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Descriptor English: Nesidioblastosis
Descriptor Spanish: Nesidioblastosis
Descriptor nesidioblastosis
Entry term(s) hiperinsulinismo familiar con nesidioblastosis pancreática
Scope note: Síndrome autosómico recesivo hereditario caracterizado por la formación desorganizada de nuevos islotes en el PÁNCREAS e HIPERINSULINISMO CONGÉNITO. Este síndrome se debe a una hiperplasia focal de las CÉLULAS DE LOS ISLOTES pancreáticos que emergen desde las estructuras ductales y forman nuevos islotes de Langerhans. Las mutaciones en las células de los islotes afectan al gen del canal de potasio KCNJ11 o al gen del transportador de casete de unión a ATP ABCC8, ambos en el CROMOSOMA 11.
Descriptor Portuguese: Nesidioblastose
Descriptor French: Nésidioblastose
Entry term(s): Hyperinsulinism, Familial, with Pancreatic Nesidioblastosis
Nesidioblastoses
Nesidioblastoses, Pancreatic
Nesidioblastosis of Pancreas
Nesidioblastosis, Pancreatic
Pancreas Nesidioblastoses
Pancreas Nesidioblastosis
Pancreatic Nesidioblastoses
Pancreatic Nesidioblastosis
Tree number(s): C06.689.150.500
C16.614.200.500
C18.452.394.968.250.500
C18.452.394.984.200.500
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D046768
Scope note: 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.
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
Previous Indexing: Hyperinsulinism (1971-2004)
Hyperplasia (1968-2004)
Islets of Langerhans (1968-2004)
Public MeSH Note: 2005; see PANCREATIC DISEASES 1983-2004
History Note: 2005; use PANCREATIC DISEASES 1983-2004
Related: ATP-Binding Cassette Transporters MeSH
KATP Channels MeSH
DeCS ID: 38627
Unique ID: D046768
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2005/01/01
Date of Entry: 2004/07/07
Revision Date: 2012/07/03
Nesidioblastosis - Preferred
Concept UI M0015793
Scope note 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.
Preferred term Nesidioblastosis
Entry term(s) Hyperinsulinism, Familial, with Pancreatic Nesidioblastosis
Nesidioblastoses
Nesidioblastoses, Pancreatic
Nesidioblastosis of Pancreas
Nesidioblastosis, Pancreatic
Pancreas Nesidioblastoses
Pancreas Nesidioblastosis
Pancreatic Nesidioblastoses
Pancreatic Nesidioblastosis



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