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Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Cystic Fibrosis
Respiratory Tract Diseases > Lung Diseases > Cystic Fibrosis
Digestive System Diseases > Pancreatic Diseases > Cystic Fibrosis
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Infant, Newborn, Diseases > Cystic Fibrosis

Preferred term

Cystic Fibrosis  

Type

  • Topical Descriptor

Broader concept

Related concepts

Entry terms

  • Mucoviscidosis

Note

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

Scope note

  • 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.

History note

  • 67; was PANCREATIC CYSTIC FIBROSIS 1963-66

In other languages

  • kystinen fibroosi

    Finnish

  • cystisk fibros
  • fibrosis cystica
  • fibrosis cystica non specificata
  • mucoviscidosis
  • mukoviskidoosi
  • määrittämätön kystinen fibroosi
  • ospecificerad cystisk fibros
  • Cystisk fibros

    Swedish

URI

http://www.yso.fi/onto/mesh/D003550

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