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Ammonul

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Ammonul

INDICATIONS

AMMONUL 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].

DOSAGE AND ADMINISTRATION

Recommended Dose

AMMONUL must be diluted with sterile 10% Dextrose Injection (D10W) before administration. The dilution and dosage of AMMONUL 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 AMMONUL must be diluted with sterile 10% Dextrose Injection at ≥ 25mL/Kg before administration. Dosage Provided
  AMMONUL 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

Administration

AMMONUL 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. AMMONUL may not be administered by any other route.

AMMONUL 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 AMMONUL 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 AMMONUL infusion to aid control of infusion-associated nausea and vomiting. Administration of analogous oral drugs, such as sodium phenylbutyrate, should be terminated prior to AMMONUL infusion.

AMMONUL 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 AMMONUL, 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 AMMONUL administration [see WARNINGS AND PRECAUTIONS]. In the non-neonatal study patient population treated with AMMONUL, 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 AMMONUL is used with hemodialysis, as the ammonia-scavenging of AMMONUL suppresses the production of ammonia from catabolism of endogenous protein and hemodialysis eliminates the ammonia and ammonia conjugates.

AMMONUL 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 AMMONUL infusion solutions except for Arginine HCl Injection, 10%, which may be mixed in the same container as AMMONUL. Other infusion solutions and drug products should not be administered together with AMMONUL infusion solution. AMMONUL solutions may be prepared in glass and PVC containers.

Arginine Administration

Intravenous arginine is an essential component of therapy for patients with carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), or argininosuccinate lyase (ASL) deficiency. Because hyperchloremic acidosis may develop after high-dose arginine hydrochloride administration, chloride and bicarbonate levels should be monitored and appropriate amounts of bicarbonate administered.

In hyperammonemic infants with suspected, but unconfirmed urea cycle disorders, intravenous arginine should be given (6 mL/kg of Arginine HCl Injection 10%, over 90 minutes followed by the same dose given as a maintenance infusion over 24 hours). If deficiencies of ASS or ASL are excluded as diagnostic possibilities, the intravenous dose of arginine HCl should be reduced to 2 mL/kg/day Arginine HCl Injection 10%.

Converting To Oral Treatment

Once elevated ammonia levels have been reduced to the normal range, oral therapy, such as sodium phenylbutyrate, dietary management and maintenance protein restrictions should be started or reinitiated.

HOW SUPPLIED

Dosage Forms And Strengths

AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10% per 10% is a sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate.

Storage And Handling

AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10% per 10% is supplied in a single use glass vial.

NDC 62592-720-50 single use vial containing 50 mL of sodium phenylacetate and sodium benzoate injection 10% per 10%.

Storage: Store at 25°C (77°F), excursions permitted to 15° - 30°C (59°86°F).

Manufactured by: Chesapeake Biological Laboratories, Inc., 1111 South Paca Street, Baltimore MD 21230. Manufactured for: Ucyclyd Pharma, Inc., a wholly-owned subsidiary of Medicis Pharmaceutical Corp., 7720 N. Dobson Road, Scottsdale, AZ 85256. Revised: June 2011

Last reviewed on RxList: 7/27/2011
This monograph has been modified to include the generic and brand name in many instances.

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