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Adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia

MedGen UID:
907576
Concept ID:
C4274082
Disease or Syndrome
Synonyms: Adult onset non-insulinoma persistent hyperinsulinemic hypoglycemia; adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia; NI-PHH; NIPHS; Non-insulinoma pancreatogenous hypoglycemia syndrome
SNOMED CT: Adult onset non-insulinoma persistent hyperinsulinemic hypoglycemia (717044000)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017189
Orphanet: ORPHA276608

Definition

Caused by abnormal insulin production by b-cells in the pancreas that can be diffuse or focal and is characterized by an excessive/ uncontrolled insulin secretion (inappropriate for the level of glycemia), recurrent episodes of profound hypoglycemia and resistance to medical management with diazoxide. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia

Recent clinical studies

Diagnosis

Gupta RA, Patel RP, Nagral S
JOP 2013 May 10;14(3):286-8. doi: 10.6092/1590-8577/1352. PMID: 23669482

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