Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
… ) in cystic fibrosis (CF) airway epithelial cells are associated with airway mucus hypersecretion…
stage of the disease, to severe airway obstruction with hyperviscous and adhesive airway

Mucus structure and properties in cystic fibrosis

BK Rubin - Paediatric respiratory reviews, 2007 - Elsevier
… We now know that CF sputum contains very little mucin when compared to normal airway
mucus from subjects without lung disease or expectorated sputum from patients with chronic …

[HTML][HTML] Development of an airway mucus defect in the cystic fibrosis rat

SE Birket, JM Davis, CM Fernandez, KL Tuggle… - JCI insight, 2018 - ncbi.nlm.nih.gov
… natural progression of the airway mucus defect in cystic fibrosis (CF) … airway mucus
abnormalities. The KO rat exhibits decreased periciliary depth, hyperacidic pH, and increased mucus

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
… Since the resulting progressive lung disease can lead to respiratory failure and ultimately
death, this review focuses on studies conducted on airway mucus, more specifically on …

CFTR, mucins, and mucus obstruction in cystic fibrosis

SM Kreda, CW Davis, MC Rose - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
… 4), which may have important clinical implications for the management of airway mucus
overproduction in CF. There is a CRE cis-site (−878/−871 nt) in the MUC5AC promoter and IL1β …

[HTML][HTML] Progress in understanding mucus abnormalities in cystic fibrosis airways

JJ Wine, GC Hansson, P König, NS Joo… - … of Cystic Fibrosis, 2018 - Elsevier
… The view proposed here is that the major effect of impaired CF airway mucus clearance is to
… compounds in airway mucus interdependent with mucins? Do altered CF mucus properties …

Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis

MW Konstan, RC Stern, CF Doershuk - The Journal of pediatrics, 1994 - Elsevier
… Comprehensive treatment of cystic fibrosis includes … agents and clearance of mucus. Although
the dominant clearance … in clearing mucus from the airways of patients with cystic fibrosis, …

Airway mucus: from production to secretion

OW Williams, A Sharafkhaneh, V Kim… - American journal of …, 2006 - atsjournals.org
… Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic
fibrosis transmembrane conductance regulator expression. Am J Respir Cell Mol Biol 2005;32:453…

Therapeutic options for hydrating airway mucus in cystic fibrosis

BE Tildy, DF Rogers - Pharmacology, 2015 - karger.com
Airway mucus secretion is a normal physiological process involved with homeostasis and
is a … However, mucus hypersecretion, with abnormal mucus consistency, causes airway

Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
mucus in muco-obstructive lung diseases, eg, cystic fibrosis (… mucus layer onto the airway
surface with the formation of adherent mucus plaques and plugs, particularly in distal airways