FDA Approved Medical Products for:

N-acetylglutamate synthetase deficiency

The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products.

Generic Name Glycerol phenylbutyrate
Trade Name
(Manufacturer Name)
(Hyperion Therapeutics, Inc.)
The FDA has approved this product to be used in this manner.
Use as a nitrogen-binding adjunctive therapy for chronic management of adult and pediatric patients > or = to 2 years of age with urea cycle disorders (UCDs) that cannot be managed by dietary protein restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements).
More Information about this product Drug Information Portal