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 …
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway …
Mucus, mucins, and cystic fibrosis
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 …
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
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 …
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
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 …
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 …
fibrosis (CF) is initiated primarily by the genetic defect. To determine if inflammation …
Mucus aberrant properties in CF: Insights from cells and animal models
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus …
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 …
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 …
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 …
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
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 …
determine whether this begins early in the illness, before the onset of infection, we examined …
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