Діазоксидрезистентний гіперінсулінізм
ORPHA:276585· Diazoxide-resistant hyperinsulinism
Визначення(English summary)
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.