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Objectives: Poor sleep is frequently reported in children with neuromuscular diseases (NMD) and cerebral palsy (CP) however breathing disorders during sleep are often the clinical focus. Periodic limb movements (PLMs) have an increased prevalence in adults with NMD and may contribute to sleep disturbance in this population. We assessed the prevalence of PLMs in children with NMD or CP., Methods: Retrospective review of polysomnography (PSG) with leg electromyography in children age 1-18 years with NMD (including Duchenne muscular dystrophy, myotonic dystrophy, spinal muscular atrophy) or CP performed at a paediatric sleep centre 2004-2022., Results: Leg electromyography was available in at least 1 PSG in 239 children (125 NMD, 114 CP), and in 2 PSGs in 105 children (73 NMD, 32 CP). At initial PSG, 72 (30 %) were female with a median age 9y and respiratory disturbance index 3.5/h (interquartile range 1.3-9.9/h). Elevated PLM index (PLMI; >5/h) occurred in 9.6 % of each of the CP and NMD groups, quantified by initial PSG. Overall, PLMI increased from baseline (median 0, maximum 33/h) to follow-up (median 0, maximum 55.8/h; p < 0.05). In those with an elevated PLMI, arousal percentage attributable to PLMs was up to 25 % (median 7.5 %)., Conclusions: Elevated PLMI occurred at a higher prevalence in children with NMD and CP than reported in other clinic-referred paediatric populations. It is important that PLMs are not overlooked as identification and treatment may help improve sleep outcomes. Further research is required to understand the pathophysiology and consequences of PLMs specifically in this population., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

There is conflicting evidence for impaired autonomic control of heart rate (HR) in adults with narcolepsy and idiopathic hypersomnolence (IH). Despite these chronic hypersomnia conditions primarily being diagnosed around the age of puberty, there are limited studies in children. The present study investigated cardiovascular control using heart rate variability (HRV) and the extent of nocturnal HR dipping during sleep in children and adolescents with narcolepsy and IH. Children having an overnight polysomnographic study followed by a multiple sleep latency test (MSLT) for investigation of excessive daytime sleepiness (EDS) between May 2010 to December 2023 were included: 28 children diagnosed with narcolepsy, 11 with IH, and 26 subjectively sleepy children who did not meet the diagnostic criteria for either narcolepsy or IH. Each clinically referred child was matched for age and sex with a control. Time domain and frequency domain HRV were calculated from ECG recorded at 512 Hz. There were no differences in either time domain or spectral analysis of HRV between clinical groups or between clinical groups and their control group. The expected sleep state differences in HRV were observed in all groups. There was also no difference in HR nocturnal dipping between groups. Despite evidence for abnormal autonomic function in adults with narcolepsy and IH, our study did not identify any abnormalities in HR, HR control, or nocturnal dipping of HR in children referred for assessment of EDS. This suggests that autonomic dysfunction may be a feature of these conditions that develops in later life., (© 2024 The Author(s). Journal of Sleep Research published by John Wiley & Sons Ltd on behalf of European Sleep Research Society.)

Background: Children with Down syndrome (DS) have a high prevalence of sleep disordered breathing (SDB) and altered cardiovascular autonomic control. We aimed to analyze the effect of DS on the surge in heart rate (HR) and pulse transit time (PTT, an inverse surrogate measure of blood pressure change) at respiratory event termination., Methods: 44 children (3-19 y) with DS and 44 typically developing (TD) children matched for SDB severity, age and sex underwent overnight polysomnography. Multilevel modelling determined the effect of DS on HR and PTT changes between a 10s pre-event to the latter half of each respiratory event (late-event) and 15s post-event during NREM and REM, accounting for SDB severity and event length., Results: The children with DS had a significantly smaller % change in HR late-event to post-event (NREM: DS 26.4 % ± 17.5 % (mean ± SD), TD 30.7 % ± 21.0 %; REM DS 16.9 % ± 15.3 %, TD 21.0 % ± 14.0 %; p < 0.05 for both) compared with TD children for obstructive events, and central events (13.2 % ± 17.0 %, TD 18.8 % ± 17.0 %; p < 0.01) during REM. %change in PTT was significantly smaller in the DS group during NREM and REM from pre-event and late-event to post-event compared with TD children for obstructive and central events., Conclusion: These results suggest children with DS have dampened HR and BP responses to respiratory events compared with TD children. Whether this is symptomatic of autonomic dysfunction or a protective factor for the cardiovascular system in children with DS remains to be elucidated., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: Sleep disturbance is an important feature of fetal alcohol spectrum disorder (FASD). We sought to describe sleep patterns in school-aged children with FASD, in comparison with a typically developing community group, and investigate the relationship between sleep and neurodevelopmental profiles., Method: The FASD cohort (N = 36) was recruited from a tertiary Australian FASD diagnostic center, and the typically developing group (N = 36) was previously recruited as a control cohort for a separate study. Sleep disturbance was assessed with the caregiver-completed Sleep Disturbance Scale for Children (SDSC) questionnaire. Neurodevelopmental assessment results for the 10 domains impaired in FASD were used for correlations with sleep disturbance., Results: In the FASD group, 80% of children scored above the SDSC cutoff, compared with 22% of the control group ( p < 0.001). Statistically significant group differences were seen for all 6 subscales of the SDSC ( p < 0.05). The most frequently affected domains in the FASD group related to difficulties with initiating and maintaining sleep (58%), sleep-wake transition disorders (44%), and disorders of arousal (42%). A statistically significant relationship was not found between sleep and the severity of neurodevelopmental impairment or impairment of a particular domain, acknowledging the limitations of our small sample size. Half of the FASD sample (52%) were taking a pharmaceutical agent to support sleep, which was not associated with lower SDSC scores., Conclusion: In this small study, sleep disturbances were frequently reported by carers of children with FASD, independent of the severity of their neurodevelopmental impairments. Persistent sleep disturbance despite the use of sleep medications highlights the need for prospective studies exploring sleep interventions in this population. Integration of behavioral sleep medicine into management is recommended for all children with FASD., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Children with Down syndrome (DS) are at increased risk of sleep-disordered breathing (SDB). We investigated sleep spindle activity, as a marker of sleep quality, and its relationship with daytime functioning in children with DS compared to typically developing (TD) children., Methods: Children with DS and SDB (n = 44) and TD children matched for age, sex and SDB severity underwent overnight polysomnography. Fast or Slow sleep spindles were identified manually during N2/N3 sleep. Spindle activity was characterized as spindle number, density (number of spindles/h) and intensity (density × average duration) on central (C) and frontal (F) electrodes. Parents completed the Child Behavior Check List and OSA-18 questionnaires., Results: In children with DS, spindle activity was lower compared to TD children for F Slow and F Slow&Fast spindles combined (p < 0.001 for all). Furthermore, there were no correlations between spindle activity and CBCL subscales; however, spindle activity for C Fast and C Slow&Fast was negatively correlated with OSA-18 emotional symptoms and caregiver concerns and C Fast activity was also negatively correlated with daytime function and total problems., Conclusions: Reduced spindle activity in children with DS may underpin the increased sleep disruption and negative effects of SDB on quality of life and behavior., Impact: Children with Down syndrome (DS) are at increased risk of sleep-disordered breathing (SDB), which is associated with sleep disruption affecting daytime functioning. Sleep spindles are a sensitive marker of sleep quality. We identified for the first time that children with DS had reduced sleep spindle activity compared to typically developing children matched for SDB severity. The reduced spindle activity likely underpins the more disrupted sleep and may be associated with reduced daytime functioning and quality of life and may also be an early biomarker for an increased risk of developing dementia later in life in children with DS., (© 2023. The Author(s).)

Introduction: Continuous positive airway pressure (CPAP) for treatment of obstructive sleep apnea (OSA) may pose a significant burden on families. We assessed the impact of CPAP for children on quality of life (QOL) and caregiver treatment burden., Methods: Prospective cohort study of children commencing outpatient CPAP in a specialist sleep centre 2020-2022. Questionnaires regarding sleep-related symptoms (PROMIS Pediatric Sleep Disturbance and Sleep-Related Impairment), QOL (OSA-18, QI-Disability), caregiver burden (Caregiver Strain Questionnaire) and overall health impact (Glasgow Children's Benefit Inventory) were completed by caregivers at CPAP commencement and 6 weeks later., Results: Twenty-six patients completed follow-up (7 female; median age 11.4 year, baseline obstructive apnea hypopnea index 10.3/h; 77% overweight or obese, 73% comorbidity other than obesity). OSA-related QOL (OSA-18) significantly improved at follow-up (p < 0.01), as did child general QOL (p < 0.001), sleep disturbance (p < 0.01) and sleep-related impairment (p < 0.001). Caregivers mostly rated CPAP as beneficial to their child's health but 19% rated CPAP as harmful or having no effect. Caregiver strain reduced at follow-up (p < 0.001) and benefit outweighed inconvenience (p < 0.0001) in 81%. CPAP adherence was correlated with overall health impact (r = 0.67, p < 0.01) but not with caregiver rating of inconvenience., Conclusions: CPAP resulted in improvements in QOL and sleep-related symptoms, and reduced caregiver strain. Perceived benefits outweighed the burden of treatment for most but not all families. CPAP adherence was moderately correlated with family-reported measures of benefit but not related to perceived inconvenience. This study provides reassuring evidence regarding the benefits and impacts of CPAP for children, many of whom already have complex health care needs., (© 2024 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

This paper investigated cortical thickness and volumetric changes in children to better understand the impact of obstructive sleep disordered breathing (SDB) on the neurodevelopment of specific regions of the brain. We also aimed to investigate how these changes were related to the behavioral and cognitive deficits observed in the condition. Neuroimaging, behavioral, and sleep data were obtained from 30 children (15 non-snoring controls, 15 referred for assessment of SDB) aged 7 to 17 years. Gyral-based regions of interest were identified using the Desikan-Killiany atlas. Student's t-tests were used to compare regions of interest between the controls and SDB groups. We found that the cortical thickness was significantly greater in the right caudal anterior cingulate and right cuneus regions and there were volumetric increases in the left caudal middle frontal, bilateral rostral anterior cingulate, left, right, and bilateral caudate brain regions in children with SDB compared with controls. Neither cortical thickness nor volumetric changes were associated with behavioral or cognitive measures. The findings of this study indicate disruptions to neural developmental processes occurring in structural regions of the brain; however, these changes appear unrelated to behavioural or cognitive outcomes., (© 2023 The Authors. Journal of Sleep Research published by John Wiley & Sons Ltd on behalf of European Sleep Research Society.)

Introduction: Sleep disorders, particularly sleep disordered breathing (SDB), are common in children with Down syndrome (DS). We investigated the relationship between SDB severity and parental psychological wellbeing and their perception of social support., Methods: 44 children with DS (3-19 years) underwent overnight polysomnography and were categorised into three groups: primary snoring, Mild and Moderate/Severe obstructive sleep apnoea (OSA). Parents completed questionnaires about their child's behaviour (Child Behavior Checklist), sleep symptoms (Pediatric Sleep Survey Instrument) and SDB-related quality of life (OSA-18), together with the DUKE-UNC Functional Social Support (DUKE) and Psychological General Well-Being Index (PGWBI) questionnaires for themselves. 34 children completed a follow-up study after 2 years., Results: There were no significant differences between SDB severity groups for parental perceived social support or psychological wellbeing. Total scores on the DUKE were below average and PGWBI scores were indicative of moderate psychological distress in all three groups. Reduced perceived levels of social support were significantly correlated with externalising child behaviour and sleep disturbance. Diminished parental psychological wellbeing was also significantly correlated with increased sleep disturbances and reduced quality of life in children. At follow-up there were no significant changes in any questionnaire outcome, however parents of children with improved SDB severity had improved PGWBI vitality scores., Conclusion: The degree of parent-reported sleep disturbance in children with DS was linked to suboptimal perceived parental social support and poor psychological wellbeing. Our results emphasise the need for enhanced awareness of the detrimental effects of sleep problems in children with DS on parental wellbeing., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: To evaluate whether kangaroo mother care (KMC) in preterm infants on non-invasive respiratory support improves indices of cardiorespiratory wellbeing., Study Design: Prospective quasi-experimental observational study., Setting: Tertiary perinatal neonatal unit., Patients: 50 very preterm infants being managed with nasal continuous positive airway pressure., Interventions: Continuous high-resolution preductal pulse-oximetry recordings using Masimo Radical-7 oximeter for 1 hour (incubator care) followed by 1 hour during KMC performed on the same day., Main Outcome Measures: Measures of cardiorespiratory stability (dips in oxygen saturations (SpO 2 )) of ≥5% less than baseline, % time spent with oxygen saturations <90%, SpO 2 variability and heart rate fluctuation and incidence of bradycardias., Results: The gestational age and birth weight of the cohort were 28.4±2.1 weeks and 1137±301 g, respectively. Dips in SpO 2 of ≥5% less than baseline were significantly fewer with KMC, median (IQR) 24 (12 to 42) vs 13 (3 to 25), p=0.001. SpO 2 variability (Delta 12 s and 2 s), (1.24±0.6 vs 0.9±0.4, p=0.005 and 4.1±1.7 vs 2.8±1.2, p<0.0001) and rapid resaturation and desaturation indices were significantly lower during KMC, compared with incubator care. Percentage time spent in oxygen saturations <90% was less with KMC (7.5% vs 2.7%, p=0.04). Mean heart rate was comparable although fluctuations in heart rate (rise by >8 bpm) were lower with KMC (43±22 vs 33±20, p=0.03). Seven (14%) infants had bradycardias during incubator care and none during KMC, p=0.012., Conclusions: KMC improves cardiorespiratory stability in ventilated preterm infants. Regular KMC has the potential to improve clinical outcomes in this vulnerable cohort., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The gold standard investigation for central disorders of hypersomnolence is the Multiple Sleep Latency Test (MSLT). As the clinical features of these disorders of hypersomnolence evolve with time in children, clinicians may consider repeating a previously non-diagnostic MSLT. Currently there are no guidelines available regards the utility and timing of repeating paediatric MSLTs., Methods: Retrospective review of children aged 3-18years with ≥2MSLTs between 2005 and 2022. Narcolepsy was defined as mean sleep latency (MSL) <8min with ≥2 sleep onset REM (SOREM); idiopathic hypersomnia (IH) was defined as MSL <8min with <2 SOREM. MSLTs not meeting these criteria were labelled non-diagnostic., Results: 19 children (9 female) with initial non-diagnostic MSLT underwent repeat MSLT, with 6 proceeding to a 3rd MSLT following 2 non-diagnostic MSLTs. The 2nd MSLT resulted in diagnosis in 6/19 (32 %) (3 narcolepsy, 3 IH); and 2/6 (33 %) 3rd MSLT were diagnostic (2 IH). Median age at initial MSLT was 7.5y (range 3.4-17.8y), with repeat performed after median of 2.9y (range 0.9-8.2y), and 3rd after a further 1.9 years (range 1.2-4.2y). Mean change in MSL on repeat testing was -2min (range -15.5min to +4.9min, p = 0.18). Of the 8 diagnostic repeat MSLTs, in addition to the MSL falling below 8 min, 2 children also developed ≥2 SOREM that had not been previously present., Conclusions: A third of repeat MSLTs became diagnostic, suggesting repeat MSLT should be considered in childhood if clinical suspicion persists. Further work needs to address the ideal interval between MSLTs and diagnostic cut-points specific to the paediatric population., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Introduction: Sleep disordered breathing (SDB) is common in children and the most common reason for adenotonsillectomy. This large observational cohort study from a specialist outpatient clinic describes the impact of intranasal steroids (INS) on symptom improvement and the need for surgery., Method: Observational cohort study of 568 children assessing the impact of INS using the OSA-5 questionnaire with clinical and surgical outcome measures., Results: The mean OSA-5 score at first visit was 7.78. Symptoms were persistent for a median 9 months (range 2-72). 51% underwent a trial of INS with 56% reporting symptomatic improvement. The mean score decreased from 8.2 to 5.5 (p < 0.0001) in those prescribed INS. They had a significantly higher symptom load (p < 0.01), turbinate size (p < 0.005) and history of atopy (p < 0.01) than the non-trial group. The rate of surgery in the non-trial group was 56% compared with 38% in those who had INS (p < 0.001). With increasing symptom burden, the reported improvement with INS and comparative reduction in surgery increased. Baseline OSA-5 scores were predictive of rates of surgery. Atopic status or age did not influence response to INS., Conclusion: The mean score at first visit and the median duration of symptoms indicated significant persistent symptoms in this cohort. The use of INS improved symptoms of SDB in 56%. The need for surgery in the group that received INS was 38% compared with 56% in those not trialling INS, despite the non-trial group having significantly less symptoms and signs. Symptomatic improvement was not influenced by age or atopic status., Competing Interests: Declaration of competing interest There is no conflict of interest. The research did not receive any specific grant from any public, commercial or not-for profit sector., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Children with Down syndrome are at increased risk of obstructive sleep disordered breathing, which has deleterious effects on daytime functioning. We aimed to examine the effects of treatment of sleep disordered breathing on sleep quality and daytime functioning in children with Down syndrome, and hypothesised that these would be improved. Thirty-four children completed a baseline study and a follow-up 2 years later. Measures at both time points included 7 days of actigraphy and parents completed a number of questionnaires assessing sleep, behaviour, daytime functioning, and quality of life. All children had overnight polysomnography at baseline; 15 children (44%) were treated. At baseline the treated group had more severe sleep disordered breathing compared with the untreated group: obstructive apneoa-hypopnoea index 29.3 ± 38.2 events/h versus 3.3 ± 5.2 events/h (p < 0.01). Actigraphy showed no significant differences in total sleep time, sleep efficiency, sleep schedules from baseline to follow up in either group. The sleep disturbance (p < 0.01) and total problems (p < 0.05) scales on the OSA-18 and the sleep disordered breathing subscale on the Paediatric Sleep Problem Survey Instrument (p < 0.01) improved in the treated children. There were no changes in any measure in the untreated children. Treatment of sleep disordered breathing improves symptoms, sleep disturbance and quality of life in children with Down syndrome, but has no demonstrable impact on actigraphic sleep measures or daytime behaviour or function. In contrast, children who were not treated, despite having less severe disease at baseline, had increased sleep disruption and no change in quality of life., (© 2023 European Sleep Research Society.)

Excessive daytime sleepiness (EDS) is common in childhood and is currently quantified using adult criteria on a multiple sleep latency test (MSLT). This study aimed to describe paediatric MSLT results, particularly focussing on a previously proposed alternative mean sleep latency (MSL) threshold for children of 12 min, and assess the impact of a 5th nap. We performed a retrospective analysis of MSLTs at a single paediatric centre from 2004 to 2021. Narcolepsy was defined as a mean sleep latency (MSL) ≤8min with ≥2 sleep onset REM (SOREM) periods. Idiopathic Hypersomnia (IH) was defined as a MSL ≤8min with <2 SOREMs. An ambiguous MSLT result was defined as a MSL 8-12min and/or ≥2 SOREM periods. Of 214 MSLTs [50 % female, median age 14.0y (range 3.3-20.1y)], narcolepsy was diagnosed in 48 (22 %), IH in 22 (10 %) and the result was ambiguous in 44 (21 %). Those with ambiguous MSLT results were older (15.6 vs 13.4y, p = 0.006) with a higher proportion of females (61 % vs 35 %, p = 0.01) in comparison to the narcolepsy group. A 5th nap was performed in 60 (28 %) of MSLTs and only changed the outcome in one case. In conclusion, MSLT results are borderline in 21 % of paediatric cases, suggesting that current adult diagnostic criteria may miss narcolepsy and IH in children. A 5th nap usually makes no difference or increases the MSL, suggesting that a four nap MSLT protocol could be used apart from rare cases where the result is borderline after the 4th nap., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: To identify the outcomes considered important, and factors influencing the patient experience, for parents and caregivers of children presenting to hospital with a severe acute exacerbation of asthma. This work contributes to the outcome-identification process in developing a core outcome set (COS) for future clinical trials in children with severe acute asthma., Design: A qualitative study involving semistructured interviews with parents and caregivers of children who presented to hospital with a severe acute exacerbation of asthma., Setting: Hospitals in 12 countries associated with the global Pediatric Emergency Research Networks, including high-income and middle-income countries. Interviews were conducted face-to-face, by teleconference/video-call, or by phone., Findings: Overall, there were 54 interviews with parents and caregivers; 2 interviews also involved the child. Hospital length of stay, intensive care unit or high-dependency unit (HDU) admission, and treatment costs were highlighted as important outcomes influencing the patient and family experience. Other potential clinical trial outcomes included work of breathing, speed of recovery and side effects. In addition, the patient and family experience was impacted by decision-making leading up to seeking hospital care, transit to hospital, waiting times and the use of intravenous treatment. Satisfaction of care was related to communication with clinicians and frequent reassessment., Conclusions: This study provides insight into the outcomes that parents and caregivers believe to be the most important to be considered in the process of developing a COS for the treatment of acute severe exacerbations of asthma., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Obstructive sleep apnoea (OSA) is common in children and adolescents with Down syndrome (DS). Clinical guidelines recommend that all children with DS have polysomnography (PSG) for assessment of OSA by the age of 4 years, but access is limited and testing may be burdensome for children and families., Methods: The purpose of this prospective cross-sectional cohort study was to identify a model to predict OSA in this group that could be tested in an external population to triage children and adolescents with DS for PSG. These models were based on a comprehensive set of potential predictive demographic, anthropometric, quality of life and sleep-related variables., Results: The results of this study show the predictive power of a model based on the sleep disordered breathing subscale of the Pediatric Sleep Survey Instrument and sleep fragmentation quantified using actigraphy in determining moderate-severe OSA in children and adolescents with DS. This model exhibits high sensitivity (82%), specificity (80%), positive predictive value (75%) and negative predictive value (86%)., Conclusions: We demonstrate the utility of a tool containing the sleep disordered breathing subscale of the Pediatric Sleep Survey Instrument and sleep fragmentation quantified using actigraphy in identifying children and adolescents with DS who have moderate/severe OSA., (© 2023 The Authors. Journal of Intellectual Disability Research published by MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

Background: This study compared measurements of sleep and wake assessed with actigraphy, sleep diary and polysomnography in children with Down syndrome (DS) and also compared measures of actigraphic sleep recording in children with DS and typically developing (TD) children., Methods: Children with DS aged 3-19 years (N = 44) referred for assessment of sleep disordered breathing (SDB) underwent overnight polysomnography, together with 1 week of actigraphy with sleep diary. Actigraphy data from the children with DS were compared with data collected from TD children, matched for age and sex., Results: 22 children (50%) with DS completed >3 consecutive nights of actigraphy with a matched sleep diary. There were no differences between bedtimes, wake times or time in bed on weeknights, weekends or over 7 nights between actigraphy and sleep diary. Total sleep time was over estimated by the sleep diary by almost 2 h and the number of night awakenings under-reported. Compared to matched TD children (N = 22), there was no difference in total sleep time, however children with DS fell asleep more quickly (p < 0.001), had more awakenings (p = 0.001) and more time awake after sleep onset (p = 0.007). Children with DS exhibited less variability in both bedtimes and wake times, and fewer had >1 h sleep schedule variability., Conclusions: Parental sleep diaries over-estimate total sleep time but accurately report bed and wake times compared to actigraphy in children with DS. Children with DS have more regular sleep patterns than TD children of the same age, which is important for optimising daytime functioning. The reasons behind this warrant further investigation., Competing Interests: Declaration of competing interest Authors declare conflict of interest none., (Crown Copyright © 2023. Published by Elsevier B.V. All rights reserved.)

Background: Resolution of sleep disordered breathing (SDB) in typically developing children normalises heart rate variability (HRV), a measure of autonomic control, to that of non-snoring controls. Children with Down Syndrome (DS) have dampened heart rate variability (HRV) but the effect of treatment is not known. To assess the effect of improvement of SDB on autonomic control we compared HRV in children with DS whose SDB improved over 2 y, to those whose SDB did not improve., Methods: 24 children (3-19 y) had a baseline and follow-up polysomnographic study 2 y later. Improved SDB was defined as a reduction in obstructive apnea hypopnea index (OAHI) to ≤ 50% of baseline. Children were grouped into Improved (n = 12) and Unimproved (n = 12). Power spectral analysis of the ECG determined low frequency (LF), high frequency (HF) power and the LF/HF ratio. Seven children in the Improved and 2 in the Unimproved group were treated following the baseline study., Results: In the Unimproved group at follow-up, LF power was lower compared to baseline during N3 and Total Sleep (p < 0.05 for both). HF power was lower during REM (p < 0.05). HRV remained unchanged between studies in the Improved group., Conclusion: Autonomic control worsened as indicated by lower LF and HF power in children whose SDB was not improved. In contrast, in those children with improved SDB, autonomic control remained the same, suggesting improvement in SDB severity prevents further worsening of autonomic control in children with DS., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objectives: To describe the incidence of and patterns of 'escalated care' (care in addition to standard treatment with systemic corticosteroids and inhaled bronchodilators) for children receiving prehospital treatment for asthma., Design: Retrospective observational study., Setting: State-wide ambulance service data (Ambulance Victoria in Victoria, Australia, population 6.5 million) PARTICIPANTS: Children aged 1-17 years and given a final diagnosis of asthma by the treating paramedics and/or treated with inhaled bronchodilators from 1 July 2019 to 30 June 2020., Primary and Secondary Outcome Measures: We classified 'escalation of care' as parenteral administration of epinephrine, or provision of respiratory support. We compared clinical, demographic and treatments administered between those receiving and not receiving escalation of care., Results: Paramedics attended 1572 children with acute exacerbations of asthma during the 1 year study period. Of these, 22 (1.4%) had escalated care, all receiving parenteral epinephrine. Patients with escalated care were more likely to be older, had previously required hospital admission for asthma and had severe respiratory distress at initial assessment.Of 1307 children with respiratory status data available, at arrival to hospital, the respiratory status of children had improved overall (normal/mild respiratory distress at initial assessment 847 (64.8%), normal/mild respiratory distress at hospital arrival 1142 (87.4%), p<0.0001)., Conclusions: Most children with acute exacerbations of asthma did not receive escalated therapy during their pre-hospital treatment from ambulance paramedics. Most patients were treated with inhaled bronchodilators only and clinically improved by the time they arrived in hospital., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To investigate the amount of time spent in periodic breathing and its consequences in infants born preterm before and after hospital discharge., Methods: Infants born preterm between 28-32 weeks of gestational age were studied during daytime sleep in the supine position at 32-36 weeks of postmenstrual age (PMA), 36-40 weeks of PMA, and 3 months and 6 months of corrected age. The percentage of total sleep time spent in periodic breathing (% total sleep time periodic breathing) was calculated and infants were grouped into below and above the median (8.5% total sleep time periodic breathing) at 32-36 weeks and compared with 36-40 weeks, 3 and 6 months., Results: Percent total sleep time periodic breathing was not different between 32-36 weeks of PMA (8.5%; 1.5, 15.0) (median, IQR) and 36-40 weeks of PMA (6.6%; 0.9, 15.1) but decreased at 3 (0.4%; 0.0, 2.0) and 6 months of corrected age 0% (0.0, 1.1). Infants who spent above the median % total sleep time periodic breathing at 32-36 weeks of PMA spent more % total sleep time periodic breathing at 36-40 weeks of PMA (18.1%; 7.7, 23.9 vs 2.1%; 0.6, 6.4) and 6 months of corrected age 0.9% (0.0, 3.3) vs 0.0% (0.0, 0.0)., Conclusions: Percentage sleep time spent in periodic breathing did not decrease as infants born preterm approached term corrected age, when they were to be discharged home. High amounts of periodic breathing at 32-36 weeks of PMA was associated with high amounts of periodic breathing at term corrected age (36-40 weeks of PMA), and persistence of periodic breathing at 6 months of corrected age., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Objectives: The association between helminthiasis and asthma remains inconclusive but can only be investigated in counties where helminthiasis is transitioning from a high to low burden. We investigated this association using data from a childhood respiratory cohort in Sri Lanka., Methods: A case-control study was nested within a population-based cohort of children aged 6-14 years in Sri Lanka. The stool samples of 190 children with asthma and 190 children without asthma were analyzed to assess the burden of helminth infestation. Logistic regression models were fitted to investigate the association of gastrointestinal helminth species with asthma., Results: Helminthiasis in children with and without asthma was 23.3% (n = 44) and 15.3% (n = 23), respectively. Those with asthma were more likely to have helminthiasis (odds ratio 3.7; 95% confidence interval 1.7, 7.7; P = 0.001), particularly with Trichiuris trichura (odds ratio 4.5; 95% confidence interval 1.6, 12.3; P = 0.004). Helminth eggs per gram of feces were not associated with asthma (P >0.05)., Conclusion: Our findings demonstrate a positive association between T. trichura infestation and asthma and point to the need to fully characterize this association to understand the likely immunological mechanism that drives it. This association highlights an important public health intervention in countries where these infestations are still prevalent, affecting 24% of the population worldwide., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objective: We aimed to investigate the frequency and severity of periodic breathing (PB) in clinically stable very preterm infants and identify infant and maternal factors associated with increased time spent and severity of PB in these infants., Method: Thirty-eight infants (28-32 weeks gestational age) who were ≥3 days off noninvasive respiratory support, were studied for 2-3 h with a daytime sleep study at 31-36 weeks postmenstrual age. Percent total sleep time spent in PB (%TSTPB) and time spent with SpO 2 <90%, <80%, and cerebral oxygenation <55% during PB were calculated. Infant and maternal characteristics were correlated with %TSTPB and hypoxia during PB., Results: The majority of infants (92%) had at least one episode of PB and infants spent a median 9.1 [interquartile range: 1.2, 15.5] %TSTPB. 80%, 37%, and 37% of infants experienced SpO 2 <90%, <80% and cerebral oxygenation <55%, respectively, during PB. Shorter duration of respiratory support, multigravida, multiparity, and maternal vitamin D deficiency were associated with higher %TSTPB. Multigravida, shorter duration on respiratory support, apnea of prematurity, and resuscitation at birth were associated with hypoxia during PB., Conclusions: The majority of very preterm infants exhibited PB when they were off respiratory support and considered clinically stable. The time spent in PB was very variable between infants and was associated with significant hypoxia in some infants. Fewer days spent on respiratory support was associated with both increased frequency and severity of PB. However, the potential contribution of PB to neurocognitive outcomes remains uncertain and warrants further investigations., (© 2022 Wiley Periodicals LLC.)

Background: Repetitive surges in heart rate (HR) at respiratory event termination underpin the altered autonomic HR control associated with sleep disordered breathing (SDB). As children born preterm are at greater risk of adverse cardiovascular outcomes, we aimed to determine whether the HR response to obstructive respiratory events was elevated compared to term-born children., Methods: Fifty children (3-12 years) born preterm, were matched for SDB severity, age and gender with term born children. Multilevel modelling determined the effect of preterm birth and arousal on HR changes between a 10s baseline to the latter half of respiratory events and 15s post event during NREM and REM., Results: 1203 events were analysed (NREM: term 380; preterm 383; REM: term 207; preterm 233). During NREM fewer events terminated in arousal in the preterm compared with term group (preterm 68%; term 84%; χ 2  = 27.2, p < 0.001). There were no differences in REM. During NREM, HR was lower in the preterm group at all event phases, with and without associated arousals (P < 0.01 for all). % change in HR from baseline to post event was higher in the preterm compared with term group (preterm: median 23% IQR (12%,34%); term: 18% (10%,29%); p < 0.01) and late event to post event (preterm: 30% (21%, 32%); term 28% (20%,39%); p < 0.01) in events associated with arousals., Conclusion: The greater magnitude of surges in HR following respiratory events terminating with arousal in preterm born children, although small, occur repeatedly throughout the night and may contribute to adverse cardiovascular outcomes, although further studies are required., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Study Objectives: Obstructive sleep disordered breathing (SDB), has adverse neurocognitive and behavioral sequelae in children, despite conventional measures of sleep disruption being unaffected. There is growing evidence that sleep spindles may serve as a more sensitive marker of sleep quality. We investigated the relationship between sleep spindles and sleep fragmentation and neurocognition across the spectrum of SDB severity in children., Methods: Children 3-12 years old referred for clinical assessment of SDB and age matched control children from the community were recruited and underwent polysomnography. Sleep spindles were identified manually during N2 and N3 sleep. Spindle activity was characterised as spindle number, density (number of spindles/h) and intensity (spindle density x average spindle duration). Children completed a battery of tests assessing global intellectual ability, language, attention, visuospatial ability, sensorimotor skills, adaptive behaviors and skills and problem behaviors and emotional difficulties., Results: Children were grouped into control, Primary Snoring, Mild OSA and Moderate/severe OSA, N = 10/group. All measures of spindle activity were lower in the SDB groups compared to the Control children and this reached statistical significance for Mild OSA (p < 0.05 for all). Higher spindle indices were associated with better performance on executive function and visual ability assessments but poorer performance on auditory attention and communication skills. Higher spindle indices were associated with better behavior., Conclusion: The reduced spindle activity observed in the children with SDB, particularly Mild OSA, indicates that sleep micro-architecture is disrupted and that this disruption may underpin the negative effects of SDB on attention, learning and memory., (Crown Copyright © 2022. Published by Elsevier B.V. All rights reserved.)

Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Periodic breathing (PB) is common in preterm infants. We aimed to characterize the contribution of ventilatory control instability to the presence and persistence of PB longitudinally., Methods: Infants born between 28 and 32 weeks of gestation were studied using daytime polysomnography at: 32-36 weeks postmenstrual age (PMA) (N = 32), 36-40 weeks PMA (N = 20), 3 months corrected age (CA) (N = 18) and 6 months CA (N = 19). Loop gain, a measure of sensitivity of the ventilatory control system, was estimated by fitting a mathematical model to ventilatory patterns associated with spontaneous sighs., Results: The time spent in PB decreased from 32-36 weeks PMA to 6 months CA (P = 0.005). Across all studies, studies with PB (N = 62) were associated with higher loop gain compared to those without PB (N = 23) (estimated marginal mean ± SEM: 0.445 ± 0.01 vs 0.388 ± 0.02; P = 0.020). A threshold of loop gain >0.415 (measured at 32-36 weeks PMA) provided a sensitivity of 86% and a specificity of 75% to detect the presence of PB at 6 months CA., Conclusions: The course of PB in preterm infants is related to changes in loop gain. Higher loop gain at 32-36 weeks PMA was associated with a greater risk of persistent PB at 6 months CA., Impact: The developmental trajectory of periodic breathing and its relationship to ventilatory control instability is currently unclear. Unstable ventilatory control is a determinant of periodic breathing in preterm infants up to 6 months corrected age. Infants who display greater ventilatory control instability at 32-36 weeks postmenstrual age may be at increased risk of persistent periodic breathing at 6 months corrected age. Assessment of ventilatory control stability may assist in the early identification of infants at risk of persistent periodic breathing and its potential adverse effects., (© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Background: Children with Down syndrome (DS) are at increased risk of obstructive sleep disordered breathing (SDB), which is associated with intermittent hypoxia and sleep disruption affecting daytime functioning. We aimed to examine the effects of treatment of SDB on sleep quality and daytime functioning in children with DS. Methods: Children with DS and SDB (n = 24) completed a baseline and follow-up overnight polysomnographic (PSG) study 22 ± 7 months (mean ± SD) later. Sleep micro-architecture was assessed using EEG spectral analysis, and parents completed a number of questionnaires assessing sleep, behavior, daytime functioning, and quality of life (QOL). Results: A total of nine children (38%) were treated. At baseline, the treated group had more severe SDB compared to the untreated group. SDB severity was significantly improved from 40.3 ± 46.9 events/h to 17.9 ± 26.9 events/h (p < 0.01) at follow up in children who were treated. There were no significant differences in sleep macro-architecture parameters from baseline to follow up in either the treated or untreated group. Sleep micro-architecture was not different between studies in the treated group, however this tended to improve in the untreated group, particularly in REM sleep. Daytime functioning and behavior were not different between the studies in either group, however, QOL improved after treatment. Conclusions: Our study identified that treatment of SDB improves severity of the disease as defined by PSG, and this was associated with parental reports of improved QOL, despite treatment having no demonstrable impacts on sleep quality, behavior, or daytime functioning.

Individuals with Prader-Willi syndrome (PWS) often have excessive daytime sleepiness and emotional/behavioral disturbances. The objective of this study was to examine whether daytime sleepiness was associated with these emotional/behavioral problems, independent of nighttime sleep-disordered breathing, or the duration of sleep. Caregivers of individuals with PWS (aged 3 to 25 years) completed the Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), and the parent version of the Developmental Behavior Checklist (DBC-P). Sleep adequacy was adjusted for age by computing sleep duration against age-specific recommendations. The associations between ESS-CHAD and the total DBC and its subscale scores were evaluated by linear regression, adjusted for sleep-related breathing difficulties, sleep adequacy, and body mass index (BMI). There were 54 responses for individuals with PWS (including 22 males) aged 4.4-24.0 (mean 12.5) years. Daytime sleepiness predicted a substantial proportion of the variance in total DBC-P scores in the unadjusted model (28%; β = 0.028; p < 0.001) and when adjusted for sleep adequacy, BMI, and sleep-related breathing difficulties (29%; β = 0.023; p = 0.007). This relationship was not moderated by BMI Z-scores, but the relationship was more prominent for children younger than 12 years than for children older than 12 years.Conclusions: These findings provide preliminary novel evidence that daytime sleepiness may drive the expression of emotional/behavioral disturbances, and should be explored as a potential modifiable risk factor for these disturbances in PWS, particularly pre-adolescent children., (© 2022. The Author(s).)

Prader-Willi Syndrome (PWS) is a rare genetic disorder associated with emotional/behavioral disturbances. These difficulties are well documented in the literature, but the positive attributes of these individuals are not described. Taking a strengths-based approach, the aim of this study was to describe the emotional/behavioral strengths and difficulties in children and young people with PWS from their parent caregivers' perspectives. Parent caregivers of 52 individuals with PWS aged 4-24 years (median = 12.1 years; including 22 males) completed the parent form of the Developmental Behavior Checklist (DBC-P), including its original two open-ended questions regarding positive traits. Prevalences of emotional/behavioral disturbances were comparable to those reported in previous literature: common behaviors of concern across studies being skin-picking (75%), impulsivity (69%), poor sense of danger (67%), lying (67%), and tantrums (54%). Total DBC-P scores showed that just over half (n = 28, 54%) had scores indicative of clinically significant behavior problems. However, thematic analysis of caregivers' written comments regarding their children's strengths resolved into three themes: warmth (94%), persistence (41%), and skills (41%). Warmth encompassed friendliness, happiness, and empathy. A strength-based approach to behavioral difficulties in PWS provides a more balanced view of the children and a more holistic foundation for interventions., (© 2022 Wiley Periodicals LLC.)

Study Objectives: Studies of sleep-disordered breathing (SDB) in children with Prader-Willi syndrome (PWS) have focused on early childhood and growth hormone (GH)-naïve children, but little is known about older children, including those on long-term GH therapy. This study aimed to describe the nature and prevalence of SDB in school-aged children with PWS in the growth hormone era., Methods: This retrospective single-center chart review included children aged 6-18 years with PWS who had overnight polysomnography not involving respiratory support over 5 years (2012-2017). The main outcome measures were the presence of obstructive sleep apnea, central sleep apnea, or hypoventilation defined by an elevated transcutaneous partial pressure of carbon dioxide (TcPCO 2 ) as per standard pediatric criteria., Results: Seventeen children (8 males; median age 11.6 years, range 6.6-16.1 years) were included. Fifteen demonstrated SDB of different types: central sleep apnea (18%), obstructive sleep apnea (24%), both obstructive and central sleep apnea (29%), or hypoventilation without obstructive or central sleep apnea (18%). Twelve (71%) children had evidence of hypoventilation. Those with hypoventilation had a higher central apnea-hypopnea index but no difference in the obstructive apnea-hypopnea index, age, sex, growth parameters, or the presence of scoliosis or sleep-related symptoms compared with those without hypoventilation., Conclusions: Sleep-related hypoventilation is common in school-aged children with PWS. The presence of central sleep apnea, including the quantification of central hypopneas, but not obstructive sleep apnea or clinical factors, predicted the presence of hypoventilation. Long-term polysomnography surveillance in children with PWS should include identification of central hypopneas and measurement of continuous pCO 2 ., Citation: Schaefer J, Davey MJ, Nixon GM. Sleep-disordered breathing in school-aged children with Prader-Willi syndrome. J Clin Sleep Med . 2022;18(4):1055-1061., (© 2022 American Academy of Sleep Medicine.)

Objective Hospital utilisation research is important in pursuing cost-saving healthcare models. Tonsillectomy is one of the most common paediatric surgeries and the most frequent reason for paediatric hospital readmission. This study aimed to report the government-funded costs of paediatric tonsillectomy in the state of Victoria, Australia, extrapolate costs across Australia, and identify the cost determinants. Methods A population-based longitudinal study was conducted with a bottom-up costing approach using linked datasets containing all paediatric tonsillectomy and tonsillectomy with adenoidectomy surgeries performed in the state of Victoria between 2010 and 2015. Results The total average annual cost of tonsillectomy hospitalisation in Victoria was A$21 937 155 with a median admission cost of A$2224 (interquartile range (IQR) 1826-2560). Inflation-adjusted annual tonsillectomy costs increased during 2010-2015 (P < 0.001), not explained by the rising number of surgeries. Hospital readmissions resulted in a total average annual cost of A$1 427 716, with each readmission costing approximately A$2411 (IQR 1936-2732). The most common reason for readmission was haemorrhage, which was associated with the highest total cost. The estimated total annual expenditure of both tonsillectomy and resulting readmissions across Australia was A$126 705 989. Surgical cost in the upper quartile was associated with younger age, male sex, lower socioeconomic status, surgery for reasons other than infection alone, overnight vs day case surgery, public hospitals and metropolitan hospitals. Surgery for obstructed breathing during sleep had the strongest association to high surgical cost. Conclusions This study highlights the cost of paediatric tonsillectomy and associated hospital readmissions. The study findings will inform healthcare reform and serve as a basis for strategies to optimise patient outcomes while reducing both postoperative complications and costs.

Background: Children with Down syndrome (DS) are at increased risk of sleep-disordered breathing (SDB), which is associated with intermittent hypoxia and sleep disruption affecting daytime functioning. We aimed to compare the impact of SDB on sleep quality in children with DS compared to typically developing (TD) children with and without SDB., Methods: Children with DS and SDB (n = 44) were age- and sex-matched with TD children without SDB (TD-) and also for SDB severity with TD children with SDB (TD+). Children underwent overnight polysomnography with sleep macro- and micro-architecture assessed using electroencephalogram (EEG) spectral analysis, including slow-wave activity (SWA, an indicator of sleep propensity)., Results: Children with DS had greater hypoxic exposure, more respiratory events during REM sleep, higher total, delta, sigma, and beta EEG power in REM than TD+ children, despite the same overall frequency of obstructive events. Compared to TD- children, they also had more wake after sleep-onset and lower sigma power in N2 and N3. The DS group had reduced SWA, indicating reduced sleep drive, compared to both TD groups., Conclusions: Our findings suggest that SDB has a greater impact on sleep quality in children with DS compared to TD children., Impact: SDB in children with DS exacerbates disruption of sleep quality, compared to TD children. The prevalence of SDB is very high in children with DS; however, studies on the effects of SDB on sleep quality are limited in this population. Our findings suggest that SDB has a greater impact on sleep quality in children with DS compared to TD children, and should be screened for and treated as soon as possible., (© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Rationale: Severe acute paediatric asthma may require treatment escalation beyond systemic corticosteroids, inhaled bronchodilators and low-flow oxygen. Current large asthma datasets report parenteral therapy only., Objectives: To identify the use and type of escalation of treatment in children presenting to hospital with acute severe asthma., Methods: Retrospective cohort study of children with an emergency department diagnosis of asthma or wheeze at 18 Australian and New Zealand hospitals. The main outcomes were use and type of escalation treatment (defined as any of intensive care unit admission, nebulised magnesium, respiratory support or parenteral bronchodilator treatment) and hospital length of stay (LOS)., Measurements and Main Results: Of 14 029 children (median age 3 (IQR 1-3) years; 62.9% male), 1020 (7.3%, 95% CI 6.9% to 7.7%) had treatment escalation. Children with treatment escalation had a longer LOS (44.2 hours, IQR 27.3-63.2 hours) than children without escalation 6.7 hours, IQR 3.5-16.3 hours; p<0.001). The most common treatment escalations were respiratory support alone (400; 2.9%, 95% CI 2.6% to 3.1%), parenteral bronchodilator treatment alone (380; 2.7%, 95% CI 2.5% to 3.0%) and both respiratory support and parenteral bronchodilator treatment (209; 1.5%, 95% CI 1.3% to 1.7%). Respiratory support was predominantly nasal high-flow therapy (99.0%). The most common intravenous medication regimens were: magnesium alone (50.4%), magnesium and aminophylline (24.6%) and magnesium and salbutamol (10.0%)., Conclusions: Overall, 7.3% children with acute severe asthma received some form of escalated treatment, with 4.2% receiving parenteral bronchodilators and 4.3% respiratory support. There is wide variation treatment escalation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)