title: Cystic fibrosis lung environment and Pseudomonas aeruginosa infection.
abstract: The airways of patients with cystic fibrosis (CF) are highly complex, subject to various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa is recognized as one of the most important pulmonary pathogens and the predominant cause of morbidity and mortality in CF. A multifarious interplay between the host, pathogens, microbiota, and the environment shapes the course of the disease. There have been several excellent reviews detailing CF pathology, Pseudomonas and the role of environment in CF but only a few reviews connect these entities with regards to influence on the overall course of the disease. A holistic understanding of contributing factors is pertinent to inform new research and therapeutics.
  
  
  
  In this article, we discuss the deterministic alterations in lung physiology as a result of CF. We also revisit the impact of those changes on the microbiota, with special emphasis on P. aeruginosa and the influence of other non-genetic factors on CF. Substantial past and current research on various genetic and non-genetic aspects of cystic fibrosis has been reviewed to assess the effect of different factors on CF pulmonary infection. A thorough review of contributing factors in CF and the alterations in lung physiology indicate that CF lung infection is multi-factorial with no isolated cause that should be solely targeted to control disease progression. A combinatorial approach may be required to ensure better disease outcomes.
  
  
  
  CF lung infection is a complex disease and requires a broad multidisciplinary approach to improve CF disease outcomes. A holistic understanding of the underlying mechanisms and non-genetic contributing factors in CF is central to development of new and targeted therapeutic strategies.
authors.affiliation: Department of Oral Biology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.
authors.forename: Anjali Y
authors.initials: AY
authors.lastname: Bhagirath
authors.affiliation: Department of Oral Biology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.
authors.forename: Yanqi
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Oral Biology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.
authors.forename: Deepti
authors.initials: D
authors.lastname: Somayajula
authors.affiliation: Department of Oral Biology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.
authors.forename: Maryam
authors.initials: M
authors.lastname: Dadashi
authors.affiliation: Department of Medical Microbiology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada.
authors.forename: Sara
authors.initials: S
authors.lastname: Badr
authors.affiliation: Department of Oral Biology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada. Kangmin.Duan@umanitoba.ca.|Department of Medical Microbiology, Rady Faculty of Health Sciences, University of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada. Kangmin.Duan@umanitoba.ca.|Biology of Breathing Group, Children's Hospital Research Institute of Manitoba, 780 Bannatyne Ave, Winnipeg, MB, R3E 0W2, Canada. Kangmin.Duan@umanitoba.ca.
authors.forename: Kangmin
authors.initials: K
authors.lastname: Duan
mesh_terms: D000818:Animals; D000890:Anti-Infective Agents / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000382:microbiology* / Q000503:physiopathology* / Q000628:therapy; D004195:Disease Models, Animal; D054884:Host-Pathogen Interactions; D006801:Humans; D008168:Lung / Q000382:microbiology*; D051379:Mice; D064307:Microbiota; D009154:Mutation; D011552:Pseudomonas Infections / Q000150:complications*; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification; D012129:Respiratory Function Tests; D013183:Sputum / Q000382:microbiology
chemical_list: D000890:Anti-Infective Agents
keywords: CFTR; Cystic fibrosis; Host-pathogen interaction; Microbiome; Non-genetic influences