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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

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.

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.

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.

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.

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.

Background Bronchiectasis is increasingly recognized as a major cause of respiratory morbidity especially in developing countries and in some ethnic populations of affluent countries. It is characterized by irreversible dilatation of airways, generally associated with chronic bacterial infection. Medical management largely aims to reduce morbidity by controlling the symptoms and by preventing the progression of bronchiectasis. Objectives To evaluate the efficacy of inhaled corticosteroids (ICS) in children and adults with bronchiectasis (a) during stable bronchiectasis; and for reducing; (b) the severity and frequency of acute respiratory exacerbations and (c) long term pulmonary decline. Search strategy The Cochrane Register of Controlled Trials (CENTRAL), the Cochrane Airways Group Specialized Register Collaboration and Cochrane Airways Group, MEDLINE and EMBASE databases were searched by the Cochrane Airways Group. The latest searches were performed in September 2007. Selection criteria All randomised controlled trials comparing ICS with a placebo or no medication. Children and adults with clinical or radiographic evidence of bronchiectasis were included, but patients with cystic fibrosis (CF) were excluded. Data collection and analysis Results of searches were reviewed against pre-determined criteria for inclusion. Main results There were no paediatric studies. Six adult studies fulfilled the inclusion criteria. Of the 303 randomised, 278 subjects completed the trials. In the short term group (ICS for less then 6 months duration), adults on huge doses of ICS (2g per day of budesonide equivalent) had significantly improved forced expiratory volume in the first second (FEV(1)), forced vital capacity (FVC), Quality of life (QOL) score and sputum volume but no significant difference in peak flow, exacerbations, cough or wheeze, when compared to adults in the control arm (no ICS). When only placebo-controlled studies were included, there were no significant difference between groups in all outcomes examined (spirometry, clinical outcomes of exacerbation or sputum volume etc). The single study on long term outcomes showed no significant effect of inhaled steroids in any of the outcomes. Authors' conclusions The present review indicates that there is insufficient evidence to recommend the routine use of inhaled steroids in adults with stable state bronchiectasis. While a therapeutic trial may be justified in adults with difficult to control symptoms and in certain subgroups, this has to be balanced with adverse events especially if high doses are used. No recommendation can be made for the use of ICS in adults during an acute exacerbation or in children (for any state) as there were no studies.

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.