Health practitioner survey: research priorities and themes

ThemeIn children and young people with bronchiectasis (unrelated to cystic fibrosis)Mean score
Finding new ways to improve treatmentWhen and how (antibiotic, dose, regimen, route (intravenous, oral or inhaled/nebulised) and duration) should pathogens other than Pseudomonas aeruginosa be eradicated, and do patient outcomes improve afterwards?4.27
What are the optimal and most cost-effective airway clearance techniques?4.26
When and how (antibiotic, dose, regimen, route (intravenous, oral or inhaled/nebulised) and duration) should P. aeruginosa be eradicated, and do patient outcomes improve afterwards?4.18
What is the best antibiotic, dose, regimen and duration for long-term oral antibiotic therapy in patients with bronchiectasis (according to the presence or absence of P. aeruginosa or other pathogens)?4.17
What are the indications of oral versus inhaled/nebulised long-term suppressive antibiotic treatment?4.14
When should airway clearance techniques be started in patients with bronchiectasis, and how often should it be done during the stable state and for exacerbations?4.09
What is the impact of long-term antibiotic therapy on anti-microbial resistance?4.03
What is the role of different mucoactive agents (e.g. inhaled hypertonic or isotonic saline, mannitol, oral erdosteine or N-acetyl cysteine)?4.03
What are the simple, reliable microbiological tests for determining lower airway infection?3.90
What are the most efficient clinical trial designs and measurable outcomes?3.83
What are the key factors leading to P. aeruginosa acquisition and infection?3.78
Which clinical and microbiological factors affect macrolide efficacy?3.71
What is the role of surgery (segmentectomy, lobectomy or pneumonectomy) and when should it be undertaken?3.62
What is the role of long-term inhaled corticosteroids?3.53
What is the role of non-pharmacological, non-airway clearance technique-based therapeutics, such as singing exercise, wind instruments and yoga during stable states and acute exacerbations?3.45
What is the best model/approach for transferring an adolescent with bronchiectasis to adult services?3.42
Finding new ways to improve diagnosisWhat are the baseline investigations to identify underlying aetiologies of bronchiectasis?3.98
What clinical factors should be present to trigger referring a child for a chest computed tomography scan to diagnose bronchiectasis?3.87
Improving knowledge and treatment of exacerbationsWhat is the optimal antibiotic therapy (dosage, how many antibiotics, type, oral versus intravenous versus inhaled/nebulised and length of therapy) for an exacerbation of bronchiectasis?4.41
What are the most important factors to prevent acute exacerbations?4.12
How to define acute exacerbations that require additional treatment?4.05
What are the causes of an exacerbation of bronchiectasis?3.84
Which are the best systemic (e.g. blood) or local (e.g. sputum) inflammatory markers for the diagnosis, management and follow-up for an exacerbation?3.82
How should the severity of an exacerbation of bronchiectasis be assessed and what is its impact on long-term outcomes?3.78
What types of biomarker(s) can be used for predicting bronchiectasis exacerbation?3.68
Improving prevention and monitoringWhat are the risk factors and causes of rapid progression of lung disease and poor outcomes (e.g. hospitalisation, lung transplantation and mortality)?4.17
How best to prevent development of bronchiectasis?4.16
Should there be paediatric-focused patient registries?4.06
What are the best and most pragmatic functional tests (such as carbon monoxide diffusing capacity, 6-min walk test, lung clearance index, endurance shuttle walk, incremental exercise tests or accelerometers) as markers for severity of the disease, outcomes and end-points for the clinic?4.00
What are the risk or protective factors for lung function decline in patients with bronchiectasis?3.91
What are the factors that predict radiographic reversibility (on a high-resolution computed tomography scan)?3.84
What is the best approach/score to evaluate radiographic severity?3.82
What types of specific patient education packages, self-management plans and patient support groups improve outcomes?3.81
Should a severity and prognostic score for children that is useful in clinical practice be developed?3.70
What comorbidities are present and how do they influence bronchiectasis severity?3.59
Is cross-infection important, what are the best strategies and is strict patient segregation required?3.59
What types of biomarker(s) can be used to monitor bronchiectasis severity during stable state, so as to define the subgroup who will benefit from more intensive treatment?3.58
Can endo-phenotyping predict severity and outcomes in children?3.54
Understanding mechanisms and biology of bronchiectasisDo different aetiologies and/or comorbidities of bronchiectasis predetermine microbiological characteristics and affect severity, patients’ quality of life and disease progression?3.92
What is the role of viruses, fungi and anaerobes (as single agents and/or polymicrobial infections), during both the stable state and exacerbation, and what is their impact upon patient severity and outcomes?3.85
Is there an increased rate of primary immune defects (e.g. mannose-binding lectin deficiency, common variable immunodeficiency, IgM or IgA deficiency, or complement deficiency)?3.78
What is the incidence and prevalence of different aetiologies of bronchiectasis across the world?3.55
What is the relationship between paediatric and adult bronchiectasis?3.54
What is the importance of host–pathogen–environment interactions?3.53
What are the molecular and cellular mechanisms and pathobiological pathways of bronchiectasis development, exacerbations and progression?3.52
What is the composition and function of the host microbiome, both during the stable state and exacerbations, and does it impact directly disease severity and progression?3.48
What are the genetic and epigenetic findings in patients with bronchiectasis compared to healthy controls, and what is their role in acquisition of specific pathogens and patients’ outcomes?3.39
What is the best experimental model system of bronchiectasis?3.24
OtherWhat are the healthcare costs of bronchiectasis management across the world?3.19