Search on: CYSTIC FIBROSIS 
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Descriptor English:   Cystic Fibrosis 
Descriptor Spanish:   Fibrosis Quística 
Descriptor Portuguese:   Fibrose Cística 
Synonyms English:   Mucoviscidosis  
Tree Number:   C06.689.202
C08.381.187
C16.320.190
C16.614.213
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:   Cystic Fibrosis Transmembrane Conductance Regulator
Mice, Inbred CFTR
 
History Note English:   67; was PANCREATIC CYSTIC FIBROSIS 1963-66 
Allowable Qualifiers English:  
blood cerebrospinal fluid
chemically induced classification
complications diet therapy
diagnosis drug therapy
economics ethnology
embryology enzymology
epidemiology etiology
genetics history
immunology metabolism
microbiology mortality
nursing pathology
prevention & control physiopathology
parasitology psychology
radiography rehabilitation
radionuclide imaging radiotherapy
surgery therapy
ultrastructure urine
ultrasonography veterinary
virology  
Record Number:   3568 
Unique Identifier:   D003550 

Occurrence in VHL: 		 

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