Sodium Phenylacetate and Sodium Benzoate

SODIUM PHENYLACETATE AND SODIUM BENZOATE- sodium phenylacetate and sodium benzoate injection, solution, concentrate
Mylan Institutional LLC

1 INDICATIONS AND USAGE

Sodium phenylacetate and sodium benzoate injection is indicated as adjunctive therapy in pediatric and adult patients for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle. During acute hyperammonemic episodes, arginine supplementation, caloric supplementation, dietary protein restriction, hemodialysis, and other ammonia lowering therapies should be considered [see Warnings and Precautions (5) ].

2 DOSAGE AND ADMINISTRATION

2.1 Recommended Dose

Sodium phenylacetate and sodium benzoate injection must be diluted with sterile 10% Dextrose Injection (D10W) before administration. The dilution and dosage of sodium phenylacetate and sodium benzoate injection are determined by weight for neonates, infants and young children, and by body surface area for larger patients, including older children, adolescents, and adults (Table 1).

Table 1. Dosage and Administration

Patient Population

Components of Infusion Solution

Sodium Phenylacetate and Sodium Benzoate Injection must be diluted with sterile 10% Dextrose Injection at ≥ 25 mL/kg before administration.

Dosage Provided

Sodium Phenylacetate and Sodium Benzoate Injection

Arginine HCl Injection, 10%

Sodium Phenylacetate

Sodium Benzoate

Arginine HCl

Patients 0 to 20 kg:

CPS and OTC Deficiency

Dose

Loading: over 90 to 120 minutes Maintenance: over 24 hours

2.5 mL/kg

2 mL/kg

250 mg/kg

250 mg/kg

200 mg/kg

ASS and ASL Deficiency

Dose

Loading: over 90 to 120 minutes Maintenance: over 24 hours

2.5 mL/kg

6 mL/kg

250 mg/kg

250 mg/kg

600 mg/kg

Patients > 20 kg:

CPS and OTC Deficiency

Dose

Loading: over 90 to 120 minutes Maintenance: over 24 hours

55 mL/m2

2 mL/kg

5.5 g/m2

5.5 g/m2

200 mg/kg

ASS and ASL Deficiency

Dose

Loading: over 90 to 120 minutes Maintenance: over 24 hours

55 mL/m2

6 mL/kg

5.5 g/m2

5.5 g/m2

600 mg/kg

2.2 Administration

Sodium phenylacetate and sodium benzoate injection is a concentrated solution and must be diluted before intravenous administration via a central venous catheter. Administration through a peripheral intravenous catheter may cause burns. Sodium phenylacetate and sodium benzoate injection may not be administered by any other route.

Sodium phenylacetate and sodium benzoate injection should be administered as a loading dose infusion over 90 to 120 minutes, followed by the same dose repeated as a maintenance infusion administered over 24 hours. Because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies, repeat loading doses of sodium phenylacetate and sodium benzoate injection should not be administered. Maintenance infusions may be continued until elevated plasma ammonia levels have been normalized or the patient can tolerate oral nutrition and medications. An antiemetic may be administered during sodium phenylacetate and sodium benzoate injection infusion to aid control of infusion-associated nausea and vomiting. Administration of analogous oral drugs, such as sodium phenylbutyrate, should be terminated prior to sodium phenylacetate and sodium benzoate injection infusion.

Sodium phenylacetate and sodium benzoate injection infusion should be started as soon as the diagnosis of hyperammonemia is made. Treatment of hyperammonemia also requires caloric supplementation and restriction of dietary protein. Non-protein calories should be supplied principally as glucose (8–10 mg/kg/min) with an intravenous fat emulsion added. Attempts should be made to maintain a caloric intake of greater than 80 kcal/kg/day. During and after infusion of sodium phenylacetate and sodium benzoate injection, ongoing monitoring of the following clinical laboratory values is crucial: plasma ammonia, glutamine, quantitative plasma amino acids, blood glucose, electrolytes, venous or arterial blood gases, AST and ALT. On-going monitoring of the following clinical responses is also crucial to assess patient response to treatment: neurological status, Glasgow Coma Scale, tachypnea, CT or MRI scan or fundoscopic evidence of cerebral edema, and/or of gray matter and white matter damage. Hemodialysis should be considered in patients with severe hyperammonemia or who are not responsive to sodium phenylacetate and sodium benzoate injection administration [see Warnings and Precautions (5.1) ]. In the non-neonatal study patient population treated with sodium phenylacetate and sodium benzoate injection, dialysis was required in 13% of hyperammonemic episodes. Standard hemodialysis was the most frequently used dialysis method. High levels of ammonia can be reduced quickly when sodium phenylacetate and sodium benzoate injection is used with hemodialysis, as the ammonia-scavenging of sodium phenylacetate and sodium benzoate injection suppresses the production of ammonia from catabolism of endogenous protein and hemodialysis eliminates the ammonia and ammonia conjugates.

Sodium phenylacetate and sodium benzoate injection solutions are physically and chemically stable for up to 24 hours at room temperature and room lighting conditions. No compatibility information is presently available for sodium phenylacetate and sodium benzoate injection infusion solutions except for Arginine HCl Injection, 10%, which may be mixed in the same container as sodium phenylacetate and sodium benzoate injection. Other infusion solutions and drug products should not be administered together with sodium phenylacetate and sodium benzoate injection infusion solution. Sodium phenylacetate and sodium benzoate injection solutions may be prepared in glass and PVC containers.

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