Sodium Phenylacetate

(USAN)
Sodium Phenylacetate Chemical formula

💊 Chemical information

Fenilacetato de sodio.
Chemical formula: C8H7NaO2 = 158.1.
CAS — 114-70-5.

💊 Profile

Sodium phenylacetate is used as adjunctive treatment for acute hyperammonaemia and associated encephalopathy in patients with enzymatic deficiencies in the urea cycle. It is given with sodium benzoate as a combined preparation for intravenous infusion in which 1 mL contains 100 mg of each component. The preparation is diluted in sterile glucose injection 10% at ≥25 mL/kg before infusion. Other similar therapies (e.g. oral sodium phenylbutyrate, see below) should be stopped before starting the infusion. A loading dose is infused over 90 to 120 minutes followed by an equivalent maintenance dose infused over 24 hours. Doses of sodium phenylacetate (together with the same amount of sodium benzoate) are 250 mg/kg for patients weighing 20 kg or less, and 5.5 g/m2 for those over 20 kg. Maintenance infusions are continued until plasma ammonia concentrations are normal or oral nutrition and therapy can be tolerated. Sodium phenylacetate has also been given by mouth.
1. The Urea Cycle Disorders Conference Group. Consensus statement from a conference for the management of patients with urea cycle disorders. J Pediatr 2001; 138 (suppl 1): S1–S5
2. Summar M. Current strategies for the management of neonatal urea cycle disorders. J Pediatr 2001; 138 (suppl 1): S30–S39
3. Batshaw ML, et al. Alternative pathway therapy for urea cycle disorders: twenty years later. J Pediatr 2001; 138 (suppl 1): S46–S55. Correction. ibid. 2002; 140: 490
4. MacArthur RB, et al. Pharmacokinetics of sodium phenylacetate and sodium benzoate following intravenous administration as both a bolus and continuous infusion to healthy adult volunteers. Mol Genet Metab 2004; 81 (suppl 1): S67–S73
5. Enns GM, et al. Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. N Engl J Med 2007; 356: 2282–92.

💊 Preparations

Proprietary Preparations

Multi-ingredient: USA: Ammonul; Ucephan.
Published May 08, 2019.