[HTML][HTML] Lung clearance index: evidence for use in clinical trials in cystic fibrosis
L Kent, P Reix, JA Innes, S Zielen, M Le Bourgeois… - Journal of Cystic …, 2014 - Elsevier
Abstract The ECFS-CTN Standardisation Committee has undertaken this review of lung
clearance index as part of the group's work on evaluation of clinical endpoints with regard to …
clearance index as part of the group's work on evaluation of clinical endpoints with regard to …
[HTML][HTML] Peripheral muscle abnormalities in cystic fibrosis: Etiology, clinical implications and response to therapeutic interventions
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF)
with major clinical implications, such as exercise intolerance and reduced quality of life …
with major clinical implications, such as exercise intolerance and reduced quality of life …
Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial
EWFW Alton, DK Armstrong, D Ashby… - The Lancet …, 2015 - thelancet.com
Background Lung delivery of plasmid DNA encoding the CFTR gene complexed with a
cationic liposome is a potential treatment option for patients with cystic fibrosis. We aimed to …
cationic liposome is a potential treatment option for patients with cystic fibrosis. We aimed to …
Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised …
J Davies, H Sheridan, N Bell… - The lancet Respiratory …, 2013 - thelancet.com
Background Ivacaftor has shown a clinical benefit in patients with cystic fibrosis who have
the G551D-CFTR mutation and reduced lung function. Lung clearance index (LCI) using …
the G551D-CFTR mutation and reduced lung function. Lung clearance index (LCI) using …
Multiple-breath washout as a lung function test in cystic fibrosis. A cystic fibrosis foundation workshop report
P Subbarao, C Milla, P Aurora, JC Davies… - Annals of the …, 2015 - atsjournals.org
The lung clearance index (LCI) is a lung function parameter derived from the multiple-breath
washout (MBW) test. Although first developed 60 years ago, the technique was not widely …
washout (MBW) test. Although first developed 60 years ago, the technique was not widely …
Cystic fibrosis gene therapy in the UK and elsewhere
U Griesenbach, KM Pytel, EWFW Alton - Human gene therapy, 2015 - liebertpub.com
The cystic fibrosis transmembrane conductance regulator (CFTR) gene was identified in
1989. This opened the door for the development of cystic fibrosis (CF) gene therapy, which …
1989. This opened the door for the development of cystic fibrosis (CF) gene therapy, which …
[PDF][PDF] The impact of highly effective modulator therapy on cystic fibrosis microbiology and inflammation
LJ Caverly, SA Riquelme, KB Hisert - Clinics in chest medicine, 2022 - Elsevier
Highly-effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” …
P Ciet, S Bertolo, M Ros, R Casciaro… - European …, 2022 - Eur Respiratory Soc
Objective Imaging represents an important noninvasive means to assess cystic fibrosis (CF)
lung disease, which remains the main cause of morbidity and mortality in CF patients. While …
lung disease, which remains the main cause of morbidity and mortality in CF patients. While …
Using chest computed tomography and unsupervised machine learning for predicting and evaluating response to lumacaftor–ivacaftor in people with cystic fibrosis
A Campredon, E Battistella, C Martin… - European …, 2022 - Eur Respiratory Soc
Objectives Lumacaftor–ivacaftor is a cystic fibrosis transmembrane conductance regulator
(CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). The …
(CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). The …
[HTML][HTML] Physiologic endpoints for clinical studies for cystic fibrosis
S Stanojevic, F Ratjen - Journal of Cystic fibrosis, 2016 - Elsevier
The cystic fibrosis (CF) drug development pipeline promises many exciting new treatments
for patients with CF, all which will require clinical studies to prove their benefits on CF lung …
for patients with CF, all which will require clinical studies to prove their benefits on CF lung …