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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.)

Asthma is among the most common medical conditions affecting children and young people, with adolescence a recognised period of increased risk, overrepresented in analyses examining recent increasing asthma mortality rates. Asthma may change significantly during this period and management also occurs in the context of patients seeking increased autonomy and self-governance whilst navigating increasing academic and social demands. A number of disease factors can destabilise asthma during adolescence including: increased rates of anaphylaxis, anxiety, depression, obesity, and, in females, an emerging resistance to corticosteroids and the pro-inflammatory effects of oestrogen. Patient factors such as smoking, vaping, poor symptom recognition, treatment non-adherence and variable engagement with health services contribute to difficult to treat asthma. Significant deficiencies in the current approach to transition have been identified by a recent EAACI task force, and subsequent asthma-specific recommendations, published in 2020 provide an important framework moving forward. As with other chronic conditions, effective transition programmes plan ahead, engage with adolescents and their families to identify the patients' management priorities and the current challenges they are experiencing with treatment. Transition needs may vary significantly across asthma patients and for more complex asthma may include dedicated transition clinics involving multidisciplinary care requiring input including, amongst others, allergy and immunology, psychological medicine, respiratory physicians and scientists and nurse specialists. Across different global regions, barriers to treatment may vary but need to be elicited and an individualised approach taken to optimising asthma care which is sustainable within the local adult healthcare system., (Copyright © 2021. Published by Elsevier Ltd.)

Paediatric spontaneous pneumothorax (PSP) management continues to lack paediatric-specific guideline recommendations. There have been increasing reports of paediatric retrospective case studies supplemented by important well designed RCT (predominantly) adult studies. Taken together, these suggest that conservative management may have an increasing role to play in the management of PSP and that aspiration may have limited utility as a first line intervention. Our local experience, as part of a multicentre retrospective analysis and subsequent audit of management since, corroborates recent published data: it highlights an increasing trend towards conservative management in spontaneous pneumothorax with similar rates of recurrence, compared to intervention, and low use of aspiration with similarly low success rates. We have therefore updated our local practice guidelines and share these with readers. Specifically, we have removed aspiration in the management of primary spontaneous pneumothorax and reserved intervention for children who are clinically unstable or show evidence of increasing air leak irrespective of pneumothorax size. Whilst the success of this change in clinical practice will need to be reviewed in the next 5-10 years, the overall low incidence of the condition, demands a multicentre, and probably multinational, collaborative approach to allow the best chance of obtaining definitive evidence to guide clinical paediatric management., (Copyright © 2021. Published by Elsevier Ltd.)

Non-tuberculous mycobacterial (NTM) (especially M. abscessus complex) infections pose a considerable challenge in the management of lung disease in patients with cystic fibrosis (CF). The apparent increase in prevalence is likely multifactorial. Emergent evidence of patient-to-patient transmission and isolation of highly resistant strains is a concern for all CF centers around the world. Treatment is often long and burdensome with multiple agents. Treatment side effects are frequent and can cause significant morbidity. Although consensus guidelines provide some direction, many units are faced with the challenges of: finding drug combinations for highly resistant strains; dealing with interruptions of treatment; discussing additional facilitating procedures in the form of gastrostomy and long-term vascular access devices; as well as supporting families emotionally and psychologically through the process., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

One of the major complications of Cystic Fibrosis (CF) is CF-Related Diabetes (CFRD), which increases in incidence with age, from 1-2% below the age of 10 years to ∼20% of adolescents and 40-50% of adults. Multiple guidelines have been published over the last few years for the diagnosis and management of CFRD, from the American Diabetes Association (ADA) / US Cystic Fibrosis Foundation, International Society for Pediatric and Adolescent Diabetes (ISPAD) and the Thoracic Society of Australia and New Zealand-Australian Diabetes Society. However, little is published about the particular issues involved in transition of patients with CFRD from paediatric to adult care, nor the issues concerning the development of CFRD during the transition period. This document seeks to provide assistance to physicians, dieticians, nurses, diabetes educators, CF patients and their families by outlining the issues surrounding CFRD during transition from paediatric to adult care., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Obesity has complex and incompletely understood effects upon the respiratory system in childhood, which differs in some aspects to those seen in adults. There is increasing evidence that excess adiposity will impact negatively upon static and dynamic respiratory function as measured through lung volumes, lung compartment mechanics, measures of airway function and exercise capability to varying degrees. Further information is needed to better understand the effects in children, and the importance of onset and duration of obesity on subsequent outcomes. Consensus about how best to express adiposity is also an essential part of this process and fat distribution is another important factor. From a clinical standpoint this creates challenges in distinguishing a deconditioned obese young person from a non-atopic asthmatic because of symptom overlap and lung function testing results, including responses seen during airway challenges. There is evidence to support the role of weight loss in achieving normalisation of lung function parameters, but as always with obesity there are enormous challenges in realising this goal for many subjects., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Allergic bronchopulmonary aspergillosis [ABPA] complicates cystic fibrosis in around 10% of teenagers. Systemic corticosteroids are the mainstay of treatment but with on-going use are associated with significant side effects prompting consideration of steroid sparing therapies. The clinical courses over ten years of two children with CF complicated by steroid dependent chronic ABPA and its successful treatment with monthly omalizumab are reported., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

The exact incidence of paediatric primary spontaneous pneumothorax (PSP) is unclear, although PSP incidence in the general population is between 6 and 18 per 100,000. PSP has been reported throughout the paediatric age range. A potential genetic predisposition for familial cases has been recently identified. Whilst there is universal consensus on the management of tension pneumothorax, lack of agreement and consistency exists across a wide range of management issues for other aspects of PSP management. Paediatric PSP may have a higher recurrence rate than adult PSP, and the presence of apical lung cysts or bullae is not predictive of recurrence. The decision for surgical intervention should be based on documented recurrence. There is a lack of paediatric evidence to guide management decisions, and extrapolation of predominantly adult data to younger age groups should not be encouraged. Given the relatively low apparent incidence, a multicentre approach to future research is required in order to generate the evidence required for informed management of PSP in children.

The incidence of congenital diaphragmatic hernia (CDH) may be as high as 1 in 2000. Over the past two decades, antenatal diagnosis rates have increased, the pathophysiology of CDH has become better understood, and advances in clinical care, including foetal surgery, have occurred. However, there remains a paucity of randomised controlled trials to provide evidence-based management guidelines. Reports of improved survival rates appear to be confined to a select subset of CDH infants, surviving to surgical repair, while the overall mortality, at over 60%, appears to be unchanged, largely due to the often forgotten 'hidden mortality' of CDH. The significant long-term morbidity in surviving infants has become apparent, and the need for long-term multidisciplinary follow up established. A total of 10% of cases may present later in life, and misdiagnosis on initial chest X-ray may lead to significant morbidity.