title: Current concepts: host-pathogen interactions in cystic fibrosis airways disease.
abstract: Chronic infection and inflammation are defining characteristics of cystic fibrosis (CF) airway disease. Conditions within the airways of patients living with CF are conducive to colonisation by a variety of opportunistic bacterial, viral and fungal pathogens. Improved molecular identification of microorganisms has begun to emphasise the polymicrobial nature of infections in the CF airway microenvironment. Changes to CF airway physiology through loss of cystic fibrosis transmembrane conductance regulator functionality result in a wide range of immune dysfunctions, which permit pathogen colonisation and persistence. This review will summarise the current understanding of how CF pathogens infect, interact with and evade the CF host.
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.
authors.forename: Anthony C
authors.initials: AC
authors.lastname: Tang
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.
authors.forename: Stuart E
authors.initials: SE
authors.lastname: Turvey
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.
authors.forename: Marco P
authors.initials: MP
authors.lastname: Alves
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Regamey
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.
authors.forename: Burkhard
authors.initials: B
authors.lastname: Tümmler
authors.affiliation: Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dominik.Hartl@med.uni-tuebingen.de.
authors.forename: Dominik
authors.initials: D
authors.lastname: Hartl
mesh_terms: D001424:Bacterial Infections / Q000150:complications; D003550:Cystic Fibrosis / Q000150:complications / Q000276:immunology / Q000382:microbiology*; D006801:Humans; D008168:Lung / Q000382:microbiology; D008172:Lung Diseases, Fungal / Q000150:complications; D012140:Respiratory Tract Diseases / Q000382:microbiology*; D014777:Virus Diseases / Q000150:complications