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Descriptor English: Cystic Fibrosis
Descriptor Spanish: Fibrosis Quística
Descriptor fibrosis quística
Entry term(s) mucoviscidosis
Scope note: Enfermedad genética autosómica recesiva de las GLÁNDULAS EXOCRINAS. Está causada por mutaciones del gen que codifica el REGULADOR DE LA CONDUCTANCIA TRANSMEMBRANA DE LA FIBROSIS QUÍSTICA, que se expresa en diversos órganos, como el PULMÓN, el PÁNCREAS las VÍAS BILIARES y las GLÁNDULAS SUDORÍPARAS. La enfermedad se caracteriza por disfunción de la secreción epitelial asociada a obstrucción ductal que da lugar a OBSTRUCCIÓN DE LAS VÍAS RESPIRATORIAS, INFECCIONES RESPIRATORIAS crónicas, INSUFICIENCIA PANCREÁTICA, insuficiencia digestiva, pérdida de sales y AGOTAMIENTO POR CALOR.
Descriptor Portuguese: Fibrose Cística
Descriptor French: Mucoviscidose
Entry term(s): Cystic Fibrosis of Pancreas
Cystic Fibrosis, Pancreatic
Cystic Fibrosis, Pulmonary
Fibrocystic Disease of Pancreas
Fibrosis, Cystic
Mucoviscidosis
Pancreas Fibrocystic Disease
Pancreas Fibrocystic Diseases
Pancreatic Cystic Fibrosis
Pulmonary Cystic Fibrosis
Tree number(s): C06.689.202
C08.381.187
C16.320.190
C16.614.213
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D003550
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.
Annotation: a specific disease entity: do not use for fibrocystic states of various organs
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 67; was PANCREATIC CYSTIC FIBROSIS 1963-66
Online Note: use CYSTIC FIBROSIS to search PANCREATIC CYSTIC FIBROSIS 1966
History Note: 67; was PANCREATIC CYSTIC FIBROSIS 1963-66
Related: Cystic Fibrosis Transmembrane Conductance Regulator MeSH
Mice, Inbred CFTR MeSH
DeCS ID: 3568
Unique ID: D003550
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1967/01/01
Date of Entry: 1999/01/01
Revision Date: 2013/07/08
Cystic Fibrosis - Preferred
Concept UI M0005551
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.
Preferred term Cystic Fibrosis
Entry term(s) Fibrosis, Cystic
Mucoviscidosis
Pulmonary Cystic Fibrosis - Narrower
Concept UI M0455430
Preferred term Pulmonary Cystic Fibrosis
Entry term(s) Cystic Fibrosis, Pulmonary
Pancreatic Cystic Fibrosis - Narrower
Concept UI M0455427
Preferred term Pancreatic Cystic Fibrosis
Entry term(s) Cystic Fibrosis of Pancreas
Cystic Fibrosis, Pancreatic
Fibrocystic Disease of Pancreas
Pancreas Fibrocystic Disease
Pancreas Fibrocystic Diseases



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