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The Evolution of Cystic Fibrosis Care.
Chest 2015; 148(2):533-542Chest

Abstract

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.

Authors+Show Affiliations

Department of Pediatrics. Electronic address: pittman_j@kids.wustl.edu.Department of Pediatrics; Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, MO.

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

25764168

Citation

Pittman, Jessica E., and Thomas W. Ferkol. "The Evolution of Cystic Fibrosis Care." Chest, vol. 148, no. 2, 2015, pp. 533-542.
Pittman JE, Ferkol TW. The Evolution of Cystic Fibrosis Care. Chest. 2015;148(2):533-542.
Pittman, J. E., & Ferkol, T. W. (2015). The Evolution of Cystic Fibrosis Care. Chest, 148(2), pp. 533-542. doi:10.1378/chest.14-1997.
Pittman JE, Ferkol TW. The Evolution of Cystic Fibrosis Care. Chest. 2015;148(2):533-542. PubMed PMID: 25764168.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The Evolution of Cystic Fibrosis Care. AU - Pittman,Jessica E, AU - Ferkol,Thomas W, PY - 2015/3/13/entrez PY - 2015/3/13/pubmed PY - 2015/10/27/medline SP - 533 EP - 542 JF - Chest JO - Chest VL - 148 IS - 2 N2 - 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. SN - 1931-3543 UR - https://www.unboundmedicine.com/medline/citation/25764168/The_Evolution_of_Cystic_Fibrosis_Care_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(15)50350-4 DB - PRIME DP - Unbound Medicine ER -