Isovaleric acidaemia (IVA) is a rare, but potentially serious, inherited condition. It means the body cant process the amino acid leucine (amino acids are 'building blocks' of protein). This causes a harmful build-up of substance in the blood and urine.

Normally, our bodies break down protein foods like meat and fish into amino acids. Any amino acids that aren't needed are usually broken down and removed from the body.

Babies with IVA are unable to fully break down the amino acid leucine.

Normally, leucine is broken down into a substance called isovaleric acid, which is then converted into energy. Babies with IVA dont have the enzyme that breaks down isovaleric acid, leading to a harmfully high level of this substance in the body.

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Medically Reviewed by a doctor on 24 Nov 2016