J.S. Elborn
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View article: Symptoms, risk of future exacerbations, and response to long-term macrolide treatment in bronchiectasis: an observational study
Symptoms, risk of future exacerbations, and response to long-term macrolide treatment in bronchiectasis: an observational study Open
EU, European Federation of Pharmaceutical Industries and the Associations Innovative Medicines Initiative Inhaled Antibiotics in Bronchiectasis and Cystic Fibrosis Consortium, European Respiratory Society.
View article: Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promotes Disease Persistence.
Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promotes Disease Persistence. Open
The dynamics describing the vicious cycle characteristic of cystic fibrosis (CF) lung disease, initiated by stagnant mucus and perpetuated by infection and inflammation, remain unclear. Here we determine the effect of the CF airway milieu,…
View article: Longitudinal changes in the cystic fibrosis airway microbiota with time and treatment
Longitudinal changes in the cystic fibrosis airway microbiota with time and treatment Open
View article: Treatment decision-making for using CFTR modulator therapy in patients with cystic fibrosis
Treatment decision-making for using CFTR modulator therapy in patients with cystic fibrosis Open
Behavioral characteristics, attitudes toward CFTRm therapies, and experience with CFTRm therapies impact physician CFTRm prescribing more than individual disease factors.
View article: Molecular endotyping in people with bronchiectasis based on response to antibiotic treatment: iBEST study
Molecular endotyping in people with bronchiectasis based on response to antibiotic treatment: iBEST study Open
Background Culture-independent molecular techniques could potentially be used to measure microbiological efficacy in response to antibiotic treatment and improve understanding of the role of the airway microbiota in determining response in…
View article: Cystic Fibrosis Microbiome-directed Antibiotic Therapy Trial in Exacerbations Results Stratified (CFMATTERS): results of a multicentre randomised controlled trial
Cystic Fibrosis Microbiome-directed Antibiotic Therapy Trial in Exacerbations Results Stratified (CFMATTERS): results of a multicentre randomised controlled trial Open
Background This study explores the effectiveness and safety of microbiome-directed antimicrobial therapy versus usual antimicrobial therapy in adult cystic fibrosis pulmonary exacerbations. Methods A multicentre two-arm parallel randomised…
View article: Use of inhaled corticosteroids in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)
Use of inhaled corticosteroids in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC) Open
Introduction Current bronchiectasis guidelines advise against the use of inhaled corticosteroids (ICS) except in patients with associated asthma, allergic bronchopulmonary aspergillosis (ABPA) and/or chronic obstructive pulmonary disease (…
View article: Airway total bacterial density, microbiota community composition and relationship with clinical parameters in bronchiectasis
Airway total bacterial density, microbiota community composition and relationship with clinical parameters in bronchiectasis Open
Stability in bacterial density and microbiota diversity, richness, evenness and dominance was observed over time at a population level but considerable fluctuation was apparent in samples from individual patients.
View article: Anxiety, depression, physical disease parameters and health-related quality of life in the BronchUK national bronchiectasis cohort
Anxiety, depression, physical disease parameters and health-related quality of life in the BronchUK national bronchiectasis cohort Open
Background Bronchiectasis is associated with psychological comorbidity and poor quality of life (QoL), yet guidelines lack focus on psychological morbidity. Using data obtained from the BronchUK database (1341 patients), we examined the li…
View article: Automated method of bronchus and artery dimension measurement in an adult bronchiectasis population
Automated method of bronchus and artery dimension measurement in an adult bronchiectasis population Open
Aim Bronchiectasis (BE) is a disease defined by irreversible dilatation of the airway. Computed tomography (CT) plays an important role in the detection and quantification of BE. The aim of this study was three-fold: 1) to assess bronchus–…
View article: Inhibition of Protease–Epithelial Sodium Channel Signaling Improves Mucociliary Function in Cystic Fibrosis Airways
Inhibition of Protease–Epithelial Sodium Channel Signaling Improves Mucociliary Function in Cystic Fibrosis Airways Open
RATIONALE: In cystic fibrosis (CF) a reduction in airway surface liquid (ASL) height compromises mucociliary clearance, favoring mucus plugging and chronic bacterial infection. Inhibitors of the epithelial sodium channel (ENaC) have therap…
View article: Levelling the playing field through the London Network of the UK clinical trials accelerator platform
Levelling the playing field through the London Network of the UK clinical trials accelerator platform Open
View article: Randomised, phase 1/2a trial of ION-827359, an antisense oligonucleotide inhibitor of ENaC
Randomised, phase 1/2a trial of ION-827359, an antisense oligonucleotide inhibitor of ENaC Open
Background Hyperactivity of epithelial sodium channel (ENaC) with increased sodium absorption is a feature of cystic fibrosis (CF). ION-827359 is a 2.5-generation antisense oligonucleotide targeted to reduce ENaC protein. This study evalua…
View article: Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)
Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC) Open
Background International guidelines recommend airway clearance management as one of the important pillars of bronchiectasis treatment. However, the extent to which airway clearance is used for people with bronchiectasis in Europe is unclea…
View article: Standards of care and educational gaps in adult cystic fibrosis units: a European Respiratory Society survey
Standards of care and educational gaps in adult cystic fibrosis units: a European Respiratory Society survey Open
Background Significant progress in the field of cystic fibrosis (CF) has substantially extended the life expectancy of patients with CF (pwCF). Consequently, the population of adult pwCF has outnumbered paediatric patients in most develope…
View article: Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)
Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC) Open
Background A validated 4-point sputum colour chart can be used to objectively evaluate the levels of airway inflammation in bronchiectasis patients. In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be…
View article: Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC)
Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC) Open
View article: In vitro activity of cefiderocol against Gram-negative pathogens isolated from people with cystic fibrosis and bronchiectasis
In vitro activity of cefiderocol against Gram-negative pathogens isolated from people with cystic fibrosis and bronchiectasis Open
These in vitro data demonstrated that cefiderocol had greater activity than most comparator antibiotics and could be an alternative treatment option for respiratory infection caused by these pathogens that has not responded to first-line t…
View article: Airway inflammation accelerates pulmonary exacerbations in cystic fibrosis
Airway inflammation accelerates pulmonary exacerbations in cystic fibrosis Open
Airway inflammation underlies cystic fibrosis (CF) pulmonary exacerbations. In a prospective multicenter study of randomly selected, clinically stable adolescents and adults, we assessed relationships between 24 inflammation-associated mol…
View article: Airway Total Bacterial Density, Microbiota Community Composition and Relationship with Clinical Parameters in Bronchiectasis
Airway Total Bacterial Density, Microbiota Community Composition and Relationship with Clinical Parameters in Bronchiectasis Open
View article: Analysis of SARS-CoV-2 antibody seroprevalence in Northern Ireland during 2020–2021
Analysis of SARS-CoV-2 antibody seroprevalence in Northern Ireland during 2020–2021 Open
View article: Longitudinal changes in the cystic fibrosis airway microbiota with time and treatment
Longitudinal changes in the cystic fibrosis airway microbiota with time and treatment Open
The airway microbiota remains stable over periods <1 year with modest shifts related to treatment apparent which might provide some additional insights to patient-level measurements.
View article: S26 Clinical, microbial and inflammatory characterisation of eosinophilic bronchiectasis
S26 Clinical, microbial and inflammatory characterisation of eosinophilic bronchiectasis Open
Introduction Eosinophilic bronchiectasis is a recently described clinical entity defined by eosinophilic inflammation in the absence of asthma and/or allergic bronchopulmonary aspergillosis. This study aimed to characterise eosinophilic br…
View article: S146 Aspergillus in bronchiectasis: data from the EMBARC registry
S146 Aspergillus in bronchiectasis: data from the EMBARC registry Open
Introduction Aspergillus fumigatus causes airway disorders including Allergic bronchopulmonary aspergillosis (ABPA), Aspergillus sensitisation (AS) and Aspergillus bronchitis (AB). The clinical significance of Asper…
View article: 6 Activity of cefiderocol against gram-negative bacteria isolated from people with cystic fibrosis and bronchiectasis
6 Activity of cefiderocol against gram-negative bacteria isolated from people with cystic fibrosis and bronchiectasis Open
View article: Analysis of SARS-CoV-2 Ig seroprevalence in Northern Ireland
Analysis of SARS-CoV-2 Ig seroprevalence in Northern Ireland Open
Background With the impact of SARS-CoV-2 upon public health directly and socioeconomically, further information was required to inform policy decisions designed to limit virus spread. This study sought to contribute to serosurveillance wor…
View article: Regulatory T cells mediate the decreased susceptibility of males to Pseudomonas aeruginosa infection
Regulatory T cells mediate the decreased susceptibility of males to Pseudomonas aeruginosa infection Open
Background Sex hormones have been shown to play a role in the susceptibility of female patients with CF to P. aeruginosa chronic infection; however, the cellular mechanisms responsible for such sex-based imbalance are poorly understood. Ac…
View article: Biologically Relevant Murine Models of Chronic Pseudomonas aeruginosa Respiratory Infection
Biologically Relevant Murine Models of Chronic Pseudomonas aeruginosa Respiratory Infection Open
Pseudomonas aeruginosa (P. aeruginosa) is an opportunistic pathogen and the leading cause of infection in patients with cystic fibrosis (CF). The ability of P. aeruginosa to evade host responses and develop into chronic infection causes si…
View article: Bridging the “Know-Do” Gaps in Five Non-Communicable Diseases Using a Common Framework Driven by Implementation Science
Bridging the “Know-Do” Gaps in Five Non-Communicable Diseases Using a Common Framework Driven by Implementation Science Open
According to the United Nations High-Level Meeting 2018, five non-communicable diseases (NCDs) including cardiovascular diseases, chronic respiratory diseases, diabetes mellitus, cancer, and mental health conditions accounted for two-third…
View article: Basic, translational and clinical aspects of bronchiectasis in adults
Basic, translational and clinical aspects of bronchiectasis in adults Open
Bronchiectasis is a common progressive respiratory disease with recognisable radiological abnormalities and a clinical syndrome of cough, sputum production and recurrent respiratory infections. Inflammatory cell infiltration into the lung,…