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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

Related topics

Related topics

47 relations
AcetylcysteineAcetylcysteine 200 MG/ML Inhalant SolutionAdenocarcinoma of the ileumAmiloride Hydrochloride

Broader (4)

Congenital DisordersLung diseasesMetabolic Diseasesairway disease

Narrower (3)

Cystic Fibrosis with Helicobacter Pylori Gastritis, Megaloblastic Anemia, and Subnormal MentalityFibrocystic Disease of PancreasLubani Al Saleh Teebi syndrome

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2011
Review
2011
Clinical Significance of Microbial Infection and Adaptation in Cystic Fibrosis
SUMMARY A select group of microorganisms inhabit the airways of individuals with cystic fibrosis. Once established within the… 
Review
2009
Review
2009
Cystic fibrosis pulmonary guidelines: airway clearance therapies.
Cystic fibrosis (CF) is a genetic disease characterized by dehydration of airway surface liquid and impaired mucociliary… 
Review
2007
Review
2007
Airway surface dehydration in cystic fibrosis: pathogenesis and therapy.
Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic bacterial infection. Studies of CF… 
Highly Cited
2007
Highly Cited
2007
Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease
Interleukin-8 (IL-8) activates neutrophils via the chemokine receptors CXCR1 and CXCR2. However, the airways of individuals with… 
Highly Cited
2005
Highly Cited
2005
Cystic Fibrosis Sputum Supports Growth and Cues Key Aspects of Pseudomonas aeruginosa Physiology
ABSTRACT The opportunistic human pathogen Pseudomonas aeruginosa causes persistent airway infections in patients with cystic… 
Highly Cited
2004
Highly Cited
2004
Association of cystic fibrosis with abnormalities in fatty acid metabolism.
BACKGROUND Patients with cystic fibrosis have altered levels of plasma fatty acids. We previously demonstrated that arachidonic… 
Highly Cited
2004
Highly Cited
2004
Clinically Feasible Biofilm Susceptibility Assay for Isolates of Pseudomonas aeruginosa from Patients with Cystic Fibrosis
ABSTRACT Pseudomonas aeruginosa is the predominant cause of chronic airway infection in cystic fibrosis (CF). CF airway isolates… 
Highly Cited
2003
Highly Cited
2003
Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis
Many children with cystic fibrosis (CF), receiving modern, aggressive CF care, have normal spirometry results. This study aimed… 
Highly Cited
1992
Highly Cited
1992
Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line.
The respiratory manifestations of cystic fibrosis (CF) are characterized by neutrophil-dominated airway inflammation. Since a… 
Highly Cited
1983
Highly Cited
1983
Pseudomonas aeruginosa isolates from patients with cystic fibrosis: a class of serum-sensitive, nontypable strains deficient in lipopolysaccharide O side chains
Twenty-six Pseudomonas aeruginosa strains from patients with cystic fibrosis were typed by the Fisher immunotyping scheme. Only 6… 
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