title: Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches.
abstract: Pseudomonas aeruginosa is a remarkably versatile environmental bacterium with an extraordinary capacity to infect the cystic fibrosis (CF) lung. Infection with P. aeruginosa occurs early, and although eradication can be achieved following early detection, chronic infection occurs in over 60% of adults with CF. Chronic infection is associated with accelerated disease progression and increased mortality. Extensive research has revealed complex mechanisms by which P. aeruginosa adapts to and persists within the CF airway. Yet knowledge gaps remain, and prevention and treatment strategies are limited by the lack of sensitive detection methods and by a narrow armoury of antibiotics. Further developments in this field are urgently needed in order to improve morbidity and mortality in people with CF. Here, we summarize current knowledge of pathophysiological mechanisms underlying P. aeruginosa infection in CF. Established treatments are discussed, and an overview is offered of novel detection methods and therapeutic strategies in development.
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.
authors.forename: Helena
authors.initials: H
authors.lastname: Lund-Palau
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.|b Department of Respiratory Paediatrics , Royal Brompton and Harefield NHS Foundation Trust , London , UK.
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Turnbull
authors.affiliation: b Department of Respiratory Paediatrics , Royal Brompton and Harefield NHS Foundation Trust , London , UK.|c National Heart and Lung Institute, Imperial College , London , UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.affiliation: d Biomolecular Medicine, Department of Surgery and Cancer, Faculty of Medicine , Imperial College , London , UK.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Bardin
authors.affiliation: e Department of Medicine , Imperial College , London , UK.
authors.forename: Loren
authors.initials: L
authors.lastname: Cameron
authors.affiliation: f Biological Sciences, Institute for Life Sciences , University of Southampton , Southampton , UK.
authors.forename: Odel
authors.initials: O
authors.lastname: Soren
authors.affiliation: c National Heart and Lung Institute, Imperial College , London , UK.
authors.forename: Natasha
authors.initials: N
authors.lastname: Wierre-Gore
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.
authors.forename: Eric W F W
authors.initials: EW
authors.lastname: Alton
authors.affiliation: c National Heart and Lung Institute, Imperial College , London , UK.
authors.forename: Jacob G
authors.initials: JG
authors.lastname: Bundy
authors.affiliation: g NIHR Wellcome Trust Clinical Research Facility, University Hospital Southampton NHS Foundation Trust , University of Southampton , Southampton , UK.
authors.forename: Gary
authors.initials: G
authors.lastname: Connett
authors.affiliation: g NIHR Wellcome Trust Clinical Research Facility, University Hospital Southampton NHS Foundation Trust , University of Southampton , Southampton , UK.
authors.forename: Saul N
authors.initials: SN
authors.lastname: Faust
authors.affiliation: h Department of Life Sciences , Imperial College , London , UK.
authors.forename: Alain
authors.initials: A
authors.lastname: Filloux
authors.affiliation: e Department of Medicine , Imperial College , London , UK.
authors.forename: Paul
authors.initials: P
authors.lastname: Freemont
authors.affiliation: i Department of Respiratory Medicine , Royal Brompton Hospital , London , UK.
authors.forename: Andy
authors.initials: A
authors.lastname: Jones
authors.affiliation: c National Heart and Lung Institute, Imperial College , London , UK.
authors.forename: Valerie
authors.initials: V
authors.lastname: Khoo
authors.affiliation: j AmpliPhi Australia , Sydney , Australia.
authors.forename: Sandra
authors.initials: S
authors.lastname: Morales
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.
authors.forename: Ronan
authors.initials: R
authors.lastname: Murphy
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.
authors.forename: Rishi
authors.initials: R
authors.lastname: Pabary
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.
authors.forename: Ameze
authors.initials: A
authors.lastname: Simbo
authors.affiliation: k Department of Microbiology , Royal Brompton Hospital , London UK.
authors.forename: Silke
authors.initials: S
authors.lastname: Schelenz
authors.affiliation: d Biomolecular Medicine, Department of Surgery and Cancer, Faculty of Medicine , Imperial College , London , UK.
authors.forename: Zoltan
authors.initials: Z
authors.lastname: Takats
authors.affiliation: k Department of Microbiology , Royal Brompton Hospital , London UK.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Webb
authors.affiliation: g NIHR Wellcome Trust Clinical Research Facility, University Hospital Southampton NHS Foundation Trust , University of Southampton , Southampton , UK.
authors.forename: Huw D
authors.initials: HD
authors.lastname: Williams
authors.affiliation: a Department of Gene Therapy, National Heart and Lung Institute , Imperial College , London , UK.|b Department of Respiratory Paediatrics , Royal Brompton and Harefield NHS Foundation Trust , London , UK.
authors.forename: Jane C
authors.initials: JC
authors.lastname: Davies
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000382:microbiology* / Q000628:therapy*; D006801:Humans; D011552:Pseudomonas Infections / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology*; D011550:Pseudomonas aeruginosa
chemical_list: D000900:Anti-Bacterial Agents
keywords: Pseudomonas aeruginosa; airway; antibiotic; biofilm; cystic fibrosis; persistence; sputum