scholarly journals Influence of the Surfactant Tyloxapol on Mucociliary Clearance in Human Respiratory Cystic Fibrosis Cells

Pharmacology ◽  
2016 ◽  
Vol 98 (1-2) ◽  
pp. 1-3
Author(s):  
Eckhard Beubler ◽  
Rainald Fischer ◽  
Gerald Untersteiner ◽  
Wolfgang Strohmaier
Keyword(s):  
Cystic Fibrosis ◽  
Mucociliary Clearance

Cystic Fibrosis in Rhinologic Practice

2002 ◽  
Vol 16 (3) ◽  
pp. 155-160 ◽  
Author(s):  
Antoni Krzeski ◽  
Dorota Kapiszewska-Dzedzej ◽  
Norbert P. Górski ◽  
Iwona Jakubczyk
Keyword(s):  
Cystic Fibrosis ◽  
Nasal Polyposis ◽  
Mucociliary Clearance ◽  
Signs And Symptoms ◽  
Control Group ◽  
Lateral Nasal Wall ◽  
Bony Destruction ◽  
Radiological Changes ◽  
Inflammatory Changes ◽  
Maxillary Sinus Hypoplasia

Background Cystic fibrosis (CF) is the most common genetic lethal disorder that affects white populations. Chronic rhinosinusitis (CRS) with extensive nasal polyposis is one of the manifestations of CF. Methods The aim of this study was to determine the prevalence and extent of CRS in CF patients. Results The study indicated that the signs and symptoms of CRS were present in all patients with CF and they were more advanced than in the control group. The most severe inflammatory changes in the paranasal sinuses were detected in patients with the dF508 gene mutation type who suffered from CRS for >3 years. Conclusions Massive nasal polyposis, dilated base of the nose, mucociliary clearance impairment, and significant radiological changes (frontal and maxillary sinus hypoplasia, bony destruction, and medial bulging of the lateral nasal wall) were identified to be the characteristic signs of the CRS in CF patients.

scholarly journals Hypertonic Saline in Treatment of Pulmonary Disease in Cystic Fibrosis

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Emer P. Reeves ◽  
Kevin Molloy ◽  
Kerstin Pohl ◽  
Noel G. McElvaney
Keyword(s):  
Cystic Fibrosis ◽  
Hypertonic Saline ◽  
Mucociliary Clearance ◽  
Inflammatory Cells ◽  
Liquid Volume ◽  
Airway Surface Liquid ◽  
Beneficial Effects ◽  
Therapeutic Benefits ◽  
Surface Liquid ◽  
Subsequent Failure

The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.

scholarly journals Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa

1997 ◽  
Vol 10 (10) ◽  
pp. 2312-2318 ◽  
Author(s):  
E.A. Cowley ◽  
C-G. Wang ◽  
D. Gosselin ◽  
D. Radzioch ◽  
D.H. Eidelman
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Knockout Mice ◽  
Mucociliary Clearance

Mucus clearance: in vivo canine tracheal vs. in vitro bullfrog palate studies

1977 ◽  
Vol 42 (5) ◽  
pp. 761-766 ◽  
Author(s):  
A. Giordano ◽  
C. K. Shih ◽  
D. S. Holsclaw ◽  
M. A. Khan ◽  
M. Litt
Keyword(s):  
Cystic Fibrosis ◽  
Mucociliary Clearance ◽  
Objective Method ◽  
Flow Properties ◽  
Therapeutic Modalities ◽  
Mucus Clearance ◽  
Clearance Studies ◽  
Frog Palate

Tracheal mucociliary clearance was studied by a radioisotope technique in pentothal-anesthetized beagles in the control, atropinized, or dehydrated state. Mucus collected from a tracheal pouch in each dog was used for in vitro bullfrog (Rana cantesbiana) palate clearance studies and compared to the in vivo clearance rates. In all three experimental states, there was a significant correlation between in vivo and in vitro rates, suggesting that tracheal pouch mucus is a good model for investigating the mucociliary flow properties of intact airway mucus. When compared to matched controls, atropine appeared to cause a slowing of the in vivo clearance rate but not of the in vitro rate. Dehydration had no effect on either. The appropriateness of the frog palate method in the study of human respiratory disease (e.g., chronic bronchitis, cystic fibrosis) as well as its potential as an objective method of assessing the effects of various therapeutic modalities in these diseases is discussed.

Effect of uridine 5'-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis.

1996 ◽  
Vol 153 (6) ◽  
pp. 1796-1801 ◽  
Author(s):  
W D Bennett ◽  
K N Olivier ◽  
K L Zeman ◽  
K W Hohneker ◽  
R C Boucher ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Mucociliary Clearance

scholarly journals Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

2016 ◽  
Vol 2016 ◽  
pp. 1-14 ◽  
Author(s):  
Gillian M. Lavelle ◽  
Michelle M. White ◽  
Niall Browne ◽  
Noel G. McElvaney ◽  
Emer P. Reeves
Keyword(s):  
Cystic Fibrosis ◽  
Animal Models ◽  
Mouse Models ◽  
Animal Studies ◽  
Mucociliary Clearance ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Bacterial Colonisation ◽  
Transport Defect ◽  
Cystic Fibrosis Transmembrane

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased mucociliary clearance, bacterial colonisation, and chronic neutrophil-dominated inflammation. Much knowledge surrounding the pathophysiology of the disease has been gained through the generation of animal models, despite inherent limitations in each. The failure of certain mouse models to recapitulate the phenotypic manifestations of human disease has initiated the generation of larger animals in which to study CF, including the pig and the ferret. This review will summarise the basic phenotypes of three animal models and describe the contributions of such animal studies to our current understanding of CF.

scholarly journals Homogeneity of Aerosol Deposition and Mucociliary Clearance are Improved Following Ivacaftor Treatment in Cystic Fibrosis

2018 ◽  
Vol 31 (4) ◽  
pp. 204-211 ◽  
Author(s):  
William D. Bennett ◽  
Kirby L. Zeman ◽  
Beth L. Laube ◽  
Jihong Wu ◽  
Gail Sharpless ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Mucociliary Clearance ◽  
Aerosol Deposition

scholarly journals Numerical and experimental investigation of mucociliary clearance breakdown in cystic fibrosis

2017 ◽  
Vol 53 ◽  
pp. 56-63 ◽  
Author(s):  
Robin Chatelin ◽  
Dominique Anne-Archard ◽  
Marlène Murris-Espin ◽  
Marc Thiriet ◽  
Philippe Poncet
Keyword(s):  
Cystic Fibrosis ◽  
Experimental Investigation ◽  
Mucociliary Clearance

scholarly journals Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Nam Soo Joo ◽  
Hyung-Ju Cho ◽  
Meagan Shinbashi ◽  
Jae Young Choi ◽  
Carlos E. Milla ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Mucociliary Clearance ◽  
Defense Mechanism ◽  
Fluid Secretion ◽  
Adrenergic Agonist ◽  
Anion Secretion ◽  
Innate Defense ◽  
Mucus Clearance ◽  
Cystic Fibrosis Airway ◽  
Submucosal Glands

AbstractMucus clearance, a primary innate defense mechanism of airways, is defective in patients with cystic fibrosis (CF) and CF animals. In previous work, the combination of a low dose of the cholinergic agonist, carbachol with forskolin or a β adrenergic agonist, isoproterenol synergistically increased mucociliary clearance velocity (MCCV) in ferret tracheas. Importantly, the present study shows that synergistic MCCV can also be produced in CF ferrets, with increases ~ 55% of WT. Synergistic MCCV was also produced in pigs. The combined agonists increased MCCV by increasing surface fluid via multiple mechanisms: increased fluid secretion from submucosal glands, increased anion secretion across surface epithelia and decreased Na+ absorption. To avoid bronchoconstriction, the cAMP agonist was applied 30 min before carbachol. This approach to increasing mucus clearance warrants testing for safety and efficacy in humans as a potential therapeutic for muco-obstructive diseases.

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