Claire Wainwright, J.S. Elborn, Bonnie W. Ramsey, Gautham Marigowda et al.
BACKGROUND: Combination treatment with the cystic fibrosis transmembrane conductance regulator (CFTR) modulators tezacaftor (VX-661) and ivacaftor (VX-770) was designed to target the underlying cause of disease in patients with cystic fibrosis. METHODS: through week 24 (calculated as a percentage) w...
David Comer, Joseph C. Kidney, Madeleine Ennis, J.S. Elborn
We hypothesised that primary bronchial epithelial cells (PBECs) from subjects with chronic obstructive pulmonary disease (COPD) respond differently to Pseudomonas aeruginosa lipopolysaccharide (LPS) after cigarette smoke extract (CSE) exposure than PBECs obtained from smokers without airflow obstruc...
Yuriko Nagano, J.S. Elborn, B. Cherie Millar, James M. Walker et al.
This study compares conventional and molecular techniques for the detection of fungi in 77 adult cystic fibrosis (CF) patients. Three different methods were investigated, i.e., (1) conventional microbiological culture (including yeasts and filamentous fungi), (2) mycological culture with CF-derived ...
Paul Buchanan, Robert K. Ernst, J.S. Elborn, Bettina Schock
CF (cystic fibrosis) is a severe autosomal recessive disease most common in Northwest European populations. Underlying mutations in the CFTR (CF transmembrane conductance regulator) gene cause deregulation of ion transport and subsequent dehydration of the airway surface liquid, producing a viscous ...
Judy Bradley, Steven W. Blume, Maria‐Magdalena Balp, D. Honeybourne et al.
The aim of our study was to discover the health status and healthcare utilisation associated with pulmonary exacerbations in cystic fibrosis (CF) and chronic Pseudomonas aeruginosa infection. Patients with CF from five UK CF centres attended two visits, 8-12 weeks apart. They were classified at visi...
J.S. Elborn
The concept of personalised or stratified therapies is not a new one. For centuries, physicians have observed that the manifestation of a disease and its response to intervention can vary according to many factors including age, sex, ethnicity, diet and the type of administered drug [1]. Hippocrates...
K. Lavery, Brenda O’Neill, Michael Parker, J.S. Elborn et al.
Objectives To investigate the efficacy of a disease-specific Expert Patient Programme (EPP) compared with usual care in patients with bronchiectasis. Design Proof-of-concept randomized controlled trial. Setting Regional respiratory center. Participants Adult patients (N=64; age, >18y) with a primary...
B. O'Neill, J.S. Elborn, J. MacMahon, JM Bradley
There should be a clear pathway through pulmonary rehabilitation and follow-on services. The aim of this survey was to determine the characteristics of the different components of the patient pathway, that is, pulmonary rehabilitation programs, ongoing exercise facilities, and support networks in No...
Anne Kjørsvik Bertelsen, J.S. Elborn, Bettina Schock
Prevotella spp. are frequently identified in Cystic Fibrosis sputum. This study examined whether infection with Prevotella nigrescens, a frequently identified member of this species, contributes to inflammation in CF bronchial epithelial cells through activation of TLR- and NF-κB signalling pathways...
J.S. Elborn
Stratified approaches to treating disease are very attractive, as efficacy is maximised by identifying responders using a companion diagnostic or by careful phenotyping. This approach will spare non-responders form potential side-effects. This has been pioneered in oncology where single genes or gen...
Rebecca Dobra, Marco Boeri, J.S. Elborn, Frank Kee et al.
INTRODUCTION: Engaging people with cystic fibrosis (CF) in clinical trials is critical to improving outcomes for this fatal disease. Following extensive exploration of engagement in CF trials we believe six key concepts require a quantitative understanding of their influence in the current CF trials...
J.S. Elborn
Anthony De Soyza, Timothy R. Aksamit, Tiemo-Joerg Bandel, Margarita Criollo et al.
<b>Background</b>: RESPIRE 1 assessed the efficacy and safety of Ciprofloxacin DPI in adult NCFB patients in a prospective, randomised, double-blind, multicentre, placebo-controlled trial. <b>Methods</b>: NCFB patients with ≥2 exacerbations in prior 12 months and positive predefined bacterial cultur...
James D. Chalmers, Eva Polverino, Anthony De Soyza, Felix C. Ringshausen et al.
<b>Introduction:</b> One of the aims of the European Bronchiectasis Registry is to improve service provision and care for bronchiectasis across Europe. The first step in this process is to understand how bronchiectasis care is currently delivered in European centres. <b>Methods:</b> Sites participat...
J.S. Elborn