cystic fibrosis

(noun)

Cystic fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder that affects most critically the lungs, and also the pancreas, liver and intestine. It is characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions.

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(noun)

An inherited condition in which the exocrine glands produce abnormally viscous mucus, causing chronic respiratory and digestive problems.

Related Terms

Examples of cystic fibrosis in the following topics:

  • Biochemical Products of Recombinant DNA Technology

    • Hereditary diseases carrier diagnosis: tests now available to determine if a person is carrying the gene for cystic fibrosis, the Tay-Sachs diseases, Huntington's disease or Duchenne muscular dystrophy.
    • Gene transfer from one organism to other: the advanced gene therapy can benefit people with cystic fibrosis, vascular disease, rheumatoid arthritis and specific types of cancers.

  • Betaproteobacteria

    • Burkholderia is a genus of proteobacteria probably best known for its pathogenic members: Burkholderia mallei, responsible for glanders, a disease that occurs mostly in horses and related animals; Burkholderia pseudomallei, causative agent of melioidosis; and Burkholderia cepacia, an important pathogen of pulmonary infections in people with cystic fibrosis (CF).

  • Gammaproteobacteria

    • (enteritis and typhoid fever), Yersinia pestis (plague), Vibrio cholerae (cholera), Pseudomonas aeruginosa (lung infections in hospitalized or cystic fibrosis patients), and Escherichia coli (food poisoning).

  • Bacterial Pneumonias

    • Exposure to birds is associated with Chlamydia psittaci; farm animals with Coxiella burnetti; aspiration of stomach contents with anaerobic organisms; and cystic fibrosis with Pseudomonas aeruginosa and Staphylococcus aureus.

  • Defining Microbes

    • Biofilms, microbial communities that are very difficult to destroy, are considered responsible for diseases such as bacterial infections in patients with cystic fibrosis, Legionnaires' disease, and otitis media.

  • ABC Transporters

    • ABC transporters are involved in tumor resistance, cystic fibrosis and a range of other inherited human diseases along with both bacterial (prokaryotic) and eukaryotic (including human) development of resistance to multiple drugs.

  • Applications of Genetic Engineering

    • Since the 1990s, gene therapy has been used in clinical trials to treat diseases and conditions such as AIDS, cystic fibrosis, cancer, and high cholesterol.

  • Predisposing Factors

    • Cystic fibrosis is a genetic disease that causes alteration of the mucus in the lungs.

  • Ecological Associations Among Microorganisms

    • Finally, some species such as Pseudomonas aeruginosa, Burkholderia cenocepacia, and Mycobacterium avium are opportunistic pathogens and cause disease mainly in people suffering from immunosuppression or cystic fibrosis.

  • Biofilms and Infections

    • Biofilms have also been implicated in less common but more lethal processes such as endocarditis, infections in cystic fibrosis, and infections of permanent indwelling devices such as joint prostheses and heart valves.