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NESIDIOBLASTOSIS
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DeCS
Descriptor
English
:
Nesidioblastosis
Descriptor
Spanish
:
Nesidioblastosis
Descriptor
Portuguese
:
Nesidioblastose
Synonyms
English
:
Hyperinsulinism, Familial, with Pancreatic Nesidioblastosis
Tree Number:
C06.689.150.500
C16.614.200.500
C18.452.394.968.250.500
C18.452.394.984.200.500
Definition
English
:
An inherited autosomal recessive
syndrome
characterized by the disorganized formation of new islets in the
PANCREAS
and
CONGENITAL HYPERINSULINISM
. It is due to focal
hyperplasia
of pancreatic ISLET
CELLS
budding off from the ductal structures and forming new
islets of Langerhans
. Mutations in the islet
cells
involve the
potassium
channel gene KCNJ11 or the ATP-binding cassette transporter gene ABCC8, both on CHROMOSOME 11.
See Related
English
:
ATP-Binding Cassette Transporters
KATP Channels
History Note
English
:
2005; use PANCREATIC DISEASES 1983-2004
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
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
RA
radiography
RI
radionuclide imaging
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
US
ultrasonography
UR
urine
VE
veterinary
VI
virology
Record Number:
38627
Unique Identifier:
D046768
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS