异戊酸血症是一种遗传病，其会导致白胺酸无法正常代谢，最后导致3-甲基丁酸的堆积。

此遗传病的发生率未知。

遗传方面，其遗传方式为体染色体隐性遗传。

医学导航：遗传代谢缺陷

代谢、k,c/g/r/p/y/i,f/h/s/l/o/e,a/u,n,m

k,cgrp/y/i,f/h/s/l/o/e,au,n,m,人名体征

药物(A16/C10)、中间产物(k,c/g/r/p/y/i,f/h/s/o/e,a/u,n,m)

Isovaleric acidemia, also called isovaleric aciduria or isovaleric acid CoA dehydrogenase deficiency, is a rare autosomal recessive metabolic disorder which disrupts or prevents normal metabolism of the branched-chain amino acid leucine. It is a classical type of organic acidemia.