congenital isolated hyperinsulinism
Findings
No curated finding names congenital isolated hyperinsulinism yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Congenital isolated hyperinsulinism (CHI), a rare endocrine disease is the most frequent cause of severe and persistent hypoglycemia in the neonatal period and early infancy and is characterized by an excessive or uncontrolled insulin secretion (inappropriate for the level of glycemia) and recurrent episodes of profound hypoglycemia requiring rapid and intensive treatment to prevent neurological sequelae. CHI comprises 2 different forms: diazoxide-sensitive diffuse hyperinsulinism and diazoxide-resistant hyperinsulinism.
Definition from the Mondo Disease Ontology (MONDO:0019010), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
3 names
Resolves to: congenital isolated hyperinsulinism
- Also called
- chipersistent hyperinsulinemic hypoglycemia of infancyPHHI