Since the first description of cystic fibrosis in 1938, there have been significant advances in
both quality of life and longevity for people living with this disease. In this article we describe …

Lung disease is the major cause of morbidity and mortality in patients with cystic fibrosis
(CF). Although CF lung disease is primarily an infectious disorder, the associated …

Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …

Rationale: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal
airways secretions, chronic endobronchial infection, and progressive airway obstruction …

Background Bronchiectasis develops early in the course of cystic fibrosis, being detectable
in infants as young as 10 weeks of age, and is persistent and progressive. We sought to …

Background The 24-week safety and efficacy of lumacaftor/ivacaftor combination therapy
was shown in two randomised controlled trials (RCTs)—TRAFFIC and TRANSPORT—in …

The French social philosopher Pierre Bourdieu is now recognised as one of the major
thinkers of the twentieth century. In a career of over fifty years, Bourdieu studied a wide …

Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that
regulates the transport of ions and the movement of water across the epithelial barrier …

While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is
now recognized as a single disease whose diverse symptoms stem from the wide tissue …

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