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Cystic Fibrosis

An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.

Year introduced: 1967

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Tree Number(s): C06.689.202, C08.381.187, C16.320.190, C16.614.213

MeSH Unique ID: D003550

Entry Terms:

  • Fibrosis, Cystic
  • Mucoviscidosis
  • Pulmonary Cystic Fibrosis
  • Cystic Fibrosis, Pulmonary
  • Pancreatic Cystic Fibrosis
  • Cystic Fibrosis, Pancreatic
  • Fibrocystic Disease of Pancreas
  • Pancreas Fibrocystic Disease
  • Pancreas Fibrocystic Diseases
  • Cystic Fibrosis of Pancreas

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