fibrosis

Examples of fibrosis in the following topics:

• Cystic Fibrosis

  • Cystic fibrosis (CF) is an autosomal recessive disorder leading to respiratory congestion, multiple organ failure, and metabolic changes.
  • The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas.
  • CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR).
  • Gene therapy has been explored as a potential cure for cystic fibrosis.
  • A graph depicting common bacteria found in the respiratory tracts of cystic fibrosis patients as a function of age.

• Factors Affecting Pulmonary Ventilation: Compliance of the Lungs

  • Low lung compliance is commonly seen in people with restrictive lung diseases, such as pulmonary fibrosis, in which scar tissue deposits in the lung making it much more difficult for the lungs to expand and deflate, and gas exchange is impaired.
  • Pulmonary fibrosis is caused by many different types of inhalation exposures, such as silica dust.
  • Pulmonary fibrosis stiffens the lungs through deposits of scar tissue, decreasing low compliance and making it more difficult for the lungs to inflate and deflate.

• Aging and the Cardiovascular System

  • The heart valves may also become thickened by fibrosis, leading to heart murmurs and less efficient pumping.
  • High magnification micrograph of senile cardiac amyloidosis (brown) and fibrosis (yellow).

• The Complement System and Heart Disease

  • After healing, there may be fibrosis and adhesion of the pericardium with the heart, leading to constriction of the heart and reduced cardiac function.
  • On healing, there will be fibrosis and reduced functional capacity .

• Lymphogranuloma Venereum

  • As healing starts, fibrosis may occur in the inflamed areas and cause obstruction of the lymphatic system and edema.
  • The fibrosis and edema are considered the third stage of LGV and are mainly permanent.

• 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.

• Asbestos-Related Diseases

  • Asbestosis specifically refers to interstitial (parenchymal) fibrosis from asbestos, and not pleural fibrosis or plaquing as can be seen in Figure 2 in the staining of lung tissue from a patient with asbestosis.
  • The result is interstitial fibrosis.
  • Micrograph of asbestosis showing the characteristic ferruginous bodies and marked interstitial fibrosis.

• Protein Folding, Modification, and Targeting

  • For example, cystic fibrosis is caused by defects in a membrane-bound protein called cystic fibrosis transmembrane conductance regulator (CFTR).
  • The most common cystic fibrosis-causing mutation is the deletion of a Phe residue at position 508 in CFTR, which causes improper folding of the protein.
  • Therefore, improved understanding of protein folding may lead to new therapies for cystic fibrosis, Creutzfeldt-Jakob, and many other diseases.

• Lung Capacity and Volume

  • An FEV1/FVC ratio of >80% indicates a restrictive lung disease like pulmonary fibrosis or infant respiratory distress syndrome.
  • VC tends to be decreased in those with restrictive lung diseases, such as pulmonary fibrosis, making VC a good diagnostic indicator of restrictive lung diseases.

• Other Fungi Involved in Respiratory Disease

  • Symptoms include productive coughing, nodules and cavitations of the lungs, fibrosis, and swollen hilar lymph nodes.