Treatments include discontinuation of protein intake, intravenous infusion of glucose and, as needed, infusion of supplemental arginine and the ammonia removal drugs, sodium phenylacetate and sodium benzoate.
Ornithine translocase deficiency
Holocarboxylase synthetase deficiency
Guanidinoacetate methyltransferase deficiency
Carbamoyl phosphate synthetase I deficiency
Malonyl-CoA decarboxylase deficiency
Aminoacylase 1 deficiency
Arginine:glycine amidinotransferase deficiency
Adenylosuccinate lyase deficiency