Hyperinsulinism due to HNF1A deficiency

Orpha code: 324575OMIM code:

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; see this term) later in life.

Disease data
Classification

Disease

Synonyms
Hyperinsulinemic hypoglycemia due to HNF1A deficiency
Hipoglikemia hiperinsulinemiczna z powodu niedoboru HNF1A deficiency
ORPHA code
324575
OMIM code
-
ICD10 code
E16.1
ICD11 code
-

No additional description.

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