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To describe the outcomes of central sleep apnea requiring home supplemental oxygen therapy in otherwise healthy term infants.All children1 year of age undergoing polysomnography between 2015 and 2020 at the Queensland Children's Hospital were retrospectively studied. Children with gestational age37 weeks, underlying syndrome, cleft palate, those with obstructive apnea-hypopnea index50% of total apnea-hypopnea index, or with underlying cardiac or pulmonary parenchymal pathology were excluded. Polysomnography parameters were extracted for periods both on and off supplemental oxygenation.Fifty-two (mean [standard deviation] age at polysomnography 32.6 [34.7] days; 21 females) term infants were included. There was a statistically significant improvement in apnea-hypopnea index on supplemental oxygen (mean [standard deviation] in room air 50.2 [36.3] vs 11.6 [9],Central sleep apnea in term infants who are otherwise healthy generally has a good prognosis, with oxygen therapy prescribed for around 6 months. Oxygen therapy was associated with improved saturations and decrease in apnea-hypopnea index when assessed with polysomnography.Hayashi A, Suresh S, Kevat A, Robinson J, Kapur N. Central sleep apnea in otherwise healthy term infants.

Aim: In children with Prader-Willi syndrome (PWS), growth hormone (GH) improves height and body composition; however, may be associated with worsening sleep-disordered breathing (SDB). Some studies have reported less SDB after GH initiation, but follow-up with polysomnography is still advised in most clinical guidelines. Methods: This retrospective, multicentre study, included children with PWS treated with GH at seven PWS treatment centres in Australia over the last 18 years. A paired analysis comparing polysomnographic measures of central and obstructive SDB in the same child, before and after GH initiation was performed with Wilcoxon signed-rank test. The proportion of children who developed moderate/severe obstructive sleep apnoea (OSA) was calculated with their binomial confidence intervals. Results: We included 112 patients with available paired data. The median age at start of GH was 1.9 years (range 0.1–13.5 years). Median obstructive apnoea hypopnoea index (AHI) at baseline was 0.43/h (range 0–32.9); 35% had an obstructive AHI above 1.0/h. Follow-up polysomnography within 2 years after the start of GH was available in 94 children who did not receive OSA treatment. After GH initiation, there was no change in central AHI. The median obstructive AHI did not increase significantly (P = 0.13), but 12 children (13%, CI95% 7–21%) developed moderate/severe OSA, with clinical management implications. Conclusions: Our findings of a worsening of OSA severity in 13% of children with PWS support current advice to perform polysomnography after GH initiation. Early identification of worsening OSA may prevent severe sequelae in a subgroup of children.

The Queensland Basic Paediatric Training Network (QBPTN) is responsible for the selection of candidates into paediatric training in Queensland. The COVID-19 pandemic necessitated interviews to be conducted ‘virtually’ as virtual Multiple-Mini-Interviews (vMMI). The study aimed to describe the demographic characteristics of candidates applying for selection into paediatric training in Queensland, and to explore their perspectives and experiences with the vMMI selection tool. The demographic characteristics of candidates and their vMMI outcomes were collected and analysed with a mixed methods approach. The qualitative component was comprised of seven semi-structured interviews with consenting candidates. Seventy-one shortlisted candidates took part in vMMI and 41 were offered training positions. The demographic characteristics of candidates at various stages of selection were similar. The mean vMMI scores were not statistically different between candidates from the Modified Monash Model 1 (MMM1) location and others [mean (SD): 43.5 (5.1) versus 41.7 (6.7), respectively, p = 0.26]. However, there was a statistically significant difference (p value 0.03) between being offered and not offered a training position for candidates from MMM2 and above. The analysis of the semi-structured interviews suggested that candidate experiences of the vMMI were influenced by the quality of the management of the technology used. Flexibility, convenience, and reduced stress were the main factors that influenced candidates’ acceptance of vMMI. Perceptions of the vMMI process focused on the need to build rapport and facilitate communication with the interviewers. vMMI is a viable alternative to face-to-face (FTF) MMI. The vMMI experience can be improved by facilitating enhanced interviewer training, by making provision for adequate candidate preparation and by having contingency plans in place for unexpected technical challenges. Given government priorities in Australia, the impact of candidates’ geographical location on the vMMI outcome for candidates from MMM >1 location needs to be further explored.

There is limited data on indications and outcomes of home continuous positive airway pressure (CPAP) therapy in the first year of life. We aimed to analyze the clinical, demographic, and polysomnographic (PSG) characteristics of a cohort of children initiated on home CPAP for treatment of sleep disordered breathing (SDB) and as respiratory support in the first year of life.Children started on CPAP in the first year of life at the Queensland Children's Hospital were retrospectively evaluated for clinicaldemographic parameters, underlying diagnoses, respiratory support, airway surgical intervention, PSG results at baseline and on CPAP.Twenty-nine infants [median age (IQR) at CPAP initiation 182 days (126-265.5); 12F] were included. Underlying aetiology included Trisomy 21 (n=6), craniofacial syndromes (n=5), hypotonia (n=8; 5 with non-craniofacial syndrome), airway malacia (n=5), skeletal dysplasia (n=2), non-syndromic upper airway obstruction (n=2) and CNLD (n=1). The median (IQR) Obstructive Apnoea-Hypopnea Index was 14\hr (6.2-31) at CPAP initiation, which improved on CPAP to 3.4\hr (1.4-6.4). Median (IQR) TcCOHome CPAP as respiratory support is an effective long-term therapy in infancy and can be weaned even if initiated early. Prospective studies with pre-defined criteria for CPAP initiation and cessation would help ascertain long term outcomes in this poorly researched group.

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.

Aim: Aerodigestive clinics (ADCs) are multidisciplinary programmes for the care of children with complex congenital or acquired conditions affecting breathing, swallowing and growth. Our objective was to describe the demographic, clinical, etiological and investigational profile of children attending the inaugural ADC at a tertiary paediatric centre in Queensland. Methods: Children referred to the ADC at Queensland Children's Hospital from August 2018 to December 2019 were included. Data on clinical, growth and lung function parameters, bronchoscopy and upper gastrointestinal endoscopy findings, thoracic imaging and comorbidities were retrospectively analysed. Results: Fifty-six children (median (range) age 4 years (3 months–15 years); 18 female) attended the ADC during this 17-month period. Forty-six (82%) children had previous oesophageal atresia with tracheo-oesophageal fistula; 43 of these were type C. Previous isolated oesophageal atresia, congenital diaphragmatic hernia and congenital pulmonary malformation were the underlying disorder in three (5%) children each, with one child having a repaired laryngeal cleft. Vertebral Anal Tracheo Esophageal Renal Limb anomalies (VACTERL)/Vertebral Anal Tracheo Esophageal renal anomalies (VATER) association was seen in 21 (38%) children. Growth was adequate (median weight and body mass index z-score −0.63 and −0.48, respectively). Thirty-four (61%) children reported ongoing wet cough, with 12 (21%) requiring previous hospital admission for lower respiratory tract infection. Fourteen (25%) had bronchiectasis on computed tomography chest and 33 (59%) had clinical tracheomalacia, apparent on bronchoscopic examination in 21 patients. Dysphagia was reported in 15 (27%) children, 11 (20%) were gastrostomy feed-dependent and 5 (9%) had biopsy-proven eosinophilic oesophagitis. Conclusion: High proportion of children attending the ADC have ongoing respiratory symptoms resulting in chronic pulmonary suppuration and bronchiectasis. Potential benefits of this model of care need to be studied prospectively to better understand the outcomes.

Chronic neonatal lung disease (CNLD) is defined as continued need for any form of respiratory support (supplemental oxygen and/or assisted ventilation) beyond 36 weeks PMA. Low‐flow supplemental oxygen facilitates discharge from hospital of infants with CNLD who are hypoxic in air and is widely used despite lack of evidence on the most appropriate minimum mean target oxygen saturations. Furthermore, there are minimal data to guide the home monitoring, titration or weaning of supplemental oxygen in these infants. The purpose of this position statement is to provide a guide for the respiratory management of infants with CNLD, with special emphasis on role and logistics of supplemental oxygen therapy beyond the NICU stay. Reflecting a variety of clinical practices and infant comorbidities (presence of pulmonary hypertension, retinopathy of prematurity and adequacy of growth), it is recommended that the minimum mean target range for SpO2 during overnight oximetry to be 93–95% with less than 5% of total recording time to be below 90% SpO2. Safety of short‐term disconnection from supplemental oxygen should be assessed before discharge, with majority of infants with CNLD not ready for discharge until supplemental oxygen requirement is ≤0.5 L/min. Sleep‐time assessment of oxygenation with continuous overnight oximetry is recommended when weaning supplemental oxygen. Palivizumab is considered safe and effective for the reduction of hospital admissions with RSV infection in this group. This statement would be useful for paediatricians, neonatologists, respiratory and sleep physicians and general practitioners managing children with CNLD., Watch the video abstract

Pneumocystis jirovecii is recognized as an opportunistic pathogen in immunosuppressed patients. We report a case of severe Pneumocystis pneumonia (PCP) in an infant with acquired combined immunodeficiency secondary to maternal chemotherapy exposure during the second and third trimesters of pregnancy. The infant required cardiorespiratory support with veno-venous extracorporeal membrane oxygenation (VV-ECMO) for severe respiratory failure. This case highlights the potential for severe acquired immunodeficiency in this patient cohort and further postnatal surveillance is highly recommended.

1 Morgagni hernia can be a rare cause of intermittent positional cyanosis without apnoea in a neonate. 2. Intermittent compressive effect of herniated abdominal contents on the right heart can cause right to left shunting of blood through a patent foramen ovale and thereby produce intermittent positional cyanosis. 3. Point-of-care echocardiography during a desaturation event can demonstrate the Morgagni hernia and the reversal of blood flow through a patent foramen ovale.

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 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. What is Known:• Individuals with Prader-Willi syndrome (PWS) commonly experience excessive daytime sleepiness and exhibit emotional/behavioral disturbances.• In the typically developing population, sleepiness is associated with emotional/behavioral disturbances, independently of sleep-disordered breathing.. What is New:• This study found evidence for a direct link between daytime sleepiness and emotional/behavioral disturbances, independent of sleep-related breathing difficulties, sleep adequacy, and body mass index.• Excessive daytime sleepiness may be a modifiable risk factor for emotional/behavioral disturbances in PWS.

We report a child with persistently low SpO2 with delayed diagnosis due to the co-existing CPAM with possible AVM. The diagnosis was only achieved after low SpO2 incidentally discovered from the child’s father. The eventual cause was Haemoglobin I-Toulouse, making both patients the first reported cases with low SpO2.

Introduction The Queensland Basic Paediatric Training Network (QBPTN) is the centralised pathway for entry into paediatric training in Queensland, Australia. In response to COVID-19 travel and social distancing restrictions imposed in 2020, QBPTN successfully adopted a Virtual Multiple Mini Interviews (vMMIs) model for the selection of candidates for entry into paediatric training. The authors describe the planning, implementation, challenges, and evaluation of candidates' and interviewers' experiences of vMMIs, including the differences between candidates from two geographical areas. Methods The contents of six vMMI stations were similar to face-to-face MMI. Implementation required the identification of ZOOMTM as a preferred online platform, securing venues, communication, development of contingency plans and central coordination by the network. Candidates' experiences with vMMI were explored through thematic analysis of the qualitative data from focus groups and free text responses, and descriptive analysis of SurveyMonkey© questionnaire responses. Experiences between 'metropolitan' and 'regional and interstate' candidates were compared. Results 5-minute stations with 2-minute pre-reading were used. 78 candidates and 14 interviewers participated in the selection process. All candidates attended the focus group. 58.7% of candidates responded to post vMMI questionnaire. 93% of survey responders were happy to undertake vMMI in the future, with 23% feeling they would have performed better in face-to-face. Experiences between 'metropolitan' and 'other' groups were similar. Positive experiences of participants were related to the user-friendly IT platform, successful pre-interview communications, preparation, convenience, time, and cost savings. Stress related IT failures and difficulties establishing rapport with interviewers were reported as the main negative experiences. Conclusion 'vMMI' is a feasible and acceptable method of selection into paediatric training. vMMI has many benefits and can be implemented relatively quickly by addressing key logistical requirements. The model under discussion could be adapted by other centres based on local needs.

Mycobacterium abscessus complex pulmonary disease (M. abscessus PD) in cystic fibrosis (CF) is challenging to treat. Current guideline therapeutic regimens involving an intensive phase of intravenous (IV) antibiotics followed by a consolidation phase of inhaled and oral antibiotics are not evidence-based. The objectives of this study were to characterize the clinical outcomes and clearance of Mycobacterium abscessus complex (M. abscessus) from respiratory cultures in children with CF M. abscessus PD.This retrospective longitudinal cohort analysis evaluated the first course of treatment for M. abscessus PD in 33 children in Queensland, Australia between 2001 and 2015. Spirometry and nutritional outcomes 2 years pretreatment and 1 year posttreatment were compared with clearance or relapse/persistence of Mycobacterium abscessus complex from respiratory cultures.Nine of 18 children who completed therapy, cleared infection. Three of 7 children who completed only intensive therapy cleared sputum compared with 0/8 children who did not. The trajectory of the percent predicted forced expiratory volume in 1 s and age standardized body mass index significantly improved posttreatment in those that cleared sputum (P0.0001).These results suggest that current treatment recommendations for M. abscessus PD are associated with some success in clearing infection in children with CF and improvement in lung function and body mass index. Clinical trials are required to determine the best treatment approaches.

Prader‐Willi syndrome (PWS) is a rare genetic condition with multi‐system involvement. The literature was reviewed to describe neurodevelopment and the behavioural phenotype, endocrine and metabolic disorders and respiratory and sleep functioning. Implications for child and family quality of life were explored. Challenging behaviours contribute to poorer well‐being and quality of life for both the child and caregiver. Recent evidence indicates healthy outcomes of weight and height can be achieved with growth hormone therapy and dietary restriction and should be the current target for all individuals with PWS. Gaps in the literature included therapies to manage challenging behaviours, as well as understanding the effects of growth hormone on respiratory and sleep function. New knowledge regarding the transition of children and families from schooling and paediatric health services to employment, accommodation and adult health services is also needed. Developing a national population‐based registry could address these knowledge gaps and inform advocacy for support services that improve the well‐being of individuals with PWS and their families.

BACKGROUND: Potentially pathogenic microorganisms are frequently isolated from tracheostomized children, although evidence for empirical therapy of respiratory exacerbation is limited. We aimed to describe upper airway microbiology as found on endotracheal aspirate (ETA) in tracheostomized children and to correlate it with lower airway microbiology through bronchoalveolar lavage fluid. METHODS: We retrospectively reviewed records and airway microbiology of all tracheostomized children under the follow-up care of Queensland Children’s Hospital. Subanalysis was based on ventilatory and multidrug-resistant organism status. Sensitivity and specificity of ETA for predicting Pseudomonas aeruginosa and Staphylococcus aureus lower airway isolation were calculated using concomitant bronchoalveolar lavage fluid culture as the accepted standard. RESULTS: From 43 children (18 female, median [interquartile range (IQR)] age 68 (41–115) months, 14 ventilated), 15 different potentially pathogenic microorganisms were isolated (mean ± SD: 3.30 ± 2.23), with S. aureus (n = 33, 77%) and P. aeruginosa (n = 29, 67%) predominating. Significantly more types of potentially pathogenic microorganisms were isolated from ventilated children (median 4.00 [IQR 3.25–5.75]) than from nonventilated children (median 2.00 [IQR 1.00–4.00] (P = .007), with 93% of ventilated children isolating S. aureus and 86% P. aeruginosa. Multidrug-resistant organisms were present in 12 (28%) children, of whom 8 were ventilated. Methicillin-resistant S. aureus (MRSA) was isolated in 9 (21%) children, of whom 6 were ventilated. For P. aeruginosa and S. aureus isolation, ETA had high sensitivity (95% and 100%, respectively) but low specificity (64.7% and 33.3%, respectively) when compared with bronchoalveolar lavage fluid. CONCLUSIONS: In children with tracheostomy, the predominant respiratory bacterial pathogens were S. aureus and P. aeruginosa, with MRSA being isolated less frequently than previously described. Multidrug-resistant organisms are isolated more frequently from ventilated children. ETA microbiology is a good screening modality, with negative ETA potentially ruling out lower airway S. aureus and P. aeruginosa. Adequately powered prospective studies with quantitative cultures could enhance understanding and guide therapy.

Tracheal and bronchial varices are rarely found in children. However, they have been described in adults with failing Fontan circuits or secondary to vascular pathology, such as portal and pulmonary hypertension. We report the presentation of haemoptysis and bronchial varices in a child, six years after a Fontan procedure for tricuspid atresia. She had tortuous mediastinal and transpleural arterial collaterals on computed tomography (CT) angiography and cardiac catheterization and subsequently underwent embolization of these collaterals. While the haemoptysis settled post embolization, the bronchial varices persisted on repeat bronchoscopy. She has since been clinically well with no further haemoptysis., Tracheal and bronchial varices are rare, especially in children. We report the first presentation of bronchial varices and haemoptysis in a child, six years after a Fontan procedure for tricuspid atresia. She had tortuous mediastinal and transpleural arterial collaterals on imaging and subsequently underwent embolization of these collaterals with haemoptysis settling after this.

ObjectiveResearch is needed to determine best practice for genomic testing in the context of child interstitial or diffuse lung disease (chILD). We explored parent’s and child’s health-related quality of life (HRQoL), parents’ perceived understanding of a genomic testing study, satisfaction with information and the study and decisional regret to undertake genomic testing.MethodsParents of children with diagnosed or suspected chILD who were enrolled in a genomic sequencing study were invited to complete questionnaires pretesting (T1) and after receiving the result (T2).ResultsParents’ (T1, n=19; T2, n=17) HRQoL was lower than population norms. Study satisfaction (T1) and perceived understanding (T2) were positively correlated (rs=0.68, p=0.014). Satisfaction with information (T1 and T2) and decisional regret (T2) were negatively correlated (T1 rs=−0.71, p=0.01; T2 rs=−0.56, p=0.03). Parents reported wanting more frequent communication with staff throughout the genomic sequencing study, and greater information about the confidentiality of test results.ConclusionsUnderstanding of genomic testing, satisfaction with information and participation and decisional regret are inter-related. Pretest consultations are important and can allow researchers to explain confidentiality of data and the variable turnaround times for receiving a test result. Staff can also update parents when there will be delays to receiving a result.

BackgroundCongenital central hypoventilation syndrome (CCHS) is a rare disorder of autonomic control. A hypoglycaemic seizure in a 4-year-old girl with CCHS led to a more detailed examination of glycaemic control in a cohort of children with CCHS.MethodsWe conducted an observational cohort study of glucose homeostasis in seven children (3 months to 12 years) with genetically confirmed CCHS using a combination of continuous glucose monitoring (CGM), fasting studies and oral glucose tolerance test (OGTT). CGM was used to compare the effect of diazoxide and dietary intervention in the index patient.ResultsHypoglycaemia was not elicited by fasting in any of the patients. Increased postprandial glycaemic variability was evident in all patients using CGM, with seven of seven patients demonstrating initial postprandial hyperglycaemia (plasma-glucose concentration >7.8 mmol/L), followed by asymptomatic hypoglycaemia (plasma-glucose concentration ≤2.8 mmol/L) in two of seven patients that was also demonstrated on OGTT. Both diazoxide and low Glycaemic Index (GI) dietary intervention reduced the proportion of CGM readings ConclusionsGlucose variability associated with autonomic dysfunction may be unrecognised in CCHS, particularly in children with more severe phenotypes. This report highlights the occurrence of hyperglycaemia as well as hypoglycaemia in CCHS. Given the challenges of recognising hypoglycaemia based on clinical symptomatology, the use of CGM may facilitate its identification allowing appropriate management. The observed normoglycaemia during fasting combined with increased postprandial plasma blood glucose level (BGL) variability is more consistent with dumping syndrome than persistent hyperinsulinism. Dietary modifications therefore may be more effective than diazoxide in managing hypoglycaemia.

Introduction: In adults with bronchiectasis, multicentre data advanced the field including disease characterisation and derivation of phenotypes such as ‘frequent exacerbator (FE)’ (≥3 exacerbations/year). However, paediatric cohorts are largely limited to single centres and no scientifically derived phenotypes of paediatric bronchiectasis yet exists. Using paediatric data from the Australian Bronchiectasis Registry (ABR), we aimed to: (a) describe the clinical characteristics and compare Indigenous with non-Indigenous children, and (b) determine if a FE phenotype can be identified and if so, its associated factors. Methods: We retrieved data of children (aged

Background Childhood interstitial lung disease (chILD) represents a rare heterogeneous group of respiratory disorders. In the absence of randomized controlled clinical trials, global collaborations have utilized case series with an aim to standardising approaches to diagnosis and management. Australasian data are lacking. The aim of this study was to calculate prevalence and report the experience of chILD in Australasia over a decade. Methods Paediatric pulmonologists in Australia and New Zealand involved in the care of patients aged 0–18 years with chILD completed a questionnaire on demographics, clinical features and outcomes, over a 10 year period. These data, together with data from the 2 reference genetics laboratories, were used to calculate prevalence. Results One hundred fifteen cases were identified equating to a period prevalence (range) of 1.5 (0.8–2.1) cases/million for children aged 0–18years. Clinical data were provided on 106 patients: the

STUDY OBJECTIVES: Continuous positive airway pressure (CPAP) has been increasingly used in children with obstructive sleep apnea (OSA), though it is unclear whether it can ever be ceased. We describe the clinical, demographic, and polysomnographic (PSG) characteristics of a cohort of children with OSA who were successfully weaned off CPAP. METHODS: From a pediatric cohort on CPAP for OSA at the Queensland Children’s Hospital between January 2016 and December 2017, a subgroup of children who were taken off CPAP were retrospectively studied. RESULTS: CPAP therapy was stopped for 53 children over a 2-year period; 29 of these were excluded from analysis due to change to bilevel support (n = 2), transition to adult care (n = 12), or cessation due to poor adherence (n = 15). A total of 24 children [median (interquartile range, IQR) age 4.1 years (1.0–10.5); 18 males] were successfully weaned off CPAP therapy based on improvement in clinical and PSG parameters; and were included in the analysis. These children had a median (IQR) apnea-hypopnea index (AHI) of 9.8 (5.7–46.0) at CPAP initiation, which improved to 3.3 (0.4–2.2) at CPAP cessation after a median (IQR) duration of 1.0 (0.5–2.0) year. The reasons for CPAP cessation included improved symptoms and/or PSG parameters with time (n = 11); improvement after airway surgery (n = 7), and improvement of body mass index (n = 2). In four children, CPAP therapy was ceased after initial trial due to low physician perceived clinical benefit. CONCLUSIONS: This is the first study describing the characteristics of children and likely reasons for successful CPAP cessation. Children on CPAP should be regularly screened for ongoing CPAP need. CITATION: King Z, Josee-Leclerc M, Wales P, Masters IB, Kapur N. Can CPAP therapy in pediatric OSA ever be stopped? J Clin Sleep Med. 2019;15(11):1609–1612.

Background and Objective: Children with bronchiectasis have recurrent exacerbations and may require hospitalization. “Hospital in the home (HITH)” is used as an alternative to hospitalization for children with cystic fibrosis (CF) but to date, there is no published data on children without CF. We describe our experience of HITH (intravenous [IV] antibiotics and at least once-daily physiotherapy-treated airway clearance therapy) in a cohort of children with bronchiectasis, comparing outcomes between hospital and HITH-based pathways. Methods: Medical records were retrospectively reviewed in children with bronchiectasis who were hospitalized in our center from July 2016 to July 2018. We compared treatment duration, symptom resolution, adverse events, oral antibiotic prescription on discharge and “time-to-next hospitalization” between children managed with the two treatment pathways. Results: Exacerbations in 63 children (median age = 6 years [range: 1-17]; females = 33, indigenous = 8) with bronchiectasis treated with IV antibiotic therapy were analyzed (HITH n = 45, 71.5%). Duration of treatment and symptom resolution was similar between groups (hospital: median = 14 days [interquartile range {IQR}: 14-14] and 12/18 [66.6%], respectively vs HITH: 14 [14-15.5] and 31/45 [69%]; P =.53 and.85, respectively). There was no significant difference in adverse events (16.6% vs 9%), prescription of oral antibiotics on discharge (44% vs 24%), or “time-to-next hospitalization” (median 42 [IQR: 24-100] vs 67 [IQR: 32-95] weeks) between hospital and HITH groups, respectively. Conclusions: In children with bronchiectasis treated for a severe exacerbation, receiving treatment in the home setting with HITH does not compromise short-term clinical outcomes compared to hospital only treatment. Prospective studies are required to provide more robust evidence in this under-researched area.

Introduction: Children with bronchiectasis have recurrent exacerbations and many require hospitalisation. ‘Hospital in the home (HITH)’ is an alternative to hospitalisation for children with Cystic-Fibrosis(CF) but there is little data in those without CF. We describe our experience of HITH in a cohort of children with bronchiectasis and compared outcomes between hospital- and HITH-based pathways. Methods: Medical records were retrospectively reviewed in children with bronchiectasis who were hospitalised in our centre from Jan-2017 to June-2018. We assessed treatment duration, symptom resolution, oral antibiotic prescription on discharge, adverse events and time to next exacerbation. Results: We analysed 54 exacerbations (HITH n=38, 70%) in 46 children [median age=4.5 (IQR 2-10.25) years; females=26, Indigenous=6]. Mean (SD) IV antibiotic therapy was for 14.9 (2.7) days. There was no difference in duration of treatment between the HITH [median=14 (IQR 14-16.25) days] vs. hospital groups [14 (14-14); p=0.65]. 21/38 (55.2%) children in the HITH group had symptom resolution at end of IV treatment compared to 10/16 (62.5%) in the hospital group; p=0.62 with 26% children in the HITH group discharged on oral antibiotics compared to 47% of the hospital group; p=0.13. There was no difference in time to next exacerbation between groups [HITH median=12 (7-15.75) weeks, hospital=9(6-24)]. No difference in adverse events was seen between the two groups. Conclusions: A high proportion of pulmonary exacerbations in children with bronchiectasis requiring IV antibiotics are managed with HITH. The equivalence of HITH to inpatient treatment remains conjectural until prospective studies are undertaken.

Introduction: Continuous Positive Airway Pressure (CPAP) has been increasingly used in treatment of paediatric Obstructive Sleep Apnoea (OSA) not resolved by surgical intervention. It is unclear what proportion of children can be successfully taken off CPAP and the factors that govern this cessation. Aims: To describe the clinical, demographic and polysomnographic (PSG) characteristics of a cohort of children on CPAP for OSA, who were weaned off CPAP. Methods: From a cohort of children on CPAP for OSA at the Queensland Children’s Hospital between 2016-17, a group of children who were taken off CPAP were selected. Those who were transitioned to adults, changed to bilevel support or stopped due to non-adherence were excluded. Results: CPAP therapy was stopped for 53 children over a two-year period, 28 of these were excluded. 24 children [mean(SD) age 6.1(5.5) years; 18 M] were successfully weaned off CPAP therapy based on clinical judgement and PSG data analysis; and were included in the final analysis. These children had a median(IQR) AHI of 9.8(5.7-46.0) at CPAP initiation, and after a median(IQR) duration of 1.0 (0.5-2.0) year, had an improvement in median AHI to 3.0(1.9–4.8) at CPAP cessation. The reasons for CPAP cessation included improvement post airway surgery in (n=7); weight loss (n=2) and improved symptoms and/or PSG parameters with time (n=11). In 4 children, CPAP therapy was ceased after initial trial due to poor tolerance and low physician perceived clinical benefit. Conclusion: This is one of the first studies describing the characteristics of children successfully weaned off CPAP therapy. Children on CPAP should be regularly screened for ongoing symptoms and need for pressure support checked as a small group can be weaned.

Background Spinal muscular atrophy (SMA) causes progressive respiratory muscle weakness but respiratory function (RF) in those using noninvasive ventilation (NIV) is not well described. Objective To describe RF in childhood SMA and assess differences between those using and not using NIV. Methods A cross‐sectional study of childhood SMA assessed polysomnography (PSG), spirometry, forced oscillation technique (FOT), lung clearance index (LCI), sniff nasal inspiratory pressures, peak cough flow, maximal inspiratory and expiratory pressure, and NIV use and indication. Results Twenty‐five children (median age [interquartile range], 8.96 [5.63] years; 10 F) with SMA 1 (n = 3), 2 (n = 15), and 3 (n = 7) were recruited. Spirometry and FOT testing was feasible in children as young as 3 years. Ten (40%) required NIV, 5 for sleep‐disordered breathing (SDB), and 5 initiated during lower respiratory tract infection (LRTI). Children requiring NIV were older (median, 10.52 vs 5.67 years; P

BACKGROUND: Bronchiectasis is being increasingly diagnosed and recognised as an important contributor to chronic lung disease in both adults and children in high‐ and low‐income countries. It is characterised by irreversible dilatation of airways and is generally associated with airway inflammation and chronic bacterial infection. Medical management largely aims to reduce morbidity by controlling the symptoms, reduce exacerbation frequency, improve quality of life and prevent the progression of bronchiectasis. This is an update of a review first published in 2000. OBJECTIVES: To evaluate the efficacy and safety of inhaled corticosteroids (ICS) in children and adults with stable state bronchiectasis, specifically to assess whether the use of ICS: (1) reduces the severity and frequency of acute respiratory exacerbations; or (2) affects long‐term pulmonary function decline. SEARCH METHODS: We searched the Cochrane Register of Controlled Trials (CENTRAL), the Cochrane Airways Group Register of trials, MEDLINE and Embase databases. We ran the latest literature search in June 2017. SELECTION CRITERIA: All randomised controlled trials (RCTs) comparing ICS with a placebo or no medication. We included children and adults with clinical or radiographic evidence of bronchiectasis, but excluded people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: We reviewed search results against predetermined criteria for inclusion. In this update, two independent review authors assessed methodological quality and risk of bias in trials using established criteria and extracted data using standard pro forma. We analysed treatment as 'treatment received' and performed sensitivity analyses. MAIN RESULTS: The review included seven studies, involving 380 adults. Of the 380 randomised participants, 348 completed the studies. Due to differences in outcomes reported among the seven studies, we could only perform limited meta‐analysis for both the short‐term ICS use (6 months or less) and the longer‐term ICS use (> 6 months). During stable state in the short‐term group (ICS for 6 months or less), based on the two studies from which data could be included, there were no significant differences from baseline values in the forced expiratory volume in the first second (FEV(1)) at the end of the study (mean difference (MD) ‐0.09, 95% confidence interval (CI) ‐0.26 to 0.09) and forced vital capacity (FVC) (MD 0.01 L, 95% CI ‐0.16 to 0.17) in adults on ICS (compared to no ICS). Similarly, we did not find any significant difference in the average exacerbation frequency (MD 0.09, 95% CI ‐0.61 to 0.79) or health‐related quality of life (HRQoL) total scores in adults on ICS when compared with no ICS, though data available were limited. Based on a single non‐placebo controlled study from which we could not extract clinical data, there was marginal, though statistically significant improvement in sputum volume and dyspnoea scores on ICS. The single study on long‐term outcomes (over 6 months) that examined lung function and other clinical outcomes, showed no significant effect of ICS on any of the outcomes. We could not draw any conclusion on adverse effects due to limited available data. Despite the authors of all seven studies stating they were double‐blind, we judged one study (in the short duration ICS) as having a high risk of bias based on blinding, attrition and reporting of outcomes. The GRADE quality of evidence was low for all outcomes (due to non‐placebo controlled trial, indirectness and imprecision with small numbers of participants and studies). AUTHORS' CONCLUSIONS: This updated review indicates that there is insufficient evidence to support the routine use of ICS in adults with stable state bronchiectasis. Further, we cannot draw any conclusion for the use of ICS in adults during an acute exacerbation or in children (for any state), as there were no studies.

With contemporary cystic fibrosis (CF) care, a lung abscess is an uncommon occurrence. We describe a case of a staphylococcal lung abscess in a teenage girl with CF who presented with a two-week history of non-specific malaise followed by two days of left posterior chest pain and fever. A chest radiograph was consistent with a left sided pulmonary abscess, which was confirmed on a CT scan of the chest. The abscess was drained under ultrasound guidance and cultured methicillin-sensitive Staphylococcus aureus. The patient responded well to antibiotic treatment with the abscess cavity showing complete radiological resolution by six weeks post drainage. Keywords: Cystic fibrosis, Pulmonary abscess

Aplasia of the epiglottis is a rare airway abnormality requiring airway and feeding interventions. We report a case of bronchiectasis in the setting of congenital aplasia of the epiglottis, secondary to early‐life aspiration events. Compensatory mechanisms for airway protection likely develop later in life.

Transient epileptic amnesia (TEA) is a recently recognised form of epilepsy of which the principle manifestation is recurrent, transient episodes of isolated memory loss. In addition to the amnesic episodes, many patients describe significant interictal memory difficulties. Performance on standard neuropsychological tests is often normal. However, two unusual forms of memory deficit have recently been demonstrated in TEA: (i) accelerated long-term forgetting (ALF): the excessively rapid loss of newly acquired memories over a period of days or weeks and (ii) remote autobiographical memory loss: a loss of memories for salient, personally experienced events of the past few decades. The neuroanatomical bases of TEA and its associated memory deficits are unknown. In this study, we first assessed the relationship between subjective and objective memory performance in 41 patients with TEA. We then analysed MRI data from these patients and 20 matched healthy controls, using manual volumetry and voxel-based morphometry (VBM) to correlate regional brain volumes with clinical and neuropsychological data. Subjective memory estimates were unrelated to performance on standard neuropsychological tests but were partially predicted by mood, ALF and remote autobiographical memory. Manual volumetry identified subtle hippocampal volume loss in the patient group. Both manual volumetry and VBM revealed correlations between medial temporal lobe atrophy and standard anterograde memory scores, but no relation between atrophy and ALF or remote autobiographical memory. These results add weight to the hypothesis that TEA is a syndrome of mesial temporal lobe epilepsy. Furthermore, they suggest that although standard anterograde memory test performance is related to the degree of mesial temporal lobe damage, this is not true for ALF and autobiographical amnesia. It is possible that these unusual memory deficits have a more diffuse physiological basis rather than being a consequence of discrete structural damage. © The Author (2008). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved.

Rationale Exacerbations in non-cystic fibrosis (CF) bronchiectasis are associated with worsening lung functions and quality of life. A standardized definition of exacerbation could improve clinical care and research. Methods: A cohort of 69 children with non-CF bronchiectasis was prospectively followed for 900 child-months. The changes in clinical, systemic, and lung function parameters from 81 exacerbations were statistically evaluated using conditional logistic regression, receiver operating characteristic, sensitivity, specificity, and positive (PPV) and negative predictive values (NPV) to formulate a definition of a pulmonary exacerbation. Formation of major and minor criteria was statistically based and models were developed. Measurements and Main Results: Wet cough and cough severity (score >= 2) over 72-hr were the best predictors of an exacerbation with area under the curve (AUC) of 0.85 (95% CI 0.79-0.92) and 0.84 (95% CI 0.77-0.91), respectively. Sputum color, chest pain, dyspnea, hemoptysis, and chest signs were significant though minor criteria. Inclusion of serum C-reactive protein, amyloid-A, and IL6 to the definition improved its specificity and PPV. Our final combined model consisted of one major with one investigatory criterion (PPV 91%, NPV 72%); two major criteria (PPV 79%, NPV 91%); or one major and two minor criteria (PPV 79%, NPV 94%). Conclusions: Pulmonary exacerbation in children with non-CF bronchiectasis can be validly predicted using a standardized assessment of clinical features, with additional systemic markers improving predictive values. This definition potentially facilitates earlier detection (leading to appropriate management) of exacerbations. Pediatr Pulmonol. 2012; 47: 68-75. (C) 2011 Wiley Periodicals, Inc.

Non-CF bronchiectasis remains a major cause of morbidity not only in developing countries but in some indigenous groups of affluent countries. Although there is a decline in the prevalence and incidence in developed countries, recent studies in indigenous populations report higher prevalence. Due to the lack of such data, epidemiological studies are required to find the incidence and prevalence in developing countries. Although the main characteristics of bronchiectasis are similar in developing and affluent countries, underlying aetiology, nutritional status, frequency of exacerbations and severity of the disease are different. Delay of diagnosis is surprisingly similar in the affluent and developing countries possibly due to different reasons. Long-term studies are needed for evidence based management of the disease. Successful management and prevention of bronchiectasis require a multidisciplinary approach, while the lack of resources is still a major problem in the developing countries.

Background The radiologic definition of airway dilatation and bronchiectasis in children has substantial limitations. Bronchoarterial (BA) ratio is a commonly used criterion to define airway dilatation despite the lack of normative pediatric data. The objective of our study was to determine the range of normal bronchial to accompanying arterial diameter ratio on high-resolution CT scan of the chest in children and compare it with the available adult data. Methods Children undergoing multidetector CT scan of the chest for nonpulmonary conditions at a single center were prospectively identified. High-resolution reconstruction was performed on those included and both airway lumen and vessel diameters were measured in the upper and lower lobes of both lungs. Mean BA ratio was calculated for each included child, and its correlation with age was assessed. Results Forty-one children were included; the mean (SD) BA ratio was 0.626 (0.068) (range, 0.437-0.739). This ratio was lower than comparable adult data (combined mean [SD], 0.676 [0.12]; P = .01). No correlation was found with age in our cohort ( r = −0.21, P = .19). There was no difference in the ratio based on laterality or lobe. Conclusions In the pediatric age group, the airway is significantly smaller than the adjoining vessel. Using the radiologic criteria of BA ratio > 1 to define bronchial dilatation would underestimate the presence and extent of bronchiectasis, leading to delayed and missed diagnosis. This highlights the need to redefine the criteria for bronchial dilatation in children.

Background Longitudinal FEV 1 data in children with non-cystic fibrosis (non-CF) bronchiectasis (BE) are contradictory, and there are no multifactor data on the evolution of lung function and growth in this group. We longitudinally reviewed lung function and growth in children with non-CF BE and explored biologically plausible factors associated with changes in these parameters over time. Methods Fifty-two children with ≥ 3 years of lung function data were retrospectively reviewed. Changes in annual anthropometry and spirometry at year 3 and year 5 from baseline were analyzed. The impact of sex, age, cause, baseline FEV 1 , exacerbation frequency, radiologic extent, socioeconomic status, environmental tobacco smoke exposure, and period of diagnosis was evaluated. Results Over 3 years, the group mean forced expiratory flow midexpiratory phase percent predicted and BMI z -score improved by 3.01 ( P = .04; 95% CI, 0.14-5.86) and 0.089 ( P = .01; 95% CI, 0.02-0.15) per annum, respectively. FEV 1 % predicted, FVC% predicted, and height z -score all showed nonsignificant improvement. Over 5 years, there was improvement in FVC% predicted (slope 1.74; P = .001) annually, but only minor improvement in other parameters. Children with immunodeficiency and those with low baseline FEV 1 had significantly lower BMI at diagnosis. Frequency of hospitalized exacerbation and low baseline FEV 1 were the only significant predictors of change in FEV 1 over 3 years. Decline in FEV 1 % predicted was large (but nonsignificant) for each additional year in age of diagnosis. Conclusions Spirometric and anthropometric parameters in children with non-CF BE remain stable over a 3- to 5-year follow-up period once appropriate therapy is instituted. Severe exacerbations result in accelerated lung function decline. Increased medical cognizance of children with chronic moist cough is needed for early diagnosis, better management, and improving overall outcome in BE.

Background: Few studies, and none in Canada, have examined the relation between maternal smoking, alcohol consumption and drug dependence during pregnancy and early neonatal morbidity. Methods: We analyzed records of singleton live births in Alberta, Canada. Markers of neonatal morbidity were Apgar scores (

Summary Children with bronchiectasis have recurrent acute pulmonary exacerbations and many of these exacerbations require hospital admission when oral therapies fail. However there is no standardized definition and little published data is available about the features of an exacerbation. Our aim was to determine the clinical and investigational features of exacerbations in bronchiectasis, the proportion that fail to resolve on oral antibiotics and the factors associated with it. Methods A retrospective cohort study of 115 respiratory exacerbations from 30 children with noncystic fibrosis bronchiectasis diagnosed on HRCT chest. Clinical features, investigations and treatment related to the exacerbations were extracted and analysed. Results Increase in frequency of cough (88%) and a change in its character (67%) were the most common symptoms associated with an exacerbation. Fever (28%), increase in sputum volume (42%) and purulence (35%) were also common features. Chest pain, dyspnea, hemoptysis and tachypnea were rare. 56% had a worsening in their chest auscultatory findings during an exacerbation. Spirometry was not significantly different between stable and exacerbation state. 35% of exacerbations failed to respond to oral antibiotic therapy and required hospital admission. Prophylactic antibiotic therapy was the only significant predictor of failure of oral therapy with adjusted odds ratio of 6.77 (95% CI 2.06–19.90; p =0.003). Conclusions Important clinical features of non-CF exacerbation in bronchiectasis were changes in cough frequency or character, and worsening chest signs; which resolved on therapies. However there is a high failure rate of oral antibiotic therapy and use of prophylactic antibiotic therapy increases this risk.

Background: Although occupational asthma is a well recognized and preventable disease, the numbers of cases presenting for compensation may be far lower than the true incidence. Methods: Workers' Compensation Board (WCB) claims for any reason 1995-2004 were linked to physician billing data. New onset adult asthma (NOAA) was defined as a billing for asthma (ICD-9 code of 493) in the 12 months prior to a WCB claim without asthma in the previous 3 years. Incidence was calculated by occupation, industry and, in a case-referent analysis, exposures estimated from an asthma specific job exposure matrix. Results: There were 782,908 WCB eligible claims, with an incidence rate for NOAA of 1.6%: 23 occupations and 21 industries had a significantly increased risk. Isocyanates (OR 1.54: 95% CI 1.01-2.36) and exposure to mixed agricultural allergens (OR=1.59: 95% CI 1.17-2.18) were related to NOAA overall, as were exposures to cleaning chemicals in men (OR=1.91:95% CI 1.34-2.73). Estimates of the number of cases of occupational asthma suggested a range of 4% to about half for the proportion compensated. Conclusions: Data linkage of administrative records can demonstrate under-reporting of occupational asthma and indicate areas for prevention.

Introduction: Our main objective was to estimate smoking prevalence as well as sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of self-reported smoking among pregnant women in Edmonton, Canada, at 15-16 weeks of gestation. Methods: We used serum samples to assemble a cohort of pregnant women who underwent an optional second-trimester screening for chromosomal and developmental anomalies. We determined cotinine concentrations for 92 self-reported smokers (11% of the cohort) and for 285 self-reported nonsmoking mothers, using adapted urinary cotinine assay. Self-reports were collected at the time of delivery. In a validation study, serum cotinine was determined for known smokers and nonsmokers and used, within a Bayesian statistical framework, to define the distribution of cutoffs that differentiate true smokers from nonsmokers. This distribution of cutoffs was used to construct multiple two-by-two tables to obtain the distribution of sensitivity, specificity, PPV, NPV, and prevalence. Results: Sensitivity was poor (M = 47.4%, SD = 17.3%), but specificity was nearly perfect (M = 94.9%, SD = 1.1%). PPV (M = 66.6%, SD = 11.7%) was smaller than NPV (M = 84.7%, SD = 14.3%). In our sample, the prevalence of true smoking at 15-16 weeks of gestation was described by a skewed distribution with a mean of 21.6% (SD = 13.8%) and a median of 16.6%. Discussion: The strength of the present study includes blinding of subjects to the intention to test their sera for a biomarker of smoking. A limitation was the use of a nonrandom sample restricted to pregnancies that resulted in live births. We discuss data collection methods that would elicit more accurate smoking histories from pregnant women.