If you are experiencing problems downloading PDF or HTML fulltext, our helpdesk recommend clearing your browser cache and trying again. If you need help in clearing your cache, please click here . Still need help? Email help@ingentaconnect.com

Cystic Fibrosis: Pathogenesis and Future Treatment Strategies

The full text article is not available for purchase.

The publisher only permits individual articles to be downloaded by subscribers.


Since the detection of the underlying gene defect, our knowledge of how the genetic mutations in cystic fibrosis cause lung disease has increased substantially, but we still lack a complete understanding of some of the pieces in the puzzle. Nevertheless, the information gained has led to new therapeutic approaches that address key factors of cystic fibrosis pathophysiology. Past therapeutic successes were largely based on targeting the consequences of the cystic fibrosis transmembrane regulator dysfunction, such as phlegm retention, infection, and inflammation, but new therapies may be able to address the underlying abnormality rather than its downstream effects. The efficacy of these treatments still needs to be established, but early studies look promising for several compounds. This review summarizes our current understanding of the pathophysiology and treatment of cystic fibrosis lung disease.
More about this publication?
Related content



Share Content

Access Key

Free Content
Free content
New Content
New content
Open Access Content
Open access content
Subscribed Content
Subscribed content
Free Trial Content
Free trial content
Cookie Policy
Cookie Policy
ingentaconnect website makes use of cookies so as to keep track of data that you have filled in. I am Happy with this Find out more