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 …

Neutrophil elastase (NE) is a major inflammatory protease released by neutrophils and is
present in the airways of patients with cystic fibrosis (CF), chronic obstructive pulmonary …

Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …

Previously, we showed that serum and monocytes from patients with CF exhibit an
enhanced NLRP3-inflammasome signature with increased IL-18, IL-1β, caspase-1 activity …

Cystic Fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, resulting in defective CFTR-mediated …

Persistent neutrophil-dominated lung inflammation contributes to lung damage in cystic
fibrosis (CF). However, the mechanisms that drive persistent lung neutrophilia and tissue …

Summary Background In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7%
hypertonic saline improved the lung clearance index in children aged 3–6 years with cystic …

Neutrophils are important effector cells of the innate immune response that fight pathogens
by phagocytosis and degranulation. Neutrophil extracellular traps (NETs) are released into …

Cystic fibrosis (CF) is one of the most common life-limiting recessive genetic disorders in
Caucasians, caused by mutations in the cystic fibrosis transmembrane conductance …

The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …