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CYSTIC FIBROSIS
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DeCS
Descriptor
English
:
Cystic Fibrosis
Descriptor
Spanish
:
fibrosis quística
Descriptor
Portuguese
:
Fibrose Cística
Synonyms
English
:
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:
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
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
DG
diagnostic imaging
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics
HI
history
IM
immunology
ME
metabolism
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology
PC
prevention & control
PX
psychology
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
UR
urine
VE
veterinary
VI
virology
Record Number:
3568
Unique Identifier:
D003550
Occurrence in VHL
:
Similar:
DeCS