Periodic limb movements during sleep in children with neuromuscular disease or cerebral palsy - An important potential contributor to sleep-related morbidity.

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.

The surge in heart rate and blood pressure at respiratory event termination is dampened in children with down syndrome.

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.

Effect of sleep disordered breathing severity in children with Down syndrome on parental wellbeing and social support.

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.

Improvement in quality of life with continuous positive airway pressure outweighs the treatment burden in children with obstructive sleep apnea.

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.

Is there a role for repeating the multiple sleep latency test across childhood when initially non-diagnostic?

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.

Sleepy Kids: are the current diagnostic criteria for multiple sleep latency tests enough?

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.

Follow-up of children with Down syndrome and sleep disordered breathing and the effects of treatment on actigraphically recorded sleep, quality of life, behaviour, and daytime functioning.

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.

Sleep spindles are reduced in children with Down syndrome and sleep-disordered breathing.

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.

Cortical grey matter changes, behavior and cognition in children with sleep disordered breathing.

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.

Assessing sleep in children with Down syndrome: Comparison of parental sleep diaries, actigraphy and polysomnography.

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.

Sleep disordered breathing improvement prevents worsening of autonomic dysfunction in children with Down syndrome.

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.

The effects of sleep disordered breathing on sleep spindle activity in children and the relationship with sleep, behavior and neurocognition.

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.

Effects of Treatment of Sleep Disordered Breathing on Sleep Macro- and Micro-Architecture in Children with Down Syndrome.

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.

Sleep-disordered breathing in school-aged children with Prader-Willi syndrome.

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 (TcPCO2) 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 pCO2. 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.

Sleep-disordered breathing and sleep macro- and micro-architecture in children with Down syndrome.

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.

Smartphone videos to predict the severity of obstructive sleep apnoea.

OBJECTIVE: Diagnosis of obstructive sleep apnoea (OSA) is made on overnight polysomnography (PSG). Given the widespread availability of smartphone video technology, we aimed to develop and test a standardised scoring system for smartphone videos and compare these scores to PSG results. METHODS: Children aged 1-16 years undergoing PSG for suspected OSA were included. Parents were asked to take 1-2 min videos of the breathing they were concerned about. Videos were scored using a newly developed and tested tool on five components: inspiratory obstructive noises (1-4), presence of obstructive events (0-1), increased work of breathing (0-1), mouth breathing (0-1) and neck extension (0-1). Video scores and the Obstructive Apnoea Hypopnoea Index (OAHI) were compared using Spearman correlation. Sensitivity, specificity, positive predictive value and negative predictive value were calculated for different cut-off scores to achieve the best results. RESULTS: Videos from 43 children (28 men (65.1%), median age 5.7 years (range 2.6-14.0 years), median OAHI 3.8/hour (range 0-82 events/hour) were included. Nine children (20.9%) had a video score of <3, all of whom had an OAHI of ≤5 events/hour. For a video score of ≥3, sensitivity was 100%; specificity was 36%; positive predictive value was 53%; and negative predictive value 100% for moderate to severe OSA (OAHI>5 events/hour) . CONCLUSION: We have developed and validated a simple clinical tool (the Monash Obstructive Sleep Apnoea Video Score) to quantify abnormalities in breathing seen on short video recordings made on a smartphone. A low score rules out moderate-severe OSA and may be valuable in the triage of children with symptoms of OSA.

Paediatric CPAP in the digital age.

OBJECTIVES: Adherence to Continuous Positive Airway Pressure (CPAP) in children can be challenging. Advancements in CPAP technology have potential to influence adherence. The aim of this study was to compare adherence rates of children with obstructive sleep apnoea (OSA) initiated on autotitrating CPAP (APAP) with remote modem monitoring compared to a cohort started on fixed pressure CPAP alone. METHODS: Children aged over 3 years starting APAP at our centre between February 2017 and February 2020 were included. Therapy data was obtained for the initial 90 days. Data was compared to a cohort of children started on CPAP between July 2004 and September 2008. RESULTS: A total of 61 patients with a median age of 14.3 years formed the APAP group, and were significantly older than the CPAP group who had a median age of 8.6 years (p = 0.02). Co-morbid conditions were present in 51% compared with 69% in the earlier cohort (p = 0.11). No significant difference was found in any adherence parameters between the groups. The value closest to achieving a significant difference was hours used per day used, with an median of 5.2 h in the CPAP group compared with 7.0 h in the APAP group (p = 0.07). Two-way ANOVA including age group (above or below 13 years) showed that both age group and treatment group (CPAP vs APAP) were significantly associated with a difference in adherence (F = 4.41, p = 0.006), with mean hours used on days used being highest in the APAP group aged under 13 years. However no significant interaction was found between age and treatment group. CONCLUSION: Despite the convenience for patients with outpatient initiation and ability to achieve optimal pressures quickly and remotely, our results show no improvement in adherence using APAP with remote monitoring, with the possible exception of children aged under 13 years. A large randomized controlled trial would be required to confirm these findings.

Factors associated with referral for polysomnography in children with Down syndrome.

OBJECTIVES: Children with Down syndrome (DS) are recommended to undergo polysomnography (PSG) by the age of four years due to the high prevalence of obstructive sleep apnea (OSA) in this group, but compliance is incomplete. To further understand referral patterns for PSG in this condition, we aimed to compare demographics, PSG results, OSA severity, behavior, daytime functioning and quality of life (QOL) between children with DS referred for sleep testing and those recruited from the community. STUDY DESIGN: Children 3-19 years with DS was included: 20 referred clinically for assessment of OSA and 24 volunteers from the community. Demographic and anthropometric data, PSG parameters, sleep-related symptoms and QOL, behavior and daytime functioning were compared between groups. RESULTS: OSA severity did not differ between groups: 50% of the clinical and 42% of the community group had moderate/severe OSA. The clinical group had a higher weight z-score, BMI z-score, waist and hip circumference and neck-to-waist ratio. Questionnaire scores for daytime functioning, behavior and QOL were not different between groups. CONCLUSIONS: Despite not being referred for clinical sleep assessment, 42% of children with DS recruited from the community had moderate/severe OSA. There was no difference in the QOL, behavior, daytime functioning and sleep symptoms questionnaires although the clinical group had a higher BMI-Z score and overt signs of obesity. These findings underscore the importance of PSG screening of all children with DS.

Nocturnal dipping of heart rate is impaired in children with Down syndrome and sleep disordered breathing.

BACKGROUND: Children with Down syndrome (DS) are at increased risk for sleep disordered breathing (SDB), which can have adverse effects on the cardiovascular system. In adults with SDB, nocturnal dipping of heart rate (HR) and blood pressure (BP) is reduced, and this is associated with an increased risk of future cardiovascular events. We aimed to compare nocturnal dipping of HR and pulse transit time (PTT) (a surrogate inverse measure of BP change) in children with DS and SDB to those of typically developing (TD) children with and without SDB. METHODS: 19 children with DS (3-18 years) were age and sex matched with 19 TD children without SDB (TD-) and with 19 TD children with matched severity of SDB (TD+). Nocturnal dipping was assessed as the percentage change in HR and PTT from wake before sleep onset to total sleep, N2, N3 and REM sleep across the night and to the first cycle of sleep. RESULTS: Children with DS exhibited reduced nocturnal dipping of HR during total sleep, N2, N3 and REM sleep and increased PTT (reduced BP dipping) in N2 sleep. Fewer children with DS exhibited a greater than 10% fall in HR between wake and N2 or REM sleep compared to TD+ children. CONCLUSIONS: Our findings demonstrate significantly reduced nocturnal dipping of HR in children with DS compared to TD children matched for SDB severity, suggesting SDB has a greater cardiovascular effect in these children. Further studies are required to fully understand the mechanisms involved and to assess if treatment of SDB improves nocturnal dipping.