This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

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 …

Background Around 20% of people with cystic fibrosis (pwCF) do not have access to the
triple combination elexacaftor/tezacaftor/ivacaftor (ETI) in Europe because they do not carry …

Background The macrolide antibiotic azithromycin has anti-inflammatory properties
potentially beneficial in cystic fibrosis. Since findings of open pilot studies seemed to show …

Background Forced expiratory volume in 1 s as a percentage of predicted (% FEV1) is a key
outcome in cystic fibrosis (CF) and other lung diseases. As people with CF survive for longer …

Study objective To evaluate the validity of spirometryself-testing during home telemonitoring
and to assess the acceptance ofan Internet-based home asthma telemonitoring system by …

The aim of this study was to evaluate how lung function and growth changed over time in
children with cystic fibrosis (CF) colonized with Pseudornonas aeruginosa (Pa) compared …

Background Daily recombinant human deoxyribonuclease (rhDNase) is an established but
expensive treatment in cystic fibrosis. Alternate-day treatment, if equally effective, would …

The diagnosis of “CF asthma” is problematic and it is difficult to determine which patients
have a combination of CF and asthma and which have asthma like symptoms caused by …

Background Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis
(CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI …