Delivery of average assured pressure support (AVAPS) through tracheostomy in paediatric patients.

Average volume-assured pressure support (AVAPS) mode has been available since 2009 and allows the ventilator to deliver a constant pre-set tidal volume by automatically adjusting the inspiratory pressures within a set range. Data in AVAPS mode use is limited in both paediatric populations, and in patients who are ventilated through a tracheostomy. This case series reports on the successful use of AVAPS mode in four paediatric patients with tracheostomy ventilation.

Understanding the ongoing learning needs of Australian paediatricians: Evaluation of a pilot paediatric video teaching programme.

AIM: The purpose of this study was to evaluate whether pre-recorded video-based lectures (VBLs) covering a range of paediatric topics are an acceptable means of providing ongoing education for consultant and trainee paediatricians in Australia. METHODS: Previous participants (paediatric consultants and junior medical officers) of a neurology outreach teleconference programme offered by a paediatric neurologist between 2017 and 2020 were invited to participate in a multi-specialty pre-recorded video-based education programme. Acceptability was explored by assessing relevance, likelihood of utilising VBL's in the future, uptake and learning activity preferences. The impact of VBLs on confidence, currency and practice was also explored. Additional data including topics of interest, preferred video format, duration, viewing method and frequency of delivery were captured, to better understand participant preferences to inform future efforts. RESULTS: A total of 135 consented; 116 returned baseline; 94 returned follow-up surveys. Preferred learning activities included a live/interactive component. Videos were considered relevant. Preferences for pre-recorded videos improved from ninth to sixth most preferred learning activity post-intervention. VBL convenience and accessibility were valued. Practice was altered in: approach to management, use of treatments, confidence in decision-making, and discussion with families and patients. The average view duration was 16 min. Longer videos yielded slightly lower audience retention rates. For future offerings, the majority endorsed a preference for a 'mixed' video format and duration of 20-40 min, offered monthly. CONCLUSION: Video-based medical education is an appealing and sustainable alternative, given the convenience of unrestricted accessibility, in meeting ongoing learning needs of Australian paediatricians and trainees.

Three generations of a family diagnosed with congenital central hypoventilation syndrome: A case series.

Congenital central hypoventilation syndrome (CCHS) is an autosomal dominant disorder characterized by alveolar hypoventilation and autonomic dysregulation secondary to mutations of the PHOX2B genes. We present five cases from three generations within the same family with varying degrees of phenotypic expression of the PHOX2B gene mutation. The cases were diagnosed following identification of CCHS in index case at birth. This case series underscores the importance of screening first-degree relatives of individuals with confirmed CCHS and alerts the clinicians to maintain a high degree of suspicion in asymptomatic family members given the high degree of phenotypic variability of CCHS.

Pediatric Average Volume Assured Pressure Support.

Average volume assured pressure support (AVAPS) is a modality of non-invasive ventilation that enables the machine to deliver a pre-set tidal volume by adjusting the inspiratory pressure support within a set range. Data on its use in the pediatric population are limited to case reports and single centre case series. This article reviews paediatric data on use of AVAPS and highlights the need for validation to help develop specific guidelines on use of AVAPS in children.

Convergent validity of the child behavior checklist sleep items in children with moderate to severe traumatic brain injury.

AIM: The Child Behavior Checklist (CBCL) includes several sleep items. We aimed to examine the convergent validity of CBCL sleep scores with validated sleep measures, and to explore their functional correlates. METHODS: This cross-sectional study included 44 children with moderate to severe TBI, aged 6-15 years. Parents completed the CBCL and Sleep Disturbance Scale for Children (SDSC), and children wore actigraphy watches. RESULTS: We found significant, albeit differential, associations between CBCL and SDSC sleep scores. Specifically: (i) "trouble sleeping" with SDSC total score, (ii) "trouble sleeping" and "nightmares" with SDSC initiating and maintaining sleep, (iii) "talks/walks in sleep" with SDSC arousal, and (iv) "overtired," "sleeps more" and CBCL sleep composite with SDSC excessive somnolence. The CBCL item "sleeps less" was the only significant predictor of functioning; children who slept less had lower social competence. No associations were found between CBCL sleep scores and actigraphy. CONCLUSIONS: The CBCL does not provide a comprehensive assessment of sleep disturbances in children with moderate to severe TBI. Nevertheless, certain CBCL sleep items demonstrate initial convergent validity with subscales of the SDSC assessing select types of sleep disturbances. The CBCL may be useful in research and clinical situations when administration of more comprehensive assessment sleep tools is not viable.

Neurocognitive outcomes of children with sleep disordered breathing: A systematic review with meta-analysis.

Children with sleep disordered breathing (SDB) are at an increased risk of neurocognitive impairments. This systematic review with meta-analysis aims to 1) establish whether SDB differentially impacts various neurocognitive domains (intelligence, attention/executive functions, memory, visual spatial skills, and language) and 2) examine the effects of several moderating factors. Database searches, conducted according to the PRISMA guidelines, identified 77 studies that met pre-determined criteria, 63 of which were included in the meta-analysis. Most studies were of moderate to high quality. Children with SDB had significant impairments in all cognitive domains, albeit of different magnitude. The largest impairments were found in intelligence (verbal and overall). With respect to severity of SDB, neurocognitive deficits were evident in children with primary snoring (PS) as well as in children with obstructive sleep apnea (OSA). Other moderators: higher body mass index, younger age at testing, using questionnaires rather than polysomnography, and employing a control group instead of normative data, related to poorer neurocognitive outcomes in select domains. Overall, our study provides robust evidence of multiple neurocognitive impairments in children with SDB, with no evidence of sparing in children with PS. The findings of our study provide impetus for research and interventions for children with SDB across all severities.

SIDS is associated with prenatal drug use: a meta-analysis and systematic review of 4 238 685 infants.

OBJECTIVE: To conduct a meta-analysis to determine the association between prenatal drug exposure and risk of sudden infant death syndrome (SIDS). DESIGN: Studies were searched using PubMed, Medline and Embase and restricted to English, with no publication date limit. Selected studies included published cohort, population or case studies comparing the incidence of SIDS among drug-exposed with drug-free controls. This study was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and Meta-Analysis of Observational Studies in Epidemiology guidelines. Data were pooled using a random-effects model to evaluate risk ratios (RR). SETTING: High-income countries. PATIENTS: Children with a history of prenatal drug exposure. INTERVENTIONS: None. MAIN OUTCOME MEASURES: RR of SIDS between drug-exposed and control infants. RESULTS: Sixteen studies (36 730 infants with any prenatal drug exposure, 21 661 exposed to opioids, 21 571 exposed to cocaine, 5031 exposed to methadone compared with 4 201 955 with no exposure). Any prenatal drug exposure was associated with an increased crude risk of SIDS (RR 7.84, 95% CI 5.21 to 11.81). Prenatal opioid exposure had the highest associative crude risk of SIDS (RR 9.76, 95% CI 5.28 to 18.05), followed by methadone (RR 9.52, 95% CI 4.60 to 19.70) and cocaine (RR 4.40, 95% CI 2.52 to 7.67). Increased crude risk persisted after adjusting for socioeconomic factors (RR 4.24, 95% CI 1.39 to 12.88). The incidence of SIDS for this cohort decreased between 1972 and 2020 but remained significantly higher than controls. CONCLUSION: Exposure to any drug of dependency during pregnancy is associated with an increased risk of SIDS after controlling for socioeconomic factors. Further study to evaluate mechanisms and contribution of other confounders (eg, smoking) is warranted.

Daytime predictors of nocturnal hypercapnic hypoventilation in children with neuromuscular disorders.

OBJECTIVES: To examine objective daytime predictors of nocturnal hypercapnic hypoventilation (NHH) and identify a forced vital capacity (FVC) z-score cut off that predicts NHH using the 2012 Global Lung Function Initiative (GLI) reference equations in pediatric neuromuscular patients. DESIGN: Single-centre retrospective medical record review. SETTING: Tertiary pediatric hospital in Australia. PATIENTS: Children (<18 years old) with a neuromuscular disorder (NMD) who had a diagnostic sleep study over a 5-year period. RESULTS: Fifty children were included, median age 11.9 years (interquartile range [IQR]: 4.5-14.3). The majority of children had a diagnosis of Duchenne Muscular Dystrophy (32%). NHH was diagnosed in 18 children (36%). Multivariate logistic regression analysis performed for the entire cohort confirmed a statistically significant association between NHH and scoliosis (odds ratio [OR]: 3.3, p = 0.03), but not age (OR: 1.01, p = 0.26), body mass index z-score (OR: 0.86, p = 0.26) or use of a wheelchair for mobility (OR: 1.25, p = 0.72). For the subset of 29 children who had spirometry testing (median age 12.9 years [IQR: 10.2-14.3]), FVC z-score was the only statistically significant predictor of NHH (OR: 0.45, p = 0.02). NHH was predicted by an FVC z-score <-3.24 (sensitivity 78%, specificity 73%), or FVC <60% predicted (sensitivity 78%, specificity 73%). There was a strong positive correlation between FVC and forced expiratory volume in 1 s z-scores (rp = 0.98, p = 0.00) and FVC and peak expiratory flow  z-scores (rp = 0.72, p = 0.00). CONCLUSION: Children with a NMD and scoliosis or a lower FVC z-score have increased odds of having NHH.

The persistence of sleep disturbance and its correlates in children with moderate to severe traumatic brain injury: A longitudinal study.

OBJECTIVES: The primary aim was to examine whether sleep disturbances persist in children in the chronic stage of recovery from moderate or severe traumatic brain injury (TBI). The secondary aim was to examine whether memory difficulties and/or other previously identified factors relate to sleep disturbances in children with moderate to severe TBI. METHODS: This longitudinal study included 21 children with moderate to severe TBI, 8-18 years old, recruited from an urban tertiary paediatric specialised brain injury rehabilitation unit. Participants were seen 5 years and again 7 years post-injury, on average. Sleep disturbances were assessed with Sleep Disturbance Scale for Children (SDSC). Correlates that were considered included indicators of TBI severity, and questionnaires assessing everyday memory, fatigue, internalizing and externalizing behaviors and pain intensity. RESULTS: The SDSC scores of children with moderate to severe TBI indicated greater disturbances in initiating and maintaining sleep, arousal, sleep-wake transition, and excessive somnolence relative to the norms, at follow-up. The mean SDSC scores and the number of participants with subclinical to clinical sleep disturbances on the SDSC remained unchanged from baseline to follow-up. At follow-up, the SDSC initiating and maintaining sleep, and excessive somnolence scales were associated with poorer everyday memory and greater fatigue. CONCLUSIONS: Children with moderate to severe TBI experience ongoing sleep disturbances for years post-injury. Greater sleep disturbances are associated with worse functional outcomes. Further research into sleep disturbances and development of treatments is important, as it could improve the outcomes of children with TBI.

Children who sustained traumatic brain injury take longer to fall asleep compared to children who sustained orthopedic injuries: actigraphy findings.

Objective:Primary: to examine objective sleep outcomes in children who sustained moderate to severe traumatic brain injury (TBI). Secondary: to examine the relation of objective sleep with subjective sleep, fatigue, and injury variables.Setting: A single tertiary brain injury unit.Participants: Children (5-15 years) with moderate to severe TBI (n = 23) or orthopedic injury (OI; n = 13).Design: Cohort study.Measures: Primary: objective sleep measure (actigraphy watch). Secondary: subjective sleep measure (questionnaire), fatigue questionnaire, and injury variables.Results: On actigraphy, children with TBI had longer sleep onset latency compared to children with OI. On the sleep questionnaire, children with TBI obtained higher scores for total sleep disturbance, initiating and maintaining sleep, and excessive somnolence. On the fatigue questionnaire, greater difficulties were found for total, sleep/rest, and cognitive fatigue for the TBI group. In the TBI group, actigraphy data did not correlate with sleep questionnaire, fatigue, or injury variables.Conclusion: Our study showed evidence of objective and subjective sleep disturbance in children with moderate to severe TBI, but these two types of sleep measures were not related. It is possible that distinct mechanisms underpin objective and subjective sleep disturbance, which may require different interventions.

Average volume-assured pressure support vs conventional bilevel pressure support in pediatric nocturnal hypoventilation: a case series.

STUDY OBJECTIVES: Average volume-assured pressure support (AVAPS) is a modality of noninvasive ventilation that provides a targeted tidal volume by automatically adjusting the inspiratory pressure support within a set range. Pediatric studies evaluating the efficacy of AVAPS in treating nocturnal hypoventilation are confined to case reports. The aim of this study was to compare AVAPS to conventional bilevel positive airway pressure (BPAP) support in improving hypercarbia in a cohort of pediatric patients with nocturnal hypoventilation. METHODS: Retrospective review of patient records at an established tertiary pediatric sleep laboratory over a 6-year period. Ventilatory and sleep study parameters from AVAPS and conventional BPAP titration studies were compared. AVAPS was used only if hypoventilation was not controlled using conventional BPAP. Inspiratory pressures, tidal volumes, and adherence were downloaded on final titrated ventilatory settings. Comparisons were made using paired t test. RESULTS: A total of 19 patients (11 boys, 8 girls; median age 10.5 years, range 1 to 20 years) were identified. Diagnoses included neuromuscular disease (n = 9), obstructive hypoventilation (n = 5), parenchymal lung disease (n = 4), and congenital central hypoventilation syndrome (n = 2). AVAPS demonstrated significant improvement in peak (P = .009) and mean (P = .001). Transcutaneous CO2 parameters compared to conventional bilevel. Oxygenation on AVAPS showed positive trend but did not reach statistical significance. AVAPS delivered higher tidal volumes (P = .04) using similar pressures. There was no statistically significant difference in obstructive apnea-hypopnea index, respiratory arousal index, sleep efficiency, and adherence between AVAPS and conventional BPAP. CONCLUSIONS: AVAPS was an effective alternative to conventional BPAP in improving hypercarbia in our selective cohort of pediatric patients. Prospective, longitudinal studies are needed to evaluate the benefits of AVAPS feature in the pediatric population.

Propranolol's effects on the sleep of infants with hemangiomas: A prospective pilot study.

OBJECTIVE: To assess propranolol's impact on sleep when used in infants and toddlers with infantile hemangioma (80% under 6 months old). METHODS: Parents and caregivers of infants and toddlers with infantile hemangioma presenting to a tertiary pediatric hospital's dermatology clinic and assessed by their dermatologist as requiring propranolol treatment were invited to participate. All participants completed an extended version of the Brief Infant Sleep Questionnaire (BISQ) prior to propranolol treatment initiation, which acted as the control, and 5 weeks after treatment commencement. Objective data were gathered through actigraphy, which utilizes a small wristwatch-like device that measures sleep-wake patterns, for 1 week prior to initiation and again 5 weeks after commencement. BISQ responses and actigraphy values from the two time points were compared. RESULTS: 55 infants and toddlers (aged 0-2.8 years, 80% under 6 months) were included. Sleep was reported as only a minor problem by most parents 5 weeks after starting propranolol (P = .049). Subgroup analysis of 45 infants <6 months old showed no significant difference in sleep while taking propranolol. Whole cohort BISQ data analysis showed a statistically significant increase in night-time sleep (P = .024), and a decrease in the number (P = .003) and duration of daytime naps (P = .025) following commencement of propranolol. Actigraphy data completed in 10 infants showed no significant difference in sleep quality before and 5 weeks after commencing propranolol. CONCLUSION: Propranolol did not significantly impair sleep quality and pattern in our cohort of infants and toddlers with infantile hemangioma. Most parents considered the impact on sleep to be only a minor problem.

Fatigue in Children With Moderate or Severe Traumatic Brain Injury Compared With Children With Orthopedic Injury: Characteristics and Associated Factors.

OBJECTIVE: To characterize fatigue in children with moderate or severe traumatic brain injury (TBI) and to identify associated factors. SETTING: Urban tertiary pediatric healthcare facility. PARTICIPANTS: Children aged 5 to 15 years with a moderate TBI (n = 21), severe TBI (n = 23), or an orthopedic injury (OI; n = 38). DESIGN: Case-control study. MAIN MEASURES: (i) Pediatric Quality of Life Inventory Multidimensional Fatigue Scale (PedsQL-MFS), completed by parents and children; (ii) Sleep Disturbance Scale for Children, completed by parents. Data on injury-specific factors and other factors of interest were also collected. RESULTS: The 2 TBI groups did not differ on any of the fatigue outcomes (child or parent ratings). Relative to the OI group, parents rated children in both TBI groups as experiencing greater fatigue. However, on self-ratings, only children with moderate TBI endorsed greater fatigue. Sleep was commonly associated with fatigue, with child sleep disturbance and child sleep hygiene associated with parent-rated and self-rated child fatigue, respectively. Individually, there were no cases of "normal" fatigue coinciding with severe sleep disturbance. However, there were several cases of severe fatigue coinciding with normal sleep. Additional factors associated with fatigue were older age at injury, longer time since injury, and/or greater internalizing difficulties. CONCLUSION: Children with moderate and severe TBI experience greater fatigue than OI controls. Parent and child ratings of fatigue appear to be associated with different factors, indicating that fatigue management may require a broad range of treatments.

A feasibility and acceptability study of cognitive behavioural treatment for insomnia in adolescents with traumatic brain injury: A-B with follow up design, randomized baseline, and replication across participants.

Difficulties falling asleep or staying asleep (symptoms of insomnia) are common following paediatric traumatic brain injury (TBI). Yet, interventions to treat insomnia in this population have not yet been reported. This single-case series examined the feasibility and acceptability of cognitive behavioral treatment for insomnia (CBT-I) for adolescents (n = 5, aged 11-13 years) with TBI, and explored changes in sleep and fatigue post-treatment. Adolescents were randomly assigned to two conditions: a 7- or 14-days baseline, followed by 4 weeks of manualised CBT-I delivered individually. To assess feasibility and acceptability we compared recruitment and retention rates, and questionnaire scores to a-priori set criteria. We explored treatment efficacy and functional gains in sleep and fatigue from baseline to follow-up using structured visual analysis of time-series graphs, and reliable change indices or changes in clinical classification. Feasibility and acceptability indicators met a-priori criteria, but therapists noticed limited adolescent engagement in sessions. Clinically significant improvements were found in sleep, in 3 out of 4 cases, and fatigue, in all cases. Our study provides preliminary evidence that CBT-I is feasible for insomnia treatment in adolescents with TBI and provides directions for development of future treatment studies.

Real-world respiratory and bulbar comorbidities of SMA type 1 children treated with nusinersen: 2-Year single centre Australian experience.

AIM: To describe the respiratory and nutritional supportive care and hospitalisations required in the real-world scenario in children with SMA type 1 treated with nusinersen. METHODS: Single-centre observational cohort study of children with SMA1 commencing nusinersen from November 2016 to September 2018. Motor, respiratory and nutritional clinical characteristics and management are described from initiation of nusinersen for a minimum of two years. RESULTS: Nine children (5 females, 4 males), median age 10.7 months (range 2.7-181.2) commenced treatment with nusinersen and outcomes were assessed over a total of 270.5 patient months and 209 hospital admissions. Supportive care in newly-diagnosed patients (n = 7) included gastrostomy insertion (n = 4) and commencement of noninvasive ventilation (n = 4) at an average of 8.3 and 4.5 months after diagnosis, respectively. The annualised hospitalisation rate was 9.3/patient/year, average length of stay (LOS) of 3.3 days (SD = 5.6). Children with two SMN2 copies required more gastrostomies (p < 0.05) and had more frequent admissions (p < 0.05). Number of total admissions halved from the first to the second year of treatment in all patients (p < 0.005). INTERPRETATION: Children with treated SMA1 experienced considerable respiratory and bulbar comorbidities, necessitating substantial respiratory and nutritional supportive care. Proactive respiratory and nutritional surveillance and management is essential in SMA1 patients treated with nusinersen.

Non-invasive home ventilation using the average volume assured pressure support feature in an infant with severe bronchopulmonary dysplasia and chronic respiratory failure.

INTRODUCTION: While majority of infants with bronchopulmonary dysplasia (BPD) can be discharged home without low flow oxygen or on supplemental low flow oxygen, some require long term home mechanical ventilation. CASE PRESENTATION: We present a case of an extremely premature infant with severe bronchopulmonary dysplasia who was successfully managed at home on a new feature of non-invasive ventilation called average volume assured pressure support (AVAPS) without the need for tracheostomy. The AVAPS feature enables the machine to deliver a consistent tidal volume by automatically adjusting the inspiratory pressure within a set range. CONCLUSION: The use of AVAPS feature in our case improved ventilation as indicated by a more stable gas exchange profile, making home non-invasive ventilation a more practicable method of managing severe BPD in this infant.

Behavioral interventions for sleep disturbances in children with neurological and neurodevelopmental disorders: a systematic review and meta-analysis of randomized controlled trials.

STUDY OBJECTIVES: Sleep disturbances are common and associated with negative functional and health consequences in children with neurological and neurodevelopmental disorders (NNDDs) and represent an important potential target for behavioral interventions. This systematic review examined the efficacy of behavioral sleep interventions (BSIs) for children with NNDDs and comorbid sleep disturbances. METHODS: A systematic search of MEDLINE, EMBASE, PsychINFO, and CENTRAL was conducted in April 2019. Randomized controlled trials (RCTs) of BSI for children with NNDDS were included. Meta-analysis and GRADE quality ratings were performed on sleep and secondary functional outcomes (cognition, academics, and behavior). RESULTS: Nine RCTs were identified (n = 690; Mage = 8.39 ±â€…2.64years; 71.11% male). The quality of the evidence was predominantly rated as moderate. Posttreatment improvements in sleep were found on self-reported sleep disturbances (total sleep disturbance [standardized mean difference, i.e. SMD = 0.89], night wakings [SMD = 0.52], bedtime resistance [SMD = 0.53], parasomnias [SMD = 0.34], sleep anxiety [SMD = 0.50]) and self-reported sleep patterns (sleep duration [SMD = 0.30], sleep onset duration [SMD = 0.75]) and (2) objectively measured actigraphic sleep patterns (total sleep time [MD = 18.09 min; SMD = 0.32], sleep onset latency [MD = 11.96 min; SMD = 0.41]). Improvements in sleep (self-reported, not actigraphy) were maintained at follow-up, but few studies conducted follow-up assessments resulting in low-quality evidence. Reduction in total behavioral problems (SMD = 0.48) posttreatment and attention/hyperactivity (SMD = 0.28) at follow-up was found. Changes in cognition and academic skills were not examined in any studies. CONCLUSIONS: BSIs improve sleep, at least in the short term, in children with NNDDs. Benefits may extend to functional improvements in behavior. More rigorous RCTs involving placebo controls, blinded outcome assessment, longer follow-up durations, and assessment of functional outcomes are required.

Comparison of nocturnal pulse oximetry with polysomnography in children with sleep disordered breathing.

PURPOSE: Polysomnography (PSG) is the gold standard in the diagnosis of obstructive sleep apnea (OSA). However, due to high cost and limited availability, this is difficult to access and often delayed. To evaluate the reliability of overnight oximetry as a screening tool for OSA diagnosis. METHOD: All children suspected of OSA who underwent an overnight oximetry and subsequent PSG between January 2014 and April 2016 were studied retrospectively. The "McGill oximetry scoring" was compared with OSA diagnosis as per the American Association of Sleep Medicine. RESULTS: A total of 110 patients had both oximetry and PSG. Sixty-one children (56%) had normal oximetry, whereas 30 (27%) had McGill grade 2 and 19 (17%) had McGill 3 and 4. Sixty-four percent (64%) of children with normal oximetry had a normal PSG. Seventy percent (70%) of children with McGill 2 had either a normal or mild OSA on PSG. All the children with McGill 3 and 4 had moderate/severe OSA by PSG. The overall sensitivity and specificity of oximetry in identifying OSA were 63% and 78%, respectively, and the positive and negative predictive values (PPV and NPV) were 78% and 64%, respectively. The sensitivity and specificity of McGill 3 and 4 in diagnosing moderate/severe OSA on PSG were 59% and 100%, respectively, and the PPV and NPV were 100% and 78%, respectively. CONCLUSION: Overnight oximetry provides satisfactory diagnostic performance in detecting moderate and severe OSA; however, a normal or McGill 2 score does not rule out OSA and a PSG is required for diagnosis.

Nasal mask average volume-assured pressure support in an infant with congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by alveolar hypoventilation and autonomic dysregulation secondary to mutations of the PHOX 2B genes. Treatment consists of assisted ventilation using positive pressure ventilators via tracheostomy, bi-level positive airway pressure (BPAP), negative pressure ventilators, or diaphragm pacing. Previous case reports have highlighted early use of nasal non-invasive BPAP use in infants with CCHS. We present a case of a 10-month-old infant who was successfully managed on a new feature of non-invasive ventilation called average volume assured pressure support (AVAPS) without the need for tracheostomy. The AVAPS feature enables the machine to automatically adjust the inspiratory pressures to deliver a constant targeted tidal volume. This feature enabled a better control of ventilation as indicated by a more stable transcutaneous carbon dioxide profile compared to conventional nasal non-invasive BPAP, making non-invasive ventilation a more accessible method of managing sleep hypoventilation in CCHS.

Quantitative assessment of nocturnal neural respiratory drive in children with and without obstructive sleep apnoea using surface EMG.

NEW FINDINGS: What is the central question of this study? Recent studies have suggested potential utility of non-normalized respiratory muscle EMG as an index of neural respiratory drive (NRD). Whether NRD measured using non-normalized surface EMG of the lateral chest wall overlying the diaphragm (sEMGcw) recorded during nocturnal clinical polysomnography can differentiate children with and without obstructive sleep apnoea (OSA) is not known. What is the main finding and its importance? Non-normalized sEMGcw was increased in children with OSA and an additional group of snoring children without OSA but subjectively increased respiratory effort compared with primary snorers. The sEMGcw has potential clinical utility in evaluation of children with sleep-disordered breathing as an objective, non-invasive, non-volitional marker of NRD. ABSTRACT: Our aim was to investigate whether neural respiratory drive measured by non-normalized surface EMG recorded from the chest wall overlying the diaphragm (sEMGcw) differentiates children with and without obstructive sleep apnoea (OSA). Polysomnography data of children aged 0-18 years were divided into the following three groups: (i) primary snorers (PS); (ii) snoring children without OSA but with increased work of breathing (incWOB; subjective physician report of increased respiratory effort during sleep); and (iii) children with OSA [obstructive apnoea-hypopnoea index (OAHI) >1 h-1 ]. Excerpts of sEMGcw obtained during tidal unobstructed breathing from light, deep and rapid eye movement sleep were exported for quantitative analysis. Overnight polysomnography data from 45 PS [median age 4.4 years (interquartile range 3.0-7.7 years), OAHI 0 h-1 (0.0-0.2 h-1 )], 19 children with incWOB [age 2.8 years (2.4-5.7 years), OAHI 0.1 h-1 (0.0-0.4 h-1 )] and 27 children with OSA [age 3.6 years (2.6-6.2 years), OAHI 3.7 h-1 (2.3-6.9 h-1 )] were analysed. The sEMGcw was higher in those with OSA [8.47 µV (5.98-13.07 µV); P < 0.0001] and incWOB [8.97 µV (5.94-13.43 µV); P < 0.001] compared with PS [4.633 µV (2.98-6.76 µV)]. There was no significant difference in the sEMGcw between children with incWOB and OSA (P = 0.78). Log sEMGcw remained greater in children with OSA and incWOB compared with PS after age, body mass index centiles, sleep stages and sleep positions were included in the mixed linear models (P < 0.0001). The correlation between sEMGcw and OAHI in children without OSA was small (rs  = 0.254, P = 0.04). The sEMGcw is increased in children with OSA and incWOB compared with PS.