The basic defect in cystic fibrosis (CF) predisposes to chronic bacterial airway infections, particularly with Pseudomonas aeruginosa. Airway infections with P. aeruginosa in individuals with CF are unique in that they chronically affect a host who is immunocompetent in terms of cellular and humoral responses but is immunocompromised by impaired airway clearance. The initially acquired P. aeruginosa clone typically persists for many years in the patients' airways and thereby diversifies by de novo point mutations and the composition of its accessory genome. Co-colonizations with 2 or more clones are preferentially observed during the first 3 years of colonization. Upper and lower airways are commonly colonized by the same clone suggesting that the sinuses are the reservoir and gateway for the colonization of the lower airways. Early antipseudomonal chemotherapy has an 80% chance to eradicate the P. aeruginosa clone. This regimen introduced in the late 1980s has shifted the median age of the onset of chronic airways colonization with P. aeruginosa from school age to early adulthood at the most successful CF centres. The measures to prevent and to treat the Pseudomonas infections in CF have been considerably improved during the last 20 years. Highly transmissible epidemic strains, however, that emerge within a clonal lineage remain a major, still unresolved health threat for the CF community. (C) 2010 Elsevier GmbH. All rights reserved.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Univ Washington, Dept Biostat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Washington, DC USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Mayer-Hamblett, Nicole
Ramsey, Bonnie W.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Washington, DC USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Ramsey, Bonnie W.
Kulasekara, Hemantha D.
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Univ Washington, Dept Microbiol, Seattle, WA 98105 USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Kulasekara, Hemantha D.
Wolter, Daniel J.
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Wolter, Daniel J.
Houston, Laura S.
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Houston, Laura S.
Pope, Christopher E.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Pope, Christopher E.
Kulasekara, Bridget R.
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Armbruster, Catherine R.
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Armbruster, Catherine R.
Burns, Jane L.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Washington, DC USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Burns, Jane L.
Retsch-Bogart, George
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Retsch-Bogart, George
Rosenfeld, Margaret
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Rosenfeld, Margaret
Gibson, Ronald L.
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Gibson, Ronald L.
Miller, Samuel I.
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Univ Washington, Dept Microbiol, Seattle, WA 98105 USA
Univ Washington, Dept Genome Sci, Seattle, WA 98105 USA
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Miller, Samuel I.
Khan, Umer
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Arizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
Biodesign Inst, Ctr Fundamental & Appl Microbi, Tempe, AZ 85281 USAArizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
Davis, Trenton J.
Karanjia, Ava, V
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Arizona State Univ, Sch Engn Matter Transport & Energy, Tempe, AZ USAArizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
Karanjia, Ava, V
Bhebhe, Charity N.
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West, Sarah B.
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Richardson, Matthew
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Univ Leicester, Coll Life Sci, Dept Resp Sci, Leicester, Leics, England
NIHR Biomed Res Ctr Resp Theme, Inst Lung Hlth, Leicester, Leics, EnglandArizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
Richardson, Matthew
Bean, Heather D.
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Arizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
Biodesign Inst, Ctr Fundamental & Appl Microbi, Tempe, AZ 85281 USAArizona State Univ, Sch Life Sci, Tempe, AZ 85287 USA
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Univ Washington, Dept Biostat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Seattle, WA USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Mayer-Hamblett, Nicole
Rosenfeld, Margaret
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Seattle, WA USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Rosenfeld, Margaret
Gibson, Ronald L.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Seattle, WA USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Gibson, Ronald L.
Ramsey, Bonnie W.
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Univ Washington, Dept Pediat, Seattle, WA 98105 USA
Seattle Childrens Hosp, Seattle, WA USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Ramsey, Bonnie W.
Kulasekara, Hemantha D.
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Univ Washington, Dept Microbiol, Seattle, WA 98105 USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
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Retsch-Bogart, George Z.
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Univ N Carolina, Dept Pediat, Chapel Hill, NC USAUniv Washington, Dept Pediat, Seattle, WA 98105 USA
Retsch-Bogart, George Z.
Morgane, Wayne
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Wolter, Daniel J.
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