… that in humans early CF lung disease is characterized by an increased mucus inflammation
and occurs in the absence of infection or structural lung disease. Based on this data the …

… abnormally acidic airway surface liquid may contribute to lung disease in people with CF (3,
39–41) and may explain the elevated risk of lung disease that we found for carriers. These …

… in cystic fibrosis (CF) is associated with development of age-related extrapulmonary
complications such as CF-related bone disease (… the severity of lung disease and malnutrition [2]. …

… Because these excluded comparator patients tended to have more severe lung disease,
comparator patients remaining in the cohort through 2016 had less severe lung function …

… Elexacaftor-Tezacaftor-Ivacaftor in patients with advanced stage lung disease resulted in
a mean improvement in ppFEV 1 of 7.9% (p<0.0001) compared to pre-treatment baseline …

… cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug approved for
the treatment of patients with cystic fibrosis (… to subjects with advanced lung disease (ppFEV 1 …

… Accelerated lung function decline in individuals with cystic fibrosis (CF) … Factors contributing
to lung function decline are not well … Lung disease at diagnosis in infants with cystic fibrosis …

… lung damage. In 1989, the discovery that CF is caused by mutations in the CFTR (cystic
fibrosis … signaling in cystic fibrosis lung disease. Am J Respir Crit Care Med 2015;191:902–913. …

… Airway dysbiosis has been associated with lung disease severity in patients with cystic fibrosis
(CF). However, the relationship between dysbiosis, airway inflammation and lung function …

… Albeit lung disease remains the main cause of morbidity and mortality, the changing
demographics of CF necessitate an increased focus also on other organ manifestations such as …