .05). Non-English language (P = .01), and absence of deflazacort prescription (P = .02) were significantly associated with higher percentage of nights used while Hispanic ethnicity (P = .01), low household income (P = .02), and absence of deflazacort prescription (P = .02) were significantly associated with higher nightly usage. Based on univariable analysis, older age and declining forced vital capacity were associated with increased percentage of nights used and increased average nightly usage. DISCUSSION: Certain clinical and socioeconomic determinants had a significant impact on NIV adherence in DMD patients, providing insight into those at risk for high versus low compliance with respiratory therapy.

Exploring the experience of family caregivers of children with medical complexity during COVID-19: a qualitative study.

BACKGROUND AND OBJECTIVES: Children with medical complexity have been disproportionately impacted by the COVID-19 pandemic and the associated changes in healthcare delivery. The primary objective of this study was to gain a thorough understanding of the lived experiences of family caregivers of children with medical complexity during the pandemic. METHODS: We conducted semi-structured interviews with family caregivers of children with medical complexity from a tertiary pediatric hospital. Interview questions focused on the aspects of caregiving for children with medical complexity, impact on caregiver mental and physical well-being, changes to daily life secondary to the pandemic, and experiences receiving care in the healthcare system. Interviews were conducted until thematic saturation was achieved. Interviews were audio recorded, deidentified, transcribed verbatim, coded and analyzed using content analysis. RESULTS: Twelve semi-structured interviews were conducted. The interviews revealed three major themes and several associated subthemes: (1) experiences with the healthcare system amid the pandemic (lack of access to healthcare services and increased hospital restrictions, negative clinical interactions and communication breakdowns, virtual care use); (2) common challenges during the pandemic (financial strain, balancing multiple roles, inadequate homecare nursing); and (3) the pandemic's impact on family caregiver well-being (mental toll, physical toll). CONCLUSIONS: Family caregivers of children with medical complexity experienced mental and physical burden due to the intense nature of their caregiving responsibilities that were exacerbated during the pandemic. Our results highlight key priorities for the development of effective interventions to support family caregivers and their children.

'Our time is precious': An exploration of parental feeding behaviours for boys with Duchenne muscular dystrophy.

INTRODUCTION: Due to the increased risk of obesity for boys with Duchenne muscular dystrophy (DMD), recent guidelines recommend that dietary intake is carefully managed. Parents play an important role in the development of their child's eating behaviours and patterns. However, despite what is known about the increased risk of obesity for children with DMD, little is known about parental feeding behaviours in this population. The objective of this study was to qualitatively explore the experiences of parents of children with DMD around their child's weight management and understand what influences their feeding behaviours. METHODS: This paper reports a secondary data analysis. Semi-structured, individual interviews were conducted and analysed using qualitative description. RESULTS: Thirteen parents were interviewed for the study. Three themes were developed: (1) parent responses to healthcare provider interactions, (2) mixed emotions contributing to feeding approach and (3) variable parenting feeding styles. Within the third theme, two subthemes arose including (1) control and preoccupation and (2) striking a balance. CONCLUSION: Given the potential impact of higher weights on the progression of DMD, it is important that healthcare providers explore feeding behaviours with families. However, it is essential that healthcare providers consider the impact of these conversations on parents, as well as the broader issues that may place additional pressure on the lives of families.

Long-term mechanical ventilation and transitions in care: A narrative review.

OBJECTIVES: Individuals dependent on long-term mechanical ventilation (LTMV) for their day-to-day living are a heterogenous population who go through several transitions over their lifetime. This paper describes three transitions: 1) institution/hospital to community/home, 2) pediatric to adult care, and 3) active treatment to end-of-life for ventilator-assisted individuals (VAIs). METHODS: A narrative review based on literature and the author's collective practical and research experience. Four online databases were searched for relevant articles. A manual search for additional articles was completed and the results are summarized. RESULTS: Transitions from hospital to home, pediatric to adult care, and to end-of-life for VAIs are complex and challenging processes. Although there are several LTMV clinical practice guidelines highlighting key components for successful transition, there still exists gaps and inconsistencies in care. Most of the literature and experiences reported to date have been in developed countries or geographic areas with funded healthcare systems. CONCLUSIONS: For successful transitions, the VAIs and their support network must be front-and-center. There should be a coordinated, systematic, and holistic plan (including a multi-disciplinary team), life-time follow-up, with bespoke consideration of jurisdiction and individual circumstances.

Effect of Different Chemical Solvents on Bond Strength of Orthodontic Brackets: An In Vitro Study.

AIM: The purpose of the present study was to evaluate the impact of various chemical solvents on bond strength of orthodontic brackets. MATERIALS AND METHODS: One hundred healthy human premolars with undamaged buccal surfaces that were extracted for orthodontic purposes were gathered. Using 37% orthophosphoric acid, primer, and Transbond XT adhesive, ceramic 0.018" × 0.022" slot orthodontic brackets were adhered to the tooth surface. Following thermocycling, all samples were divided into four groups, with 25 samples in each group: group I: control; group II: application of ethanol; group III: application of acetone; and group IV: application of dimethyl sulfoxide (DMSO). Following the debonding tests, a double-ocular stereomicroscope was used to inspect the tooth surfaces. Additionally, adhesive remnant index (ARI) values were evaluated at 40× magnification. Data were recorded and statistically analyzed. RESULTS: The bond strength was lesser in acetone applied group (16.18 ± 3.64) followed by DMSO applied group (22.08 ± 2.86), ethanol applied group (24.36 ± 4.02), and control group (27.14 ± 3.68). There was a highly significant difference found between the chemical solvents group. The ARI score 3 was present in control (12%), ethanol (8%), and DMSO (4%), and it was absent in acetone applied group. The ARI score 0 was more in acetone applied group (24%). CONCLUSION: The present study concluded that the reduced debonding force was found with the application of acetone solvent followed by DMSO, ethanol, and control groups. Applying acetone can be a substitute technique to help with ceramic bracket debonding. CLINICAL SIGNIFICANCE: Orthodontic bracket debonding cannot occur without shear bond strength (SBS). The need for an ideal debonding technique for ceramic brackets without negative consequences arises from the risk of enamel damage that frequently follows the process. Acetone treatment prior to ceramic bracket debonding could be an alternate clinical technique to preventing enamel damage and facilitating debonding. How to cite this article: Bhushan R, Singh S, Sam G, et al. Effect of Different Chemical Solvents on Bond Strength of Orthodontic Brackets: An In Vitro Study. J Contemp Dent Pract 2023;24(12):940-943.

Mobility aids predict mortality after transcatheter aortic valve implantation.

BACKGROUND: Selection of appropriate patients for transcatheter aortic valve implantation (TAVI) can be challenging. Many factors can influence post-procedure outcomes. Traditional surgical scoring systems do not discriminate effectively. Medical parameters and functional indices can characterize mortality risk. Mobility is an important predictive functional index but is largely defined using subjective criteria. AIM: To describe the relationship between mobility, objectively defined by the requirement for gait aids to ambulate, and all-cause 30-day and long-term mortality in patients undergoing TAVI. METHODS: Mobility aid use was assessed in 1444 consecutive patients undergoing TAVI in a single center. Patients were categorized into "unaided," "1-stick," and "higher aid" groups based on the Brighton Mobility Index. Mortality tracking was obtained via the NHS Spine Portal in February 2021. RESULTS: Patients were aged 82 (IQR 78-86). 66% of patients walked unaided, 26% walked with 1 stick, and 8% required more assistance (e.g., 2 sticks, a Rollator, a Zimmer frame, or a wheelchair). Overall 30-day mortality for the whole cohort was 3.5%; 1-year mortality was 12.2%. Mobility was a significant predictor of 30-day mortality (p = 0.025). Use of a higher aid was associated with a mortality odds ratio of 2.83 (95% CI: 1.39-5.74). Mobility was also a significant predictor of long-term mortality (p < 0.001). Odds ratios for 1-stick and higher aid groups were 1.45 (95% CI: 1.21-1.72) and 2.01 (95% CI: 1.55-2.60), respectively. CONCLUSION: Objective assessment of mobility by gait aid use predicts both short and long-term survival in patients undergoing TAVI. Increased dependence on mobility aids is associated with a worse prognosis.

Outpatient Respiratory Management of Infants, Children, and Adolescents with Post-Prematurity Respiratory Disease: An Official American Thoracic Society Clinical Practice Guideline.

Background: Premature birth affects millions of neonates each year, placing them at risk for respiratory disease due to prematurity. Bronchopulmonary dysplasia is the most common chronic lung disease of infancy, but recent data suggest that even premature infants who do not meet the strict definition of bronchopulmonary dysplasia can develop adverse pulmonary outcomes later in life. This post-prematurity respiratory disease (PPRD) manifests as chronic respiratory symptoms, including cough, recurrent wheezing, exercise limitation, and reduced pulmonary function. This document provides an evidence-based clinical practice guideline on the outpatient management of infants, children, and adolescents with PPRD. Methods: A multidisciplinary panel of experts posed questions regarding the outpatient management of PPRD. We conducted a systematic review of the relevant literature. The Grading of Recommendations, Assessment, Development, and Evaluation approach was used to rate the quality of evidence and the strength of the clinical recommendations. Results: The panel members considered the strength of each recommendation and evaluated the benefits and risks of applying the intervention. In formulating the recommendations, the panel considered patient and caregiver values, the cost of care, and feasibility. Recommendations were developed for or against three common medical therapies and four diagnostic evaluations in the context of the outpatient management of PPRD. Conclusions: The panel developed recommendations for the outpatient management of patients with PPRD on the basis of limited evidence and expert opinion. Important areas for future research were identified.

Assessing air travel safety in neuromuscular disease: standard versus prolonged hypoxic challenge tests.

PURPOSE: The hypoxic challenge test (HCT) is used to evaluate safety for air travel in individuals with respiratory disease by breathing in 15% oxygen for 20 min. Our aim was to determine if a prolonged HCT, lasting 120 min, identified more individuals with neuromuscular disease at potential risk than the standard HCT lasting 20 min. METHODS: This was a cross-sectional study. All of the clinical testing took place at SickKids, Toronto, Canada. Patients were included in the study if they had a diagnosis of NMD, greater than 6 years of age, resting oxygen saturation ≥ 94%, and partial pressure of carbon dioxide (pCO2) ≤ 45 mmHg. Notable exclusion criteria were left ventricular ejection fraction < 30%, presence of a tracheostomy, and use of non-invasive ventilation for more than 12 h daily. Participants underwent a standard HCT as well as the prolonged HCT on the same day. RESULTS: Twenty-three patients consented to the study. One patient was withdrawn because he was unable to follow the study procedures. The 22 study participants had a mean age of 14.9 years (standard deviation (SD) of 5 years). Seventeen (77%) participants were male. Two participants were withdrawn on the day of testing due to hypercapnia. Twenty participants completed the standard and prolonged HCTs. None of the participants had a positive standard or prolonged HCT. CONCLUSION: Our results suggest that performing a standard or prolonged HCT may, in fact, not be of clinical utility in individuals with less severe NMD.

Fathers' Experiences in Alberta Family Integrated Care: A Qualitative Study.

BACKGROUND: The Family Integrated Care (FICare) program adapted for Alberta (AB) level II neonatal intensive care units (NICUs) aims to increase parental involvement and support during their NICU stay. The experience of fathers of preterm infants in a FICare program is currently unknown. PURPOSE: To describe the experiences of fathers of preterm infants born at 320/7 to 346/7 weeks' gestational age with AB FICare. METHODS: A qualitative substudy of a multicenter prospective cluster randomized controlled trial of FICare in 10 level II NICUs across Alberta. Fathers of preterm infants participated in a semistructured interview after discharge when their infants were at least 2 months' corrected gestational age. Journal entries written by fathers while in the NICU from the FICare intervention sites were also collected. Data were analyzed thematically and the interview and journal data were triangulated. FINDINGS: Thirteen fathers (9 from the FICare intervention and 4 from standard care) participated in semistructured interviews and there were 24 journals collected. Seven themes emerged: fear of the unknown, mental preparation, identifying the father's role, parenting with supervision, effective communication, postneonatal intensive care transition, and family life. Fathers enrolled in AB FICare attributed their level of confidence and positive neonatal intensive care experience that continued postdischarge to the care and attention they received during hospitalization. CONCLUSION: AB FICare may improve experiences for fathers of preterm infants in the NICU with continuation postdischarge. Future research should include designing and evaluating father-specific NICU programs.

Predicting Perioperative Respiratory Adverse Events in Children With Sleep-Disordered Breathing.

BACKGROUND: No evidence currently exists to quantify the risk and incidence of perioperative respiratory adverse events (PRAEs) in children with sleep-disordered breathing (SDB) undergoing all procedures requiring general anesthesia. Our objective was to determine the incidence of PRAEs and the risk factors in children with polysomnography-confirmed SDB undergoing procedures requiring general anesthesia. METHODS: Retrospective review of all patients with polysomnography-confirmed SDB undergoing general anesthesia from January 2009 to December 2013. Demographic and perioperative outcome variables were compared between children who experienced PRAEs and those who did not. Generalized estimating equations were used to build a predictive model of PRAEs. RESULTS: In a cohort of 393 patients, 51 PRAEs occurred during 43 (5.6%) of 771 anesthesia encounters. Using generalized estimating equations, treatment with continuous positive airway pressure or bilevel positive airway pressure (odds ratio, 1.63; 95% confidence interval [CI], 1.05-2.54; P = .031), outpatient (odds ratio, 1.37; 95% CI, 1.03-1.91; P = .047), presence of severe obstructive sleep apnea (odds ratio, 1.63; 95% CI, 1.09-2.42; P = .016), use of preoperative oxygen (odds ratio 1.82; 95% CI, 1.11-2.97; P = .017), history of prematurity (odds ratio, 2.31; 95% CI, 1.33-4.01; P = .003), and intraoperative airway management with endotracheal intubation (odds ratio, 3.03; 95% CI, 1.79-5.14; P < .001) were associated with PRAEs. CONCLUSIONS: We propose the risk factors identified within this cohort of SDB patients could be incorporated into a preoperative risk assessment tool that might better to identify the risk of PRAE during general anesthesia. Further investigation and validation of this model could contribute to improved preoperative risk stratification, decision-making (postoperative admission and level of monitoring), and health care resource allocation.

Volume-assured pressure support mode for noninvasive ventilation: can it improve overnight adherence in children with neuromuscular disease?

PURPOSE: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD). METHODS: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes. RESULTS: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes. CONCLUSION: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD.

The impact of polysomnograms and family-centred decision making in children with medical complexity.

Objectives: To determine whether a change in clinical management (e.g., new tracheostomy or adenotonsillectomy) occurred following a polysomnogram (PSG) in children with medical complexity (CMC) and to explore whether families' goals of care (regarding results and treatment implications) were discussed prior to the completion of a PSG. Methods: All CMC enrolled in a complex care program at the Hospital for Sick Children, Canada, who underwent a baseline PSG from 2009 to 2015 were identified. Exclusion criteria included (1) PSGs for ventilation titration and (2) PSGs outside the study time frame. Health records were retrospectively reviewed to determine demographics, medical histories, families' wishes, PSG results, and their impact on clinical care. Descriptive statistics were used to summarize results. Results: Of 145 patients identified, 96 patients met inclusion criteria. Fifty (52%) were male. Median age was 3 years. Forty-eight (50%) were diagnosed with clinically significant (i.e., moderate to severe obstructive sleep apnea, central sleep apnea, and/or hypoventilation) sleep-related breathing disorders. Of those diagnosed, 9 (19%) had surgery, 25 (52%) underwent respiratory technology initiation, and 3 (6%) underwent both. In the remaining 11 (23%) patients, treatment was either considered too risky or did not align with the families' wishes. Only 3 of 96 patients had clear documentation of their families' wishes prior to PSG completion. Conclusion: Recognizing the burden of medical tests for both the child and the health care system, a process of shared-decision making that includes clarifying a family's wishes may be prudent prior to conducting a PSG.

Virtual mask fitting in pediatric patients during COVID-19: A case series.

INTRODUCTION: The COVID-19 pandemic has been an unprecedented threat to our health care system. Clinicians had to pivot and develop creative and timely "virtual" solutions to provide clinical care. Our aim was to develop a standardized approach to virtual "mask fitting" for children who are either being initiated or are already on existing long-term ventilation (LTV) at a pediatric hospital. CASE AND OUTCOMES: We present three cases involving the care of children who required mask fitting for noninvasive ventilation (NIV). LTV team consultations were delivered via videoconference or phone. With the guidance of the respiratory therapist (RT), the family caregiver (FC) took measurements on their child using a standardized clinical approach (developed by the LTV RTs). Based on the measurements, an appropriate mask was selected. Successful mask fit was based on patient/FC reports, as well as objective leak data obtained from the NIV download data. DISCUSSION: Virtual clinics used for managing patients in our LTV program were feasible and efficient resulting in improved workflow for the RTs and convenience for patients and FCs. Patients and FCs had significantly less pressure to attend in-person clinics and expressed high satisfaction in terms of their experience and importantly, meeting respiratory care needs. Within the context of COVID-19, remote patient education and intervention can be delivered effectively, while reducing the risk of exposure from in-person visits to hospital. CONCLUSION: A virtual/telemedicine program to manage pediatric patients requiring mask fitting for LTV was a feasible option during COVID-19.

Antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis.

BACKGROUND: Stenotrophomonas maltophilia is one of the most common emerging multi-drug resistant organisms found in the lungs of people with cystic fibrosis and its prevalence is increasing. Chronic infection with Stenotrophomonas maltophilia has recently been shown to be an independent predictor of pulmonary exacerbation requiring hospitalization and antibiotics. However, the role of antibiotic treatment of Stenotrophomonas maltophilia infection in people with cystic fibrosis is still unclear. This is an update of a previously published review. OBJECTIVES: The objective of our review is to assess the effectiveness of antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis. The primary objective is to assess this in relation to lung function and pulmonary exacerbations in the setting of acute pulmonary exacerbations. The secondary objective is to assess this in relation to the eradication of Stenotrophomonas maltophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched a registry of ongoing trials and the reference lists of relevant articles and reviews. Date of latest search: 03 March 2020. SELECTION CRITERIA: Randomized controlled trials of Stenotrophomonas maltophilia mono-infection or Stenotrophomonas maltophilia co-infection with Pseudomonas aeruginosa in either the setting of an acute pulmonary exacerbation or a chronic infection treated with suppressive antibiotic therapy. DATA COLLECTION AND ANALYSIS: Both authors independently assessed the trials identified by the search for potential inclusion in the review. MAIN RESULTS: We identified only one trial of antibiotic treatment of pulmonary exacerbations that included people with cystic fibrosis with Stenotrophomonas maltophilia. However, this trial had to be excluded because data was not available per pathogen. AUTHORS' CONCLUSIONS: This review did not identify any evidence regarding the effectiveness of antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis. Until such evidence becomes available, clinicians need to use their clinical judgement as to whether or not to treat Stenotrophomonas maltophilia infection in people with cystic fibrosis. Randomized clinical trials are needed to address these unanswered clinical questions.

Putting our best foot forward: Clinical, treatment-based and ethical considerations of nusinersen therapy in Canada for spinal muscular atrophy.

Spinal muscular atrophy (SMA) is the most common genetic cause of infant mortality. SMA is a spectral disorder and is categorised based on symptom onset and severity. The median life expectancy for infants with SMA presenting before 6 months of age is less than 2 years without respiratory support. To date, there is no cure for SMA. In June 2017, nusinersen was approved in Canada as the first disease-modifying drug for SMA because of its demonstrated benefits on motor function and survival in clinical trials. However, with a price tag of almost 1 million dollars for the first year of therapy, careful clinical, treatment-based and ethical consideration of the principles of (i) best interests; (ii) universality; (iii) portability; (iv) public administration; (v) accessibility; and (vi) comprehensiveness are important guideposts to ensure transparent and equitable allocation of health-care resources for nusinersen and all other future orphan drugs.