label: Mitochondrial Hydroxyacyl-Coenzyme A Dehydrogenase Hyperinsulinism
label_lc: mitochondrial hydroxyacyl-coenzyme a dehydrogenase hyperinsulinism
description: "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."