Mitochondrial Hydroxyacyl-Coenzyme A Dehydrogenase Hyperinsulinism

**Semantic type:** Disease or Syndrome

**Definition:** Hyperinsulinism due to inactivating mutation(s) in the gene HADH, encoding mitochondrial (short-chain) hydroxyacyl-coenzyme A dehydrogenase, resulting in loss of inhibition of glutamate dehydrogenase (GDH). This condition is also characterized by protein-induced hypoglycemia, but in contrast to GLUD1-associated hyperinsulinism, hyperammonemia is absent.

**Synonyms:** - HADH-Associated Hyperinsulinism - SCHAD Hyperinsulinism