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PMID: 11932230 Published · ppublish English Historical Article Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Review

Lung infections associated with cystic fibrosis.

Clinical microbiology reviews ·Vol. 15 ·No. 2 ·2002-04-00 ·Pages 194-222

Lyczak JB, Cannon CL, Pier GB

Abstract

While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host.

MeSH Terms
Bacterial Infections/microbiology Cystic Fibrosis/genetics,history,microbiology,therapy Cystic Fibrosis Transmembrane Conductance Regulator/genetics Haemophilus influenzae/isolation & purification History, 20th Century Humans Lung/microbiology Mutation Pseudomonas aeruginosa/isolation & purification Staphylococcus aureus/isolation & purification
Chemicals
CFTR protein, human Cystic Fibrosis Transmembrane Conductance Regulator
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Lyczak Jeffrey B
Channing Laboratory, Brigham and Women's Hospital, Harvard Medical School, Children's Hospital, Boston, MA 02115, USA.
Cannon Carolyn L
Pier Gerald B
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Article Info
Journal
Clinical microbiology reviews
Abbr.
Clin Microbiol Rev
ISSN
0893-8512
DOI
10.1128/CMR.15.2.194-222.2002
Published
2002-04-00
Pages
194-222
Language
English
Region
United States
NLM ID
8807282
PMCID
PMC118069
Subset
IM
Grants
NHLBI NIH HHS · HL 58398 · United States
NIAID NIH HHS · AI 48917 · United States
NEI NIH HHS · EY 06805 · United States
NEI NIH HHS · F32 EY006805 · United States
NHLBI NIH HHS · R01 HL058398 · United States
NIAID NIH HHS · R01 AI048917 · United States
External Links
PubMed source DOI: 10.1128/CMR.15.2.194-222.2002 PMC Full Text View journal details
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