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Advances in cystic fibrosis therapies.
Curr Opin Pediatr 2006; 18(6):604-13CO

Abstract

PURPOSE OF REVIEW

Over the past four decades, outcomes for patients with cystic fibrosis have improved dramatically. Major contributors to this improvement are a better understanding of disease pathogenesis and the systematic conduct of clinical trials evaluating new therapies designed to address these defects. This review describes recent developments in cystic fibrosis pulmonary therapies intended to treat various facets of the disease, including several treatments currently in development.

RECENT FINDINGS

The mainstays of therapy for cystic fibrosis, such as nutritional support and mechanical mucus clearance, are now supplemented with aggressive antibiotic regimens intended to suppress or eradicate bacterial colonization, anti-inflammatory agents, and new approaches that improve mucociliary clearance. Therapies in development address the underlying ion transport defect found in cystic fibrosis airways and also include small-molecule agents that restore function to the mutant cystic fibrosis transmembrane conductance regulator.

SUMMARY

Recent advances in therapies for cystic fibrosis offer the promise of improved outcomes and longer lives for patients with cystic fibrosis.

Authors+Show Affiliations

Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA. smrowe@uab.eduNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

17099358

Citation

Rowe, Steven M., and John P. Clancy. "Advances in Cystic Fibrosis Therapies." Current Opinion in Pediatrics, vol. 18, no. 6, 2006, pp. 604-13.
Rowe SM, Clancy JP. Advances in cystic fibrosis therapies. Curr Opin Pediatr. 2006;18(6):604-13.
Rowe, S. M., & Clancy, J. P. (2006). Advances in cystic fibrosis therapies. Current Opinion in Pediatrics, 18(6), pp. 604-13.
Rowe SM, Clancy JP. Advances in Cystic Fibrosis Therapies. Curr Opin Pediatr. 2006;18(6):604-13. PubMed PMID: 17099358.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Advances in cystic fibrosis therapies. AU - Rowe,Steven M, AU - Clancy,John P, PY - 2006/11/14/pubmed PY - 2007/2/9/medline PY - 2006/11/14/entrez SP - 604 EP - 13 JF - Current opinion in pediatrics JO - Curr. Opin. Pediatr. VL - 18 IS - 6 N2 - PURPOSE OF REVIEW: Over the past four decades, outcomes for patients with cystic fibrosis have improved dramatically. Major contributors to this improvement are a better understanding of disease pathogenesis and the systematic conduct of clinical trials evaluating new therapies designed to address these defects. This review describes recent developments in cystic fibrosis pulmonary therapies intended to treat various facets of the disease, including several treatments currently in development. RECENT FINDINGS: The mainstays of therapy for cystic fibrosis, such as nutritional support and mechanical mucus clearance, are now supplemented with aggressive antibiotic regimens intended to suppress or eradicate bacterial colonization, anti-inflammatory agents, and new approaches that improve mucociliary clearance. Therapies in development address the underlying ion transport defect found in cystic fibrosis airways and also include small-molecule agents that restore function to the mutant cystic fibrosis transmembrane conductance regulator. SUMMARY: Recent advances in therapies for cystic fibrosis offer the promise of improved outcomes and longer lives for patients with cystic fibrosis. SN - 1040-8703 UR - https://www.unboundmedicine.com/medline/citation/17099358/Advances_in_cystic_fibrosis_therapies_ L2 - http://dx.doi.org/10.1097/MOP.0b013e3280109b90 DB - PRIME DP - Unbound Medicine ER -