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Start free with EleplanDiazoxide-resistant hyperinsulinism
ORPHA:276585Clinical group
Also called Diazoxide-resistant hyperinsulinemic hypoglycemia
What it is
A form of congenital isolated hyperinsulism 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. Pancreatic involvement can be diffuse or focal.
Key facts
- Classified as
- Clinical group
Recorded for the broader condition
- Prevalence
- 1-5 / 10 000 (China)Congenital isolated hyperinsulinism
- Age of onset
- Childhood, Infancy, NeonatalCongenital isolated hyperinsulinism
- Inheritance
- Autosomal dominant, Autosomal recessiveCongenital isolated hyperinsulinism
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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