3 hydroxyisobutyric aciduria 3 hydroxyisobutyric aciduria 3-hydroxyisobutyric aciduria is a disorder of valine metabolism characterised by urinary excretion of 3-hydroxyisobutyric acid. 3 hydroxyisobutyric aciduriaother namesdisorder of valine metabolismthis condition is inherited in an autosomal recessive manner