Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

[HTML][HTML] Physicochemical properties of mucus and their impact on transmucosal drug delivery

J Leal, HDC Smyth, D Ghosh - International journal of pharmaceutics, 2017 - Elsevier
Mucus is a selective barrier to particles and molecules, preventing penetration to the
epithelial surface of mucosal tissues. Significant advances in transmucosal drug delivery …

Secretory cells dominate airway CFTR expression and function in human airway superficial epithelia

K Okuda, H Dang, Y Kobayashi, G Carraro… - American journal of …, 2021 - atsjournals.org
Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for CF …

Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel …

N Mayer-Hamblett, F Ratjen, R Russell… - The Lancet …, 2023 - thelancet.com
Background Reducing treatment burden is a priority for people with cystic fibrosis, whose
health has benefited from using new modulators that substantially increase CFTR protein …

Muco-obstructive lung diseases

RC Boucher - New England Journal of Medicine, 2019 - Mass Medical Soc
Muco-Obstructive Lung Diseases This review covers the normal mechanisms of mucus
formation and how they are abnormal in common conditions—such as COPD, cystic fibrosis …

Cystic fibrosis lung environment and Pseudomonas aeruginosa infection

AY Bhagirath, Y Li, D Somayajula, M Dadashi… - BMC pulmonary …, 2016 - Springer
Background The airways of patients with cystic fibrosis (CF) are highly complex, subject to
various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa …

Cystic fibrosis lung disease: an overview

NL Turcios - Respiratory care, 2020 - rc.rcjournal.com
Although better insights into the natural course of cystic fibrosis (CF) have led to treatment
approaches that have improved pulmonary health and increased the life expectancy of …

Airway mucus function and dysfunction

JV Fahy, BF Dickey - New England journal of medicine, 2010 - Mass Medical Soc
Pulmonary defense against environmental injury depends on airway mucus, which traps
inhaled toxins that are then cleared from the lungs by ciliary beating and cough. This review …

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis

SM Rowe, SL Heltshe, T Gonska… - American journal of …, 2014 - atsjournals.org
Rationale: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR)
potentiator recently approved for patients with CF age 6 and older with the G551D mutation …

Stenotrophomonas maltophilia: an emerging global opportunistic pathogen

JS Brooke - Clinical microbiology reviews, 2012 - Am Soc Microbiol
Stenotrophomonas maltophilia is an emerging multidrug-resistant global opportunistic
pathogen. The increasing incidence of nosocomial and community-acquired S. maltophilia …