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Mycobacterium abscessus is an emerging pathogen in cystic fibrosis (CF) lung disease. Hospital transmission of M. abscessus has been described. This paper details the investigation into possible cross-transmission of M. abscessus locally at our paediatric hospital CF centre, and the subsequent infection control response.Whole genome sequencing (WGS) of M. abscessus respiratory isolates with epidemiological linkage analysis using hospital electronic medical records.6.7% (22/328) of CF patients had M. abscessus isolated from respiratory specimens. WGS revealed a cluster of three patients with genomically related isolates that differed by7 single nucleotide polymorphisms (SNPs), suggesting a shared recent ancestor and probable cross-transmission. Epidemiological investigation revealed multiple potential crossovers between patients with genomically similar M. abscessus isolates.Cross-infection of NTM occurs in CF hospital patients. Hospital infection control practices should be upgraded to reflect this. Consensus is needed between centres.

The traditional model of cystic fibrosis (CF) care revolves around regular (usually three monthly) in person appointments with the multidisciplinary CF team at specialised CF care centres. This article is protected by copyright. All rights reserved.

Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner. The aim of this review is to provide evidence-based information and clinical algorithms to guide the nutritional and gastrointestinal management of patients with CF.

Introduction In this cohort study spanning an 18-year period, we evaluated the prevalence and associated mortality rate of epidemic strains of pseudomonas aeruginosa (PsA), especially Australian Epidemic Strain Type 1 (AES1), in a pediatric cystic fibrosis center practicing cohort segregation and early PsA eradication. Methods Cohort segregation was introduced in January 2000. PsA clonal strain was determined by pulse-field-gel-electrophoresis (PFGE) at the time of routine collection of airway specimens. Children with PsA underwent eradication treatment with anti-pseudomonal antibiotics over 2-3 months. We analyzed changes in prevalence and mortality from 1999 to 2016. Results The prevalence of AES1 declined from 69 (20%) in 1999 to 16 (5.4%) in 2006, to 1 (0.4%) in 2016. The prevalence of PsA overall diminished less over the same period, from 128 (37%) patients in 1999 to 57 (23%) in 2016. New acquisition of AES1 became less common over time, with no new cases identified from 2011. Those who contracted AES1 had a greater risk of death than those who did not (Odds Ratio 4.9, 95%CI 2.5-9.6). Patients with other AES PsA types were uncommon (AES2 n = 5, AES5 n = 2, AES14 n = 3, AES19 n = 1). Conclusions Cohort segregation was associated with reduction in AES1 prevalence ascertained by PFGE surveillance for patients in a single large pediatric cystic fibrosis center. Other alterations in practice such as early eradication treatment may also have contributed to reduced PsA prevalence. These factors combined with the transition of chronically infected patients over time to adult centers has eliminated AES1 from our clinic, with an accompanying mortality decrease.

Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner. The aim of this review is to provide evidence-based information and clinical algorithms to guide the nutritional and gastrointestinal management of patients with CF.

Aim: Identifying predictors of pulmonary exacerbations in cystic fibrosis (CF) allows for earlier detection and timely treatment, possibly reducing negative impact. Methods: A prospective longitudinal analysis of children aged 0-7 years in the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) study between 2005 and 2015. Inflammatory markers from routine annual bronchoalveolar lavage fluid (BALF) were examined against pulmonary exacerbations (hospitalisation for intravenous antibiotics) in the 6 months post-BALF. Kaplan-Meier curves were created and Weibull regression was used, adjusting for annually repeated measurements in the same patients. Results were adjusted for known risk factors of exacerbations. Results: 1200 BALF samples were taken from 322 children with a total of 492 person-years follow-up. 226 pulmonary exacerbations were recorded. 20% of children were admitted in the 6 months following BALF and in those the median time to admission was 19 days (interquartile range 9-83 days). The risk for a pulmonary exacerbation was increased if neutrophil elastase was detectable (hazard ratio (HR) 3.76, 95%-CI 2.16-3.95, p Conclusion: BALF inflammatory markers are significant predictors of pulmonary exacerbations in young children with CF.

Sarcoidosis is a rare disease, especially in the preschool age group where it usually presents with eye, joint, and skin involvement sparing the lungs. Multiple treatment regimes have been described with oral prednisolone being the usual first line treatment. We describe a case of a 4-year-old boy presenting with pulmonary sarcoidosis with no other organ involvement. The child was successfully managed with pulse intravenous methylprednisolone followed by oral prednisolone and has had a good outcome. This case reports a rare presentation of preschool sarcoidosis as well as the novel and successful use of pulse methylprednisolone in paediatric pulmonary sarcoidosis.

A 20-month-old girl weighing 12 kg presented with 2 weeks of worsening dry cough and wheeze. Aside from respiratory symptoms she was otherwise well, without fever or weight loss. There was no history of foreign body inhalation or choking. She had no significant personal or family medical history, and her immunisations were up to date. Prior to admission she had been treated for 1 week for a community-acquired pneumonia with oral amoxicillin and roxithromycin, as well as for a first presentation asthma exacerbation with oral prednisolone (2 mg/kg) and inhaled salbutamol, three hourly. Despite these interventions no clinical improvement had been noted. On examination, her respiratory rate and oxygen saturations were normal. She had mild respiratory distress, a loud biphasic wheeze and a prolonged expiratory phase. A chest X-ray (CXR) demonstrated hyperinflation of the right lung (figure 1A). Given the suspicion of a foreign body obstructing a right-sided airway, a rigid bronchoscopy was performed which showed an isolated submucosal lesion in the right main bronchus causing airway narrowing (figure 1B). To further characterise the lesion, a CT of the chest was performed, which demonstrated a subcarinal mass (figure 1C), causing significant extrinsic compression of the distal right main bronchus and bronchus intermedius. There was no intraluminal perforation of the mass. Figure 1 (A) Chest X-ray at presentation showing hyperinflation of right lung. (B) Image from rigid bronchoscopy showing occlusion of right main bronchus by submucosal lesion. (C) Initial CT scan showing subcarinal mass compressing the right main bronchus. (D) Lymph node biopsy demonstrating necrosis with nuclear debris surrounded by a thin layer of caseation and epithelioid cells with occasional Langhans giant cells. (E) Repeat CT scan showing reduction in size of mass with ongoing right main bronchus compression. The most likely differential diagnoses for a subcarinal mass in this patient include a neoplastic …