Isovaleric Acidemia (IVA)



Isovaleric acidemia (IVA) is an inherited condition in which the body is unable to break down certain proteins properly, which can lead to a harmful buildup of organic acids and toxins in the body. If untreated, IVA can cause brain damage and even death. However, if the condition is identified early in life and proper treatment is begun, children with IVA often lead healthy lives. There are two main forms of IVA, each differing by the age of onset. In one form, signs appear 1 to 14 days after birth. In the other form, signs of IVA do not appear until later in infancy or childhood (called chronic/intermittent IVA). These two forms share similar signs, but the signs of chronic/intermittent IVA are less severe than IVA in babies. Signs of IVA include poor appetite, sleeping longer or more often, tiredness, vomiting, a “sweaty feet” smell, and seizures (epilepsy). Many of these signs may occur when a baby eats foods the body cannot break down. Symptoms can be triggered by long periods of time without eating, illnesses, and infections.

Condition Type:

Core Conditions


Isovaleric acidemia (IVA) is estimated to affect 1 out of every 230,000 babies born in the United States.

More Information for Parents:
Also known as:
  • IVA
  • Isovaleric acid-CoA dehydrogenase deficiency
  • IVD deficiency
  • Isovaleryl CoA carboxylase deficiency

Core Conditions