Health practitioner survey: research priorities and themes
| Theme | In children and young people with bronchiectasis (unrelated to cystic fibrosis) | Mean score |
| Finding new ways to improve treatment | When 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 diagnosis | What 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 exacerbations | What 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 monitoring | What 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 bronchiectasis | Do 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 | |
| Other | What are the healthcare costs of bronchiectasis management across the world? | 3.19 |