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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.

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.

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.

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.

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.

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

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.

The burden of disease in children with non-cystic fibrosis (non-CF) bronchiectasis is unknown. Our study aimed to identify the determinants of quality of life (QOL) and parental mental health in this group of patients and their parents and to evaluate the effect of exacerbations on these parameters.Parents of 69 children (median age 7 years) with non-CF bronchiectasis prospectively completed two questionnaires (parent-proxy cough-specific quality of life [PC-QOL] and the Depression, Anxiety, and Stress Scale [DASS]) at stable and exacerbation states. Data on clinical, investigational, and lung function parameters were also collected.During the stable state, the median interquartile range (IQR) PC-QOL score was 6.5 (5.3-6.9) and the DASS 21-item questionnaire score was 6 (0-20). Being of a young age correlated with a worse QOL (r(5) = 0.242, P = .04) but radiologic extent, lung function, underlying cause, environmental tobacco smoke exposure, and chronic upper-airway disease did not influence these scores. Exacerbations caused significant worsening in the PC-QOL scores (median [IQR], 4.6 [3.8-5.4]; P = .001) and DASS scores (median [IQR], 22 [9-42]; P.001; 38% with elevated anxiety, 54% with abnormal depression/stress scores during exacerbation). The presence of viral infection, hypoxia, and hospitalization did not influence the exacerbation PC-QOL and DASS scores.There is a significant burden of disease, especially during exacerbation, on parents of children with bronchiectasis. Prevention, early detection, and appropriate treatment of exacerbations are likely to reduce psychologic morbidity in this group.

Background: Infection and inflammation are important in the pathogenesis of bronchiectasis. However, there are few published data describing the lower airway microbiology and cellularity in children. Methods: Children with non-cystic fibrosis (CF) bronchiectasis who underwent bronchoalveolar lavage (BAL) within 4 weeks of diagnosis were identified by a retrospective patient-record review. The effects of infection (>= 10(5) colony-forming units of respiratory bacteria/ml; or detectable Pseudomonas aeruginosa; mycobacteria, fungi, mycoplasma, or respiratory viruses) on airway cellularity and the impact of age, gender, indigenous status, immune function, radiographic involvement and antibiotic usage on infection risk were evaluated. Results: Of 113 children [ median age 63 months (IQR 32-95)] with newly diagnosed bronchiectasis, 77 (68%) had positive BAL cultures for respiratory bacterial pathogens. Haemophilus influenzae was most commonly detected, being present in 53 (47%) BAL specimens. P. aeruginosa was found in just 7 (6%) children, five of whom had an underlying disorder, while mycobacterial and fungal species were not detected. Respiratory viruses were identified in 14 (12%) children and Mycoplasma pneumoniae in two others. Overall, 56 (49.5%) children fulfilled our definition of a lower airway infection and of these, 35 (63%) had more than one pathogen present. Compared to children without infection, children with infection had higher total cell counts (610 vs. 280 X 10(6)/L), neutrophil counts (351 vs. 70 X 10(6)/L), and neutrophil percentages (69% vs. 34%). Age at diagnosis was most strongly associated with infection. Conclusions: BAL microbiology of children with newly diagnosed bronchiectasis is dominated by H. influenzae. In the absence of CF, isolation of P. aeruginosa may suggest a serious comorbidity in this group. Airway neutrophilia is common, especially with higher bacterial loads. Pediatr Pulmonol. 2012; 47:300-307. (C) Wiley Periodicals, Inc.

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.To formulate a clinically useful definition of pulmonary exacerbation for pediatric non-CF bronchiectasis.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.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%).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.

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.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.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.In the pediatric age group, the airway is significantly smaller than the adjoining vessel. Using the radiologic criteria of BA ratio1 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.

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.Fifty-two children withor = 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.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.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.

Bronchiectasis is increasing recognised as a co-morbidity in many respiratory illness. Anti inflammatory drugs may reduce the inflammatory cascade and thus reduce symptoms and slow long term pulmonary decline.To assess the role of non steroid anti inflammatory drugs (NSAIDs) on symptom control and natural history of the disease in children and adults with bronchiectasis.The Cochrane Central Register of Controlled Trials (CENTRAL), the Cochrane Airways Group Specialised Register, MEDLINE and EMBASE databases were searched by the Cochrane Airways Group up to December 2006.Only randomised controlled trials were considered. Patients with radiological or clinical evidence of bronchiectasis were included. Patients with Cystic Fibrosis were excluded.The titles, abstracts and citations were independently reviewed by two reviewers to assess potential relevance for full review. No eligible trials were identified and thus no data were available for analysis.No randomised or controlled trials were found.There are no randomised controlled that examined the effect of oral NSAIDs in patients with bronchiectasis. In view of some benefit shown by inhaled NSAIDs in bronchiectasis, RCTs are clearly needed to study the beneficial effect of oral NSAIDs in patients with bronchiectasis.