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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.

Background: Previous studies have shown evidence of hypoxemia and hypercapnia during cardiopulmonary exercise test (CPET) evaluation in children with congenital central hypoventilation syndrome (CCHS). However, there are no longitudinal studies which compared CPET findings to polysomnogram (PSG) or PHOX2B mutation, to date.Objectives: To describe the longitudinal CPET findings in a cohort of children with CCHS and correlate the findings to the PSG results.Methods: This retrospective study was conducted in children with CCHS followed in the Long-term Ventilation Program at SickKids, Toronto, Canada between September, 2013 and January, 2020. CCHS genetic mutation, age of diagnosis, ventilatory support, family history, disease associations of CCHS, CPETs, and PSG parameters were recorded and analyzed.Results: A total of nine patients with CCHS (46 CPETs and 46 PSGs) were enrolled. Four (44.4%) children had polyalanine repeat mutations. The mean (SD) age at the time of diagnosis and duration of ventilatory usage were 3.2 ± 3.4 years and 11.5 ± 2.8 years, respectively. All abnormal CPETs had hypercapnia in at least 1 phase of the exercise test. Hypercapnia (12/46; 26.1%) at peak of exercise was the most common abnormality. None of the children experienced an oxygen desaturation below 90%. End-tidal CO 2 (PetCO 2 ) at rest and at peak exercise in the CPETs were significantly correlated with PSG TcCO 2 while PetCO 2 at anaerobic threshold was correlated with CO 2 in pre-PSG capillary blood gas. Conclusion:Nocturnal hypoventilation may impact the CPET results in CCHS children. Serial CPETs should be considered standard clinical care for all CCHS children.

Pediatric sepsis remains a major health problem and is a leading cause of death and long-term disability worldwide. This study aims to characterize epidemiologic, therapeutic, and outcome features of pediatric severe sepsis and septic shock in three Asian countries. DESIGN:A multicenter retrospective study with longitudinal clinical data over 1, 6, 24, 48, and 72 hours of PICU admission. The primary outcome was PICU mortality. Multivariable logistic regression analysis was used to identify factors at PICU admission that were associated with mortality SETTING: Nine multidisciplinary PICUs in three Asian countries. PATIENTS:Children with severe sepsis or septic shock admitted to the PICU from January to December 2017. INTERVENTION: None. MEASUREMENT AND MAIN RESULTS: A total of 271 children were included in this study. Median (interquartile range) age was 4.2 years (1.3-10.8 yr). Pneumonia (77/271 [28.4%]) was the most common source of infection. Majority of patients (243/271 [90%]) were resuscitated within the first hour, with fluid bolus (199/271 [73.4%]) or vasopressors (162/271 [59.8%]). Fluid resuscitation commonly took the form of normal saline (147/199 [74.2%]) (20 mL/kg [10-20 mL/kg] over 20 min [15-30 min]). The most common inotrope used was norepinephrine 81 of 162 (50.0%). Overall PICU mortality was 52 of 271 (19.2%). Improved hemodynamic variables (e.g., heart rate, blood pressure, and arterial lactate) were seen in survivors within 6 hours of admission as compared to nonsurvivors. In the multivariable model, admission severity score was associated with PICU mortality. CONCLUSIONS:Mortality from pediatric severe sepsis and septic shock remains high in Asia. Consistent with current guidelines, most of the children admitted to these PICUs received fluid therapy and inotropic support as recommended.

Background To promote an acceptance rate of COVID-19 immunization among Thai children, concerns about parental vaccination hesitancy should be urgently studied. This study aimed to examine the parental COVID-19 vaccination hesitancy (PVh) level and influencing factors among Thai parents of children 5–18 years of age. Methods This cross-sectional survey was conducted in Thailand during May and June of 2022. The Google forms for data collection were distributed to parents (a father, a mother, or one who nurtures and raises a child) via various online social media. Data regarding PVh level, relevant attitudes, experiences of COVID-19 and COVID-19 vaccination (EC&V), and family contexts (FC) were collected and analyzed using descriptive statistics. Mann–Whitney U test was used to compare the differences among groups of parents based on EC&V and FC. The factors influencing PVh were assessed by multiple regression analysis. Results Four hundred and eighty-eight parents completed the online questionnaire. Their median (IQR) age was 41 (35–47) years. They lived in different provinces from all regions across Thailand. Ninety percent of them were authorized persons to make decision about children vaccination. Fifty-eight percent of the respondents had vaccine hesitancy, defined as PVh level at moderate or above. Parents who had ever refused COVID-19 vaccination for themselves or refused to vaccinate their children against any other diseases had statistically significant higher levels of PVh (p value < 0.001). Conversely, the parents who had finished the initial COVID-19 vaccine had lower PVh levels with statistical significance (p value = 0.001). Attitude towards COVID-19 (AC), attitude towards COVID-19 vaccine (AV), and perceived behavioral control (PC) of the parents negatively influenced PVh with statistical significance, according to the results of the multiple regression analysis (Betas = − 0.307, − 0.123, and − 0.232, respectively). Conclusions COVID-19 vaccination hesitancy was commonly found among Thai parents. The factors of the hesitancy were multifaceted. Different experiences regarding COVID-19 vaccination for themselves and any vaccinations for their children were associated with different PVhs. The attitudes especially AC, AV, and PC statistically influenced PVh. These findings should be exploited for national and local policy planning as well as public campaigns.

Purpose To evaluate the impact of the COVID-19 pandemic on non-invasive positive airway pressure (PAP) usage among children with sleep-disordered breathing (SDB). Methods PAP usage data in children with SDB aged 1 to 18 years old at The Hospital for Sick Children, Canada, were analyzed. The PAP usage data were recorded for 3 months prior to and 3 months following the COVID-19 lockdown in Ontario, Canada. The primary outcomes of interest were (i) percentage of days that PAP was used for ≥ 4 h and (ii) average daily usage of PAP based on days when PAP was used. Results A total of 151 children were included. The mean (± SD) age and BMI were 12.6 ± 4.1 years and 28.7 ± 12.4 kg/m 2 , respectively. The median (IQR) percentage of days of PAP usage for ≥ 4 h and average nightly PAP usage was significantly higher during compared with prior to the pandemic (76.7 [19.0–94.0] vs 62.0 [15.5–89.0]%, p = 0.02, and 406.0 [244.0–525.0] vs 367.0 [218.0–496.0] min, p = 0.006, respectively). Within this cohort, 95/151 (63%) children with SDB showed increased PAP usage and 56/151 (37%) either decreased the amount of time they used PAP or stopped PAP use altogether. Conclusions COVID-19 pandemic has provided opportunities for increased PAP usage in a significant number of children with SDB. A subset of children with prior evidence for suboptimal PAP usage showed further decreases in PAP usage during the pandemic. This information is critical for clinicians to provide anticipatory guidance to encourage PAP usage both during the pandemic and beyond. Supplementary Information The online version contains supplementary material available at 10.1007/s11325-021-02409-w.

BackgroundPolysomnography (PSG) is the gold standard for the diagnosis of pediatric sleep‐disordered breathing (SDB). However, the literature characterizing the indications for inpatient PSGs and the impact on clinical decision‐making is limited.ObjectiveTo determine the indications, results, and outcomes for children undergoing inpatient PSGs at our institution.MethodsWe performed a retrospective review of children aged 0–18 years who underwent inpatient diagnostic PSGs between July 2018 and July 2021 at SickKids, Toronto, Canada. Baseline characteristics, indications, and management were reviewed and characterized by descriptive statistics.ResultsEighty‐eight inpatient PSGs were performed in 75 children (male 62.7%). Median (interquartile range) age and body mass index z‐score were 1.5 (0.2, 10.8) years and 0.27 (−1.58, 2.66), respectively. The most common indication for inpatient PSG was initiation and titration of ventilation (n = 34/75, 45.3%). Of the 75 children, 48 (64%) had multiple complex chronic conditions (CCCs). Sixty children (80%) underwent a baseline PSG for either the entire night or a portion of the night. Of these studies, 54 (90%) had clinically significant SDB of which isolated obstructive sleep apnea (OSA; 17/60, 28.3%) was the most common. The following management was undertaken for the 54 patients with SDB; respiratory technology (88.9%), surgical intervention (31.5%), positional therapy (1.9%), intranasal steroids (3.7%), and no further intervention (5.6%), respectively.ConclusionsOur study highlights that inpatient PSG was an important diagnostic tool resulting in directed medical and surgical management. Future multicenter studies are needed to compare indications for inpatient PSGs across institutions to develop evidence‐based clinical practice guidelines.

We found the association between ULTE4 and therapeutic response to monteleukast. The uLTE₄ level of ≥ 1,457 pg/mg creatinine could predict the therapeutic response to montelukast in children who had ATH and mild OSA.