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Objective: To determine whether 4 years of colonization with a novel Australian cluster strain (The 'Hunter' strain) of Burkholderia cepacia (B. cepacia) in cystic fibrosis (CF) patients was associated with more rapid decline in nutritional status and pulmonary function than in non-colonized contemporaries from the same CF clinic.Methodology: A retrospective review of respiratory function and nutritional data from a single multidisciplinary paediatric CF clinic over 4 years (1993-97).Results: Paired spirometry data for 1993 and 1997 were available in 47 patients without (n = 31) and with B. cepacia (n = 16) colonization (mean (+/- SD) ages in 1993: 12.1 years +/- 4.0 vs 12.6 years +/- 6.5; P = 0.83). Their percentage predicted forced expiratory volume in 1 s (FEV1) (94.2% +/- 16.7 vs 85.9% +/- 21.2; P = 0.19) were not significantly different. The averaged annual fall in FEV1 over 4 years was also not significantly different (3.8% +/- 3.8 vs 3.6% +/- 3.7; P = 0.82). Weight percentile (Wt%), height percentile (Ht%) and percentage age weight for height (%WFH) were not significantly different between groups in 1993. By 1997, Wt% (36.7% +/- 25.1 vs 22.3% +/- 19.6; P = 0.04) and Ht% (42.5% +/- 29.6 vs 17.6% +/- 19.4; P = 0.002) but not %WFH (102% +/- 10.0 vs 106% +/- 11.2; P > 0.10) were lower in subjects with B. cepacia.Conclusions: In adolescent CF patients, colonization with the Hunter strain of B. cepacia was associated with a deterioration in some nutritional parameters but not with an accelerated decline in FEV1 over 4 years. As varying pathogenicity of B. cepacia strains may account for differing rates of pulmonary decline, further assessment of the consequences of colonization with certain strains of B. cepacia in CF is needed. [ABSTRACT FROM AUTHOR]

Haemoptysis occurs in up to 25 % of young people with Cystic fibrosis (CF) [1]. We undertook a literature review and described the management approach to haemoptysis in CF between 2010 and 2020 at an Australian tertiary paediatric centre, The Children's Hospital Westmead, Sydney, New South Wales, using a retrospective review of the medical records which identified 67 episodes. Sixty episodes met inclusion criteria, including 31 patients. Using the US CF Foundation guidelines, episodes were classified as scant (53.3 %), moderate (38.3 %) or massive (8.3 %). Fifty-two percent of patients were female, mean age at presentation was 15.4 years (SD+/- 2.4) and 58 % were homozygous for the Fdel508 genotype. Twelve episodes (9 patients) required bronchial artery embolization (BAE). BAE was used in all cases of massive haemoptysis 5/5 (100 %), 6/23 (22 %) episodes of moderate and 1/32 (3 %) episode of scant haemoptysis as an elective procedure for recurrent haemoptysis. Our literature review and institutional experience highlights the need for up-to-date management guidelines in the management of haemoptysis in Cystic Fibrosis. Based on our experience, we provide a proposed algorithm to help guide the management of haemoptysis in CF., Competing Interests: Conflict of Interest The authors have no conflict of interest to disclose., (Copyright © 2022. Published by Elsevier Ltd.)

Introduction: Cystic fibrosis (CF) is a multisystem condition that is complicated by recurrent pulmonary infections requiring aggressive antibiotic treatment. This predisposes the patient to complications such as sensorineural hearing loss, renal impairment, hypersensitivity and the development of antibiotic resistance. Pseudomonas aeruginosa is one of the more common organisms which cause recurrent infections and result in greater morbidity and mortality in people living with CF. Bacteriophages have been identified as a potential alternative or adjunct to antibiotics. We hypothesise that bacteriophage therapy is a safe and well-tolerated treatment in children with CF infected with P. aeruginosa infection in their airways., Methods: This single-arm, open-labelled, non-randomised trial will run for a maximum period of 36 months with up to 10 participants. Adolescents (≥12 years and <18 years of age) who continue to shed P.aeruginosa (within 3 months of enrolment) despite undergoing eradication therapy previously, will be considered for this trial. Non-genetically modified bacteriophages that have demonstrated obligate lytic activity against each of the study participants' P. aeruginosa strains will be selected and prepared according to a combination of established protocols (isolation, purification, sterility testing and packaging) to achieve close to good manufacturing practice recommendations. The selected bacteriophage will be administered endo-bronchially first under direct vision, followed by two times a day nebulisation for 7 days in addition to standard CF treatment (intravenous antibiotics, physiotherapy to be completed as inpatient for 10-14 days). Safety and tolerability will be defined as the absence of (1) fever above 38.5°C occurring within 1 hour of the administration of the nebulised bacteriophage, (2) a 10% decline in spirometry (forced expiratory volume in 1 s %) measured preadministration and postadministration of the first dose of nebulised bacteriophage. Clinical reviews including repeat sputum cultures and spirometry will be performed at 3, 6, 9 and 12 months following bacteriophage treatment., Ethics and Dissemination: Our clinical trial is conducted in accordance with (1) good clinical practice, (2) Australian legislation, (3) National Health and Medical Research Council guidelines for the ethical conduct of research., Trial Registration Number: Australia and New Zealand Clinical Trial Registry (ACTRN12622000767707)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

The role of the expert witness is clear in the eyes of the court but that understanding is less consistent for the medical professionals who may be called on in this capacity. The fundamental key is the clinician's purpose in using their experience, knowledge and training as they provide the court with an unbiased assessment of the clinical issues that have arisen in the case. Using the Australian context, this article provides guidance for clinicians who are asked to provide the court with their expertise through written and spoken representations, drawing upon experience and the use of the "Expert Witness Code of Conduct"., (Copyright © 2021. Published by Elsevier Ltd.)

Aim: To describe the respiratory and nutritional supportive care and hospitalisations required in the real-world scenario in children with SMA type 1 treated with nusinersen., Methods: Single-centre observational cohort study of children with SMA1 commencing nusinersen from November 2016 to September 2018. Motor, respiratory and nutritional clinical characteristics and management are described from initiation of nusinersen for a minimum of two years., Results: Nine children (5 females, 4 males), median age 10.7 months (range 2.7-181.2) commenced treatment with nusinersen and outcomes were assessed over a total of 270.5 patient months and 209 hospital admissions. Supportive care in newly-diagnosed patients (n = 7) included gastrostomy insertion (n = 4) and commencement of noninvasive ventilation (n = 4) at an average of 8.3 and 4.5 months after diagnosis, respectively. The annualised hospitalisation rate was 9.3/patient/year, average length of stay (LOS) of 3.3 days (SD = 5.6). Children with two SMN2 copies required more gastrostomies (p < 0.05) and had more frequent admissions (p < 0.05). Number of total admissions halved from the first to the second year of treatment in all patients (p < 0.005)., Interpretation: Children with treated SMA1 experienced considerable respiratory and bulbar comorbidities, necessitating substantial respiratory and nutritional supportive care. Proactive respiratory and nutritional surveillance and management is essential in SMA1 patients treated with nusinersen., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

The widely diverse impacts of SAR-CoV-2 infection resulting in the COVID-19 pandemic cannot be held in more stark relief when contrasting the devastating impact upon China, Italy, Great Britain, America and Brazil with the considerably milder course in the geographically isolated countries of Australia and New Zealand and the densely populated Vietnam. Children in the Asia-Pacific region, as with children all over the world to date, have fared better than older adults. Other countries in the Asia-Pacific region, including Indonesia and India have struggled to deal with the pandemic because of a lack of health infrastructure, inability to provide sufficient testing and isolation and widespread poverty. This article will provide a snapshot of the impact of COVID-19 upon countries in the Asia-Pacific region in the six months since the first case of the novel zoonotic coronavirus infection appeared in China., (Copyright © 2020. Published by Elsevier Ltd.)

The sobering reality of the COVID-19 pandemic is that it has brought people together at home at a time when we want them apart in the community. This will bring both benefits and challenges. It will affect people differently based upon their age, health status, resilience, family support structures, and socio-economic background. This article will assess the impact in high income countries like Australia, where the initial wave of infection placed the elderly at the greatest risk of death whilst the protective measures of physical distancing, self-isolation, increased awareness of hygiene practices, and school closures with distance learning has had considerable impact on children and families acutely and may have ramifications for years to come., (Crown Copyright © 2020. Published by Elsevier Ltd. All rights reserved.)

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 SpO 2 during overnight oximetry to be 93-95% with less than 5% of total recording time to be below 90% SpO 2 . 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., (© 2020 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.)

Background: The impact of universal 13-valent pneumococcal conjugate vaccine immunization on pediatric empyema rates and pathogens in Australia is not known. We aimed to describe empyema epidemiology, clinical characteristics and treatment during an 8-year period., Methods: A retrospective study between 2011 and 2018 of empyema cases admitted to a large pediatric referral hospital, for management with either pleural drainage and fibrinolytics or surgical intervention., Results: There were 195 cases in 8 years. Empyema incidence and ICU admission rates significantly increased during the study with a peak incidence of 7.1/1000 medical admissions in 2016 (χ for trend of incidence 37.8, P < 0.001 and for ICU admissions 15.3, P < 0.001). S. pneumoniae was the most common pathogen (75/195, 39%) with serotype 3 the most detected (27/75: 27%). S. pyogenes compared with S. pneumoniae had significantly fewer days of fever before admission (3.9 vs. 6.4, mean difference 2.4, 95% CI: 0.84-4.08, P = 0.003) and higher proportion requiring direct ICU admission (6/75; 8% vs. 7/15; 47%, P < 0.001). Compared with S. pneumoniae, cases with no pathogen detected by culture or PCR had fewer days of fever post intervention (4.4 vs. 7.4 days, mean difference 2.7 days, P = 0.002). S. aureus occurred more commonly in infants (10/25; 40% vs. 1/75; 1%, P < 0.001) and children of indigenous background (5/25; 20% vs. 1/75; 1%, P < 0.001) compared with S. pneumoniae., Conclusions: We report increasing rates of pediatric empyema with higher proportions requiring ICU treatment. The most common pathogens detected were S. pneumoniae, S. aureus and S. pyogenes. Despite high 13-valent pneumococcal conjugate vaccine coverage, serotype 3 was the most common S. pneumoniae serotype identified.

Purpose: Oesophageal atresia and tracheo-oesophageal atresia require surgical repair in early infancy. These children have significant disease-related morbidity requiring frequent radiological examinations resulting in an increased malignancy risk., Methods: A single-centre, retrospective review was performed of radiation exposure in children with OA/TOF born 2011-2015. Medical records were reviewed to determine the number and type of imaging studies involving ionising radiation exposure enabling the calculation of the estimated effective dose per child over the first year of life., Results: Forty-nine children were included. Each child underwent a median of 19 (IQR 11.5-35) imaging studies, which were primarily plain radiography (median = 14, IQR 7-26.5). The overall median estimated effective dose per patient was 4.7 (IQR 3.0-9.4) mSv, with the majority of radiation exposure resulting from fluoroscopic imaging (median 3.3 mSv, IQR 2.2-6.0). 'Routine' postoperative oesophagrams showed no leak in 35/36 (97%) with the remaining study showing an insignificant leak that did not alter management., Conclusions: Careful consideration should be given to the use of imaging in OA/TOF to minimise morbidity in these vulnerable infants. Oesophagrams in children without the symptoms of anastomotic leak or stricture should be discontinued. Standardisation of monitoring protocols with regard to radiation exposure should be considered.

Cannabis, like the mythic shape-shifter, presents in various guises, morphing with the perspective and context of the observer. Arguments about cannabis are confused by a myriad of debates-medical, social, ethical and political-as if a single conceptual umbrella can capture the variety and granularity of marijuana-related issues. This paper responds to marijuana use as it is commonly practised by youth in Australia. It has little to say about synthetic cannabinoids, specific medicinal cannabinoids, or medicinal properties of marijuana. We address those adolescents genetically and environmentally vulnerable to mental illness, with specific emphasis on indigenous and neurodevelopmentally impaired young people who show patterns of usage and response very different to adults and more resilient members of the population. Specifically, the practice of mixing tobacco with marijuana by aboriginal youth, and the resultant coalition of dependencies, will likely presage a rise in pulmonary and central nervous system pathology over the coming decades. Aboriginal youth begin using earlier, persist longer, and take greater quantities of cannabis than non-indigenous youth. This paper recommends practical interventions to reduce the multiple health consequences of chronic cannabis use in young people, especially indigenous young people., (Copyright © 2016. Published by Elsevier Ltd.)

Aim: Empyema can be managed conservatively with intravenous antibiotics or invasively with a drain inserted under image guidance or via surgical evacuation. Both approaches are successful but comparisons of the method of drainage are few. This study compared clinical outcomes for empyema in previously well children from a single centre over a 12 year period., Methods: A retrospective analysis of cases over 12 years from the Children's Hospital at Westmead in Sydney was undertaken. Ethics committee approval was obtained., Results: Seventy two cases were identified from medical records, 12 cases were excluded and 60 cases remained. The mean age was 4.7±4.3 years and there was a slight male preponderance. Treatment was divided into surgical management with a large bore drain alone [n=25] and minimally invasive management with the use of a "pigtail catheter" and intrapleural fibrinolytic ["Urkoinase"][n=35]. At presentation the mean heart rate and respiratory rate were not statistically different. The median (range) number of doses of urokinase was 5.66 doses (1-12). More fluid was drained with the use of urokinase [594 ml (25-4575 ml) vs. 195 ml (10-1426 ml); p=0.006], but this did not influence the rate of resolution of fever or the length of hospital stay. A pathogen was isolated in 42.9% of the urokinase group and 68% of the surgical group which approached statistical significance [p=0.054]., Conclusions: Both large bore surgical drains and "pigtail catheter" drains with the instillation of urokinase lead to similarly favourable treatment outcomes. Either treatment could be recommended depending on local expertise and preferences., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

A clinician and the chair of the hospital drug committee debate the merits of the use of palivizumab as prophylaxis against respiratory syncytial virus infection for a preterm baby with oxygen-dependent, chronic lung disease and discuss the issue of public funding of palivizumab. An ethicist comments on ethical aspects of the debate about cost-effectiveness., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

The prevalence of severe obesity and associated co-morbidities is increasing in adolescence. Although support for long-term whole-of-family lifestyle change is the mainstay of paediatric obesity treatment, there is increasing recognition of the place of other therapies,including bariatric surgery, in the management of severely obese adolescents. While there are rising numbers of reports of bariatric surgery in adolescents, there are as yet no Australian or New Zealand recommendations available to guide decisions as to which adolescents should receive such surgery and how they should best be managed. This paper presents a summary of the recommendations that are contained within the full position paper developed on behalf of the Royal Australasian College of Physicians Paediatric Policy and Advocacy Committee Working Party on Bariatric Surgery for Adolescents, working in conjunction with the Australia and New Zealand Association of Paediatric Surgeons and the Obesity Surgery Society of Australia and New Zealand.

Chronic neonatal lung disease (CNLD) is defined as a supplemental oxygen requirement beyond 36 weeks' postmenstrual age, with more severely affected infants requiring oxygen beyond a full-term-equivalent age. Low-flow supplemental oxygen facilitates discharge from hospital of infants with CNLD who develop hypoxia in air. There is a lack of data on the most appropriate minimum mean target oxygen saturation (Spo(2)) level. Reflecting a variety of clinical practices and infant comorbidities (frequency of oxygen desaturation, presence of pulmonary hypertension, retinopathy of prematurity, and adequacy of growth), the minimum mean target range for Spo(2) during overnight oximetry should be 93%-95%. The effect of supplemental oxygen on carbon dioxide retention should be considered before deciding on an oxygen flow. Most infants with CNLD are not ready for discharge until their supplemental oxygen requirement is < or = 0.5 litres per minute delivered through a nasal cannula. The safety of short-term disconnection from supplemental oxygen should be assessed before discharge. Assessment of oxygenation during sleep with continuous overnight oximetry or polysomnography is recommended when weaning infants from supplemental oxygen. Discontinuation of oxygen therapy is based on clinical assessments and documentation of adequate oxygenation in room air. There is limited objective evidence on which to base recommendations.