Recent advances in cystic fibrosis

Clin Chest Med. 2012 Jun;33(2):307-28. doi: 10.1016/j.ccm.2012.02.006. Epub 2012 Mar 29.

Abstract

Cystic fibrosis (CF) is an inherited chronic disease that remains a common cause of morbidity and mortality in affected patients, mostly in the young. A wealth of knowledge has been gained into the genetics, pathophysiology, and clinical manifestation of the disease. In parallel with these new insights into the disease, novel treatments have been developed or are under development that have had a major impact on quality of life and survival. Improvement in the delivery of care to patients in CF centers, using a team-based approach, and constant review of process, and by quality improvement projects, have also had an impact on outcomes in CF.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis* / genetics
  • Cystic Fibrosis* / microbiology
  • Cystic Fibrosis* / physiopathology
  • Cystic Fibrosis* / therapy
  • Delivery of Health Care / standards
  • Delivery of Health Care / trends*
  • Humans
  • Outcome Assessment, Health Care
  • Quality Improvement