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Cystic Fibrosis

Known as: MUCOVISCIDOSIS, Cystic fibrosis NOS, disease fibrocystic pancreas 
A congenital, autosomal, metabolic disorder affecting the exocrine glands. The secretions of exocrine glands are abnormal, resulting in excessively… 
National Institutes of Health

Papers overview

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Review
2003
Review
2003
This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and… 
Highly Cited
2000
Highly Cited
2000
The bacterium Pseudomonas aeruginosa permanently colonizes cystic fibrosis lungs despite aggressive antibiotic treatment. This… 
Highly Cited
2000
Highly Cited
2000
The lungs of cystic fibrosis (CF) patients are chronically infected for years by one or a few lineages of Pseudomonas aeruginosa… 
Highly Cited
1999
Highly Cited
1999
BACKGROUND AND METHODS We conducted two multicenter, double-blind, placebo-controlled trials of intermittent administration of… 
Review
1996
Review
1996
Respiratory infections with Pseudomonas aeruginosa and Burkholderia cepacia play a major role in the pathogenesis of cystic… 
Highly Cited
1995
Highly Cited
1995
The mechanisms underlying the initiation of lung disease and early respiratory morbidity in cystic fibrosis (CF) are poorly… 
Highly Cited
1993
Highly Cited
1993
BACKGROUND Limited data have suggested that sleep-disordered breathing, a condition of repeated episodes of apnea and hypopnea… 
Highly Cited
1992
Highly Cited
1992
CYSTIC fibrosis transmembrane conductance regulator (CFTR) is a plasma membrane Cl− channel regulated by cyclic AMP-dependent… 
Highly Cited
1992
Highly Cited
1992
Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known…