Isovaleric acidemia (IVA) (McKusick 243500) is a rare inherited condition, caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10), leading to accumulation of isovaleryl-CoA and its metabolites including free isovaleric acid, 3-hydroxyisovalerate and N-isovalerylglycine. In Europe, dietary management of isovaleric acidemia (IVA) may vary widely. In Feb 27, 2017, A. Pinto and others published an article in << Mol Genet Metab Rep >> which title is “Dietary practices in isovaleric acidemia: A European survey”, reported the dietary practices in isovaleric acidemia at Europe. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability.
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