Targeting of cathepsin S reduces cystic fibrosis-like lung disease

Targeting Lung Inflammation in Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive disease found mostly in the Caucasian population; a major complication of CF that often leads to mortality is lung inflammation. Playing a key role in the inflammation, as demonstrated by several studies, is the continuous recruitment of neutrophils to the lung [1-3]. Despite these millions of activated neutrophils, CF patients cannot adequately de...

Origins of cystic fibrosis lung disease.

A t the basic level, we know the genetic cause of cystic fibrosis: it is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR).1,2 At the clinical level, we know that chronic bacterial airway infection, prominent neutrophilic inflammation and mucus in airways, and progressive bronchiectasis characterize advanced cy...

The genetics of cystic fibrosis lung disease

Since the isolation of the gene responsible for cystic fibrosis in 1989 we have witnessed rapid progress in our understanding of this common genetic disorder. Progressively more detailed knowledge of the disease causing mutations, the gene expression patterns, the protein structure, and its function as an ion channel has allowed the development of reliable screening programmes, the creation of ...

The genesis of cystic fibrosis lung disease.

Cystic Lung Disease in a Young man

A 32-year-old man was admitted in the Emergency Department with acute dyspnea secondary to spontaneous pneumothorax. He had history of spontaneous pneumothorax 2 years ago.He was active cigarette smoker (10 pack/year). The family history was unremarkable for lung diseases. Additionally, he had no systemic complaints.The lung HRCT revealed multiple bizarre-shaped cysts distributed in both lungs ...