Adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia
diseaseOn this page
Also known as NI-PHH
Summary
Adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia (MONDO:0017189) is a disease. A subtype of familial hyperinsulinism — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide)
- Phenotypes (HPO): 21
Clinical features
Signs & symptoms
Clinical features (HPO)
21 HPO clinical features (Orphanet curated; top 21 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000825 | Hyperinsulinemic hypoglycemia | Very frequent (80-99%) |
| HP:0000975 | Hyperhidrosis | Very frequent (80-99%) |
| HP:0000980 | Pallor | Very frequent (80-99%) |
| HP:0001254 | Lethargy | Very frequent (80-99%) |
| HP:0001259 | Coma | Very frequent (80-99%) |
| HP:0001337 | Tremor | Very frequent (80-99%) |
| HP:0001649 | Tachycardia | Very frequent (80-99%) |
| HP:0001985 | Hypoketotic hypoglycemia | Very frequent (80-99%) |
| HP:0002344 | Progressive neurologic deterioration | Very frequent (80-99%) |
| HP:0003162 | Fasting hypoglycemia | Very frequent (80-99%) |
| HP:0003324 | Generalized muscle weakness | Very frequent (80-99%) |
| HP:0004324 | Increased body weight | Very frequent (80-99%) |
| HP:0004510 | Pancreatic islet-cell hyperplasia | Very frequent (80-99%) |
| HP:0012378 | Fatigue | Very frequent (80-99%) |
| HP:0000842 | Hyperinsulinemia | Frequent (30-79%) |
| HP:0012051 | Reactive hypoglycemia | Frequent (30-79%) |
| HP:0000713 | Agitation | Occasional (5-29%) |
| HP:0001249 | Intellectual disability | Occasional (5-29%) |
| HP:0001250 | Seizure | Occasional (5-29%) |
| HP:0002315 | Headache | Occasional (5-29%) |
| HP:0002329 | Drowsiness | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia |
| Mondo ID | MONDO:0017189 |
| Orphanet | 276608 |
| SNOMED CT | 717044000 |
| UMLS | C4274082 |
| MedGen | 907576 |
| GARD | 0021056 |
| Is cancer (heuristic) | no |
Also known as: NI-PHH
Disease family
This is a subtype of familial hyperinsulinism. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › digestive system disorder › pancreas disorder › endocrine pancreas disorder › hyperinsulinism › familial hyperinsulinism › adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia
Related subtypes (2): hyperinsulinism due to INSR deficiency, congenital isolated hyperinsulinism
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 0.
Related Atlas pages
No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.