Isovaleric acidemia (IVA) is an autosomal recessively inherited organic aciduria characterized by a deficiency in isovaleryl-CoA dehydrogenase, that has wide clinical variability and that can present in infancy with acute manifestations of vomiting, failure to thrive, seizures, lethargy, a characteristic ''sweaty feet'' odor, acute pancreatitis and mild to severe developmental delay or in childhood with metabolic acidosis (brought on by prolonged fasting, an increased intake of protein-rich food or infections) and that can be fatal if not treated immediately. Chronic intermittent presentations and asymptomatic patients have also been reported. The disease is isovaleric acidemia (Monarch Disease Ontology identifier MONDO_0009475). Also known as: Isovalericacidemia, isovaleric acid CoA dehydrogenase deficiency, isovaleric aciduria, isovaleryl-CoA dehydrogenase deficiency.