Isovaleric acidemia (IVA)


Isovaleric acidemia (IVA) is an inherited condition in which the body is unable to break down certain proteins properly. It is an organic acid condition because it can lead to a harmful buildup of organic acids and toxins in the body. Health problems related to IVA range from very mild to life-threatening. If untreated, IVA can cause brain damage and even death. Treatment usually includes a special diet and medication. If the condition is identified early in life and proper treatment is begun, children with IVA often can lead healthy lives.

This condition is also known as: isovaleric acid-CoA dehydrogenase deficiency, IVD deficiency, isovaleryl CoA carboxylase deficiency.

For more information about Isovaleric acidemia, visit Baby's First Test: Isovaleric acidemia and Screening, Technology, and Research in Genetics (STAR-G): Isovaleric Acidemia. Please see Medically Prescribed Formula for information about medically prescribed formula and pharmacologic interventions for inborn errors of metabolism. Visit Board Certified Metabolic Providers (PDF: 2 pages/204KB) for a listing of medical specialists and clinic contact information.