Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
Defective expression and function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis

N Regamey, L Tsartsali, TN Hilliard, O Fuchs, HL Tan… - Thorax, 2012 - thorax.bmj.com
Background Studies in cystic fibrosis (CF) generally focus on inflammation present in the
airway lumen. Little is known about inflammation occurring in the airway wall, the site …

Early pulmonary inflammation and lung damage in children with cystic fibrosis

A Schultz, S Stick - Respirology, 2015 - Wiley Online Library
Individuals with cystic fibrosis (CF) suffer progressive airway inflammation, infection and
lung damage. Airway inflammation and infection are present from early in life, often before …

Lower airway inflammation in infants with cystic fibrosis detected by newborn screening

DS Armstrong, SM Hook, KM Jamsen… - Pediatric …, 2005 - Wiley Online Library
Controversy exists over whether the lower airway inflammation that characterizes cystic
fibrosis (CF) is initiated primarily by the genetic defect. To determine if inflammation …

Mucus aberrant properties in CF: Insights from cells and animal models

C Ehre, GC Hansson, DJ Thornton… - Journal of Cystic …, 2023 - Elsevier
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus …

Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression

SH Leir, S Parry, T Palmai-Pallag, J Evans… - American journal of …, 2005 - atsjournals.org
Abnormalities in mucus properties and clearance make a major contribution to the pathology
of cystic fibrosis (CF). Our aim was to test the hypothesis that the defects in CF mucus are a …

New insights into pulmonary inflammation in cystic fibrosis

S Rao, J Grigg - Archives of disease in childhood, 2006 - adc.bmj.com
Persistent lower airway infection with inflammation is the major cause of morbidity and
mortality in cystic fibrosis. This review examines the recent advances in the understanding of …

[HTML][HTML] The microbial community of the cystic fibrosis airway is disrupted in early life

J Renwick, P McNally, B John, T DeSantis, B Linnane… - PloS one, 2014 - journals.plos.org
Background Molecular techniques have uncovered vast numbers of organisms in the cystic
fibrosis (CF) airways, the clinical significance of which is yet to be determined. The aim of …

Lower airway inflammation in infants and young children with cystic fibrosis

DS Armstrong, K Grimwood, JB Carlin… - American journal of …, 1997 - atsjournals.org
Airway inflammation is an important component of cystic fibrosis (CF) lung disease. To
determine whether this begins early in the illness, before the onset of infection, we examined …