Deficiencies in enzymes of the urea cycle

Also known as: Urea cycle enzyme deficiency / Disorders of urea cycle metabolism / Disorder of urea cycle metabolism, unspecified / Urea cycle disorder / Urea Cycle Disorders, Inborn / Disorder of the urea cycle metabolism (disorder)

DrugDrug NameDrug Description
DB13207Arginine glutamateTwo amino acids indicated in the treatment of hyperammonemia and in combination for total parenteral nutrition.
DB09269Phenylacetic acidAn agent used as an adjunct to treat acute hyperammonemia and associated encephalopathy in adult and pediatric patients with deficiencies in enzymes of the urea cycle.
DB06819Phenylbutyric acidAn agent indicated for the adjunctive therapy for the management of chronic urea cycle disorders due to deficiencies in specific enzymes, including the neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy.
DrugDrug NamePhaseStatusCount