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BMC Pulmonary Medicine

, 16:174

Infectious, Rare and Idiopathic Pulmonary Diseases


BackgroundThe 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.

DiscussionIn 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.

ConclusionCF 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.

KeywordsCystic fibrosis Host-pathogen interaction Microbiome CFTR Non-genetic influences AbbreviationsASLAirway surface liquid

ATPAdenosine tri-phosphate

cAMPCyclic adenosine monophosphate

CCSPClub cell secretory protein

CFCystic fibrosis

CFTRCystic fibrosis transmembrane conductance regulator

hMSCHuman mesenchymal stem cell

ICLIntracellular loop


iNKTInvariant natural killer cells


MDSCMyeloid-derived suppressor cell

MHCMajor histocompatibility complex

NAIPNeuronal apoptosis inhibitory protein

NBDNuclear-binding domain

NETNeutrophil extracellular trap

NLRNod-like receptor

NLRC4NLR family CARD domain containing protein 4

PAMPPathogen-associated molecular pattern

PMNPolymorphonuclear leukocytes

RNDResistance-nodulation-cell division

ROSReactive oxygen species

T6SSType six secretion system

TLRToll-like receptor

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Autor: Anjali Y. Bhagirath - Yanqi Li - Deepti Somayajula - Maryam Dadashi - Sara Badr - Kangmin Duan


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