Progress in understanding mucus abnormalities in cystic fibrosis airways

Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), los...

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Bibliographic Details
Published in:Journal of cystic fibrosis 2018-03, Vol.17 (2), p.S35-S39
Main Authors: Wine, Jeffrey J., Hansson, Gunnar C., König, Peter, Joo, Nam Soo, Ermund, Anna, Pieper, Mario
Format: Article
Language:English
Subjects:
ates of america
bicarbonate
CF piglet
Cystic fibrosis
epithelial-cells
Ferret
HCO3
Hypertonic saline
lung
Lungmedicin och allergi
Microscopic optical coherence tomography
mucin
Mucociliary clearance
Mucus
p15064
p6842
pathogenesis
Respiratory Medicine and Allergy
Respiratory System
secretion
Submucosal glands
surface dehydration
v105
v114
β-ENaC transgenic mouse
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Summary:Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), loss of CFTR function compromises these defences, and with present standard of care virtually all people with CF eventually develop mucus accumulation, plugging and chronic infections. This review focuses on how mucus is affected by CFTR loss.
ISSN:1569-1993
1873-5010
DOI:10.1016/j.jcf.2017.09.003