In patients with cystic fibrosis (CF) lung damage secondary to chronic infection is the main
cause of death. Treatment of lung disease to reduce the impact of infection, inflammation …

Despite significant advances in treatment strategies targeting the underlying defect in cystic
fibrosis (CF), airway infection remains an important cause of lung disease. In this two-part …

BACKGROUND: A range of physical therapies (including airway clearance and physical
training) are used in cystic fibrosis (CF). The aim of this paper is to summarise the main …

Background Increasing the activity of defective cystic fibrosis transmembrane conductance
regulator (CFTR) protein is a potential treatment for cystic fibrosis. Methods We conducted a …

Background Cystic fibrosis is a life-limiting disease that is caused by defective or deficient
cystic fibrosis transmembrane conductance regulator (CFTR) protein activity. Phe508del is …

Background Combination treatment with the cystic fibrosis transmembrane conductance
regulator (CFTR) modulators tezacaftor (VX-661) and ivacaftor (VX-770) was designed to …

Background Ataluren was developed to restore functional protein production in genetic
disorders caused by nonsense mutations, which are the cause of cystic fibrosis in 10% of …

CT scanning can also aid in identifying an aetiology of bronchiectasis eg Allergic
bronchopulmonary aspergillosis (ABPA), Non-tuberculous mycobacteria (NTM), primary …

BACKGROUND: Survival from cystic fibrosis is increasing rapidly. Estimates of the extent of
this improvement should allow health care facilities to be planned to deal with the expanding …

Rationale: Pulmonary infection in cystic fibrosis (CF) is polymicrobial and it is possible that
anaerobic bacteria, not detected by routine aerobic culture methods, reside within infected …