ABSTRACT
Another patient with 3-hydroxyisobutyric acidemia who had malformations, massive acidosis and hypotonia [3] was found to have defective activity of 3-hydroxyisobutyryl CoA deacylase [4]. 3-Hydroxyisobutyric aciduria and 3-aminoisobutyric aciduria has been reported along with hypermethioninemia in an apparently healthy baby [5].
