Hyperinsulinism due to HNF1A deficiency
Hyperinsulinism due to HNF1A deficiency
Definition
Hyperinsulinism due to HNF1A deficiency is a form of diazoxide-sensitive diffuse hyperinsulinism (DHI), characterized by transient or persistent hyperinsulinemic hypoglycemia (HH) in infancy that is responsive to diazoxide, evolving in to maturity-onset diabetes of the young subtype 1 (MODY-1) later in life.
Also known as hyperinsulinemic hypoglycemia due to HNF1A deficiency — per MONDO