Congenital isolated hyperinsulinism
Congenital isolated hyperinsulinism
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.
Also known as chi, Congenital Hyperinsulinism, persistent hyperinsulinemic hypoglycemia of infancy, PHHI — per MONDO
Also identified as
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Alimentary part of gastrointestinal system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Alimentary part of gastrointestinal system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Endocrine gland | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Pancreas | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Pancreas | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |