Executive summary The past six decades have seen remarkable improvements in health
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …

Cystic fibrosis (CF) is a life-limiting autosomal recessive multisystem disease. While its
burden of morbidity and mortality is classically associated with pulmonary disease, CF also …

Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to improve lung
function in people with cystic fibrosis (PWCF). However, its biological effects remain …

Evidence‐based medicine requires strong scientific evidence upon which to base treatment.
In rare diseases, study populations are often small, and thus this evidence is difficult to …

Pseudomonas aeruginosa is a gram-negative non–lactose fermenting oxidase-positive
bacterium that causes infection in patients with compromised immune systems and/or …

Pulmonary exacerbations treated with intravenous antibiotics have significant, well-
characterized negative consequences on clinical outcomes in cystic fibrosis (CF). The …

Introduction: Progressive lung disease is the major cause of morbidity and mortality in
patients with cystic fibrosis (CF). Methods of correctly predicting the future progression of …

Although oxidative stress is thought to play a pivotal role in the pathogenesis of inflammatory
airway diseases, its assessment in clinical practice remains elusive. In recent years, it has …

Background Pulmonary exacerbations are associated with significant lung function decline
from baseline in cystic fibrosis (CF) and it is not well understood why some patients do not …

Cystic fibrosis (CF) is the most common life-limiting genetic disease in Caucasian patients.
Continued advances have led to improved survival, and adults with CF now outnumber …