What is Urea Cycle Disorders?

Hemodialysis is very effective at reducing plasma ammonia and should immediately be initiated if elevated hyperammonemia is observed. Ammonia scavenger medications such as Ammonul IV are also useful. Ammonul IV acts by removing glycine and glutamate from plasma thereby their reducing contribution to ammonia formation.

The medications that have been approved by the US FDA for the treatment of urea cycle disorders include Ammonul (Ucyclyd Pharma), Buphenyl (Horizon Therapeutics), and Ravicti (Horizon Therapeutics).

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Carglumic acid is approved by the FDA as adjunctive therapy for acute hyperammonemia and as a maintenance therapy for chronic hyperammonemia due to the deficiency of the hepatic enzyme N-acetyl glutamate synthase (NAGS). Ammonul (sodium phenylacetate and sodium benzoate) Injection is a sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate, used for the treatment of hyperammonemia in urea cycle disorders.

In the current market scenario, recommended therapies to possibly slow the progression of the disease include sodium benzoate, sodium phenylacetate, Glycerol phenylbutyrate, sodium phenylbutyrate, and Antiemetic agents. These drugs lower blood ammonia concentrations by conjugation reactions involving acylation of amino acids. Sodium phenylbutyrate is a prodrug, which is metabolized to phenylacetate.

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