… cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The elucidation of
the pathogenesis of CF lung disease … of the cystic fibrosis transmembrane regulator protein (…

… of the disease. This review focuses on the lung involvement in cystic fibrosis and summarizes
new … of CF and pathogenesis of related lung disease. Current therapeutic modalities, novel …

… to explain why we are still struggling to understand CF lung disease. The pattern of CF lung
disease is unlike that of any other lung disease. In CF, infections of the smaller airways are …

… This concept also is congruent with the failure of exogenous antimicrobial agents, eg as
part of the chronic therapy for CF lung disease, to be curative, as reviewed elsewhere in this …

… of cystic fibrosis lung disease. We summarise the current knowledge and the methods used
to characterise and quantify early lung disease. … lung disease in patients with cystic fibrosis. …

… the lung disease. Respiratory disease such as that in humans does not develop in mice with
cftr mutations. However, lung disease that mimics that in humans with cystic fibrosis occurs …

… cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung
disease of cystic fi… in studying the pathogenesis of cystic fibrosis has been limited because …

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 of CF …

… lung … lung disease and to identify effective and safe interventions through systematic controlled
trials. It is well recognized that among patients who have cystic fibrosis (CF), lung disease …

… Lung disease in cystic fibrosis is primarily due to a defect in the cystic fibrosis transmembrane
regulating protein (CFTR). This results in abnormal chloride transfer across epithelial …