CFTR modulators: shedding light on precision medicine for cystic fibrosis

M Lopes-Pacheco - Frontiers in pharmacology, 2016 - frontiersin.org
Cystic fibrosis (CF) is the most common life-threatening monogenic disease afflicting
Caucasian people. It affects the respiratory, gastrointestinal, glandular and reproductive …

Measurements of deposition, lung surface area and lung fluid for simulation of inhaled compounds

E Fröhlich, A Mercuri, S Wu… - Frontiers in …, 2016 - frontiersin.org
Modern strategies in drug development employ in silico techniques in the design of
compounds as well as estimations of pharmacokinetics, pharmacodynamics and toxicity …

Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches

H Lund-Palau, AR Turnbull, A Bush… - Expert review of …, 2016 - Taylor & Francis
Pseudomonas aeruginosa is a remarkably versatile environmental bacterium with an
extraordinary capacity to infect the cystic fibrosis (CF) lung. Infection with P. aeruginosa …

Mucus hyperconcentration as a unifying aspect of the chronic bronchitic phenotype

B Button, WH Anderson, RC Boucher - Annals of the American …, 2016 - atsjournals.org
Abnormalities in mucus production and qualitative properties such as mucus hydration are
central to the pathophysiology of airway disease including cystic fibrosis, asthma, and …

Palivizumab for prophylaxis against respiratory syncytial virus infection in children with cystic fibrosis

KA Robinson, OA Odelola… - Cochrane Database of …, 2016 - cochranelibrary.com
Background Respiratory syncytial virus infection causes acute lung infection in infants and
young children worldwide, resulting in considerable morbidity and mortality. Children with …

Pharmacological analysis of epithelial chloride secretion mechanisms in adult murine airways

A Gianotti, L Ferrera, AR Philp, E Caci… - European Journal of …, 2016 - Elsevier
Defective epithelial chloride secretion occurs in humans with cystic fibrosis (CF), a genetic
defect due to loss of function of CFTR, a cAMP-activated chloride channel. In the airways …

Numerical flow simulation: A new method for assessing nasal breathing

T Hildebrandt, J Osman, L Goubergrits - HNO, 2016 - Springer
The current options for objective assessment of nasal breathing are limited. The maximum
they can determine is the total nasal resistance. Possibilities to analyze the endonasal …

Exploring the mechanisms behind cigarette smoke-induced internalization of CFTR

AJ Marklew - 2016 - theses.ncl.ac.uk
Chronic obstructive pulmonary disease (COPD) the third leading cause of death, with an
estimated 65 million cases worldwide. Despite this, most research to date has focused on …

Rational drug development for mucous dehydration and mucous metaplasia

TG O'Riordan - Journal of Aerosol Medicine and Pulmonary Drug …, 2016 - liebertpub.com
Airway diseases that are clinically characterized by mucous hypersecretion are associated
with dehydrated secretions and impaired mucociliary clearance. The failure to clear pro …

[图书][B] Establishing a Methodology to Measure the pH of the Airway Surface Liquid in non-CF and CF Human Airway Epithelial Cells

R Goldstein - 2016 - search.proquest.com
Human lungs maintain proper airway surface liquid homeostasis for mucociliary clearance,
hydration, and as the first line of defense against inhaled pathogens. In Cystic Fibrosis, the …