DeCS Server - List Exact Term

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DeCS

Descriptor English:

Cystic Fibrosis

Descriptor Spanish:

Fibrosis Quística

Descriptor Portuguese:

Fibrose Cística

Synonyms English:

Mucoviscidosis

Tree Number:

Definition English:

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.

Indexing Annotation English:

a specific disease entity: do not use for fibrocystic states of various organs

See Related English:

History Note English:

67; was PANCREATIC CYSTIC FIBROSIS 1963-66

Allowable Qualifiers English:

Record Number:

3568

Unique Identifier:

D003550

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