Succinyl-CoA:3-ketoacid CoA transferase deficiency (SCOTD) is a defect in ketone body utilization characterized by severe, potentially fatal intermittent episodes of ketoacidosis. The disease is succinyl-CoA:3-ketoacid CoA transferase deficiency (Monarch Disease Ontology identifier MONDO_0009492). Also known as: OXCT1 deficiency, SCOT deficiency, Succinyl CoA:3-oxoacid CoA transferase deficiency, succinyl-CoA acetoacetate transferase deficiency, succinyl-CoA:3-oxoacid CoA transferase deficiency.