FDA Approved Medical Products for:

Urea cycle disorders

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 Benzoate and phenylacetate
Trade Name
(Manufacturer Name)
Ammonul®
(Ucyclyd Pharma, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Adjunctive therapy in the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.
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Generic Name Sodium phenylbutyrate
Trade Name
(Manufacturer Name)
Buphenyl®
(Ucyclyd Pharma, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Adjunctive therapy in the chronic managment of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase.
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Generic Name Glycerol phenylbutyrate
Trade Name
(Manufacturer Name)
Ravicti
(Hyperion Therapeutics, Inc.)
Indication
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).
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Generic Name Benzoate and phenylacetate
Trade Name
(Manufacturer Name)
Ucephan
(Immunex Immunex)
Indication
The FDA has approved this product to be used in this manner.
For adjunctive therapy in the prevention and treatment of hyperammonemia in patients with urea cycle enzymopathy due to carbamylphosphate synthetase, ornithine, transcarbamylase, or argininosuccinate synthetase deficiency.
More Information about this product Drug Information Portal