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Recent Advances in Chest Medicine |

The Evolution of Cystic Fibrosis CareManagement of Cystic Fibrosis

Jessica E. Pittman, MD, MPH; Thomas W. Ferkol, MD
Author and Funding Information

From the Department of Pediatrics (Drs Pittman and Ferkol) and the Department of Cell Biology and Physiology (Dr Ferkol), Washington University School of Medicine, St. Louis, MO.

CORRESPONDENCE TO: Jessica E. Pittman, MD, MPH, Division of Pediatric Allergy, Immunology, and Pulmonary Medicine, Washington University School of Medicine, Campus Box 8116, 660 S Euclid, St. Louis, MO 63110; e-mail: pittman_j@kids.wustl.edu


FUNDING/SUPPORT: The authors were supported by the Cystic Fibrosis Foundation Therapeutics (Dr Pittman), the Children’s Discovery Institute (Dr Ferkol), and the National Institutes of Health (NIH) [Grants HL101465 (Dr Ferkol), HL116211 (Drs Pittman and Ferkol), HL096458 (Dr Ferkol), and K12 HL120002 (Dr Pittman)].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;148(2):533-542. doi:10.1378/chest.14-1997
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Cystic fibrosis (CF) is the most common life-limiting inherited illness of whites. Most of the morbidity and mortality in CF stems from impaired mucociliary clearance leading to chronic, progressive airways obstruction and damage. Significant progress has been made in the care of patients with CF, with advances focused on improving mucociliary clearance, minimizing inflammatory damage, and managing infections; these advances include new antimicrobial therapies, mucolytic and osmotic agents, and antiinflammatory treatments. More recently, researchers have targeted disease-causing mutations using therapies to promote gene transcription and improve channel function, which has led to impressive physiologic changes in some patients. As we develop more advanced, allele-directed therapies for the management of CF, it will become increasingly important to understand the specific genetic and environmental interactions that cause the significant heterogeneity of lung disease seen in the CF population. This understanding of CF endotypes will allow for more targeted, personalized therapies for future patients. This article reviews the genetic and molecular basis of CF lung disease, the treatments currently available, and novel therapies that are in development.

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