AMMONUL- sodium phenylacetate and sodium benzoate injection, solution, concentrate
Ucyclyd Pharma Inc.
AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10% per 10% (a nitrogen binding agent), is sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate. The pH of the solution is between and 8. Sodium phenylacetate is crystalline, white to off-white powder with strong, offensive odor. It is soluble in water. Sodium benzoate is white and odorless, crystalline powder that is readily soluble in water.
Sodium phenylacetate has molecular weight of 158.13 and the molecular formula C8 H7 NaO2 Sodium benzoate has molecular weight of 144.11 and the molecular formula C7 H5 NaO2 .
Each mL of AMMONUL contains 100 mg of sodium phenylacetate and 100 mg of sodium benzoate, and Water for Injection. Sodium hydroxide and/or hydrochloric acid may have been used for pH adjustment.
AMMONUL injection is sterile, concentrated solution intended for intravenous administration via central line only after dilution
Mechanism of Action
Urea cycle disorders can result from decreased activity of any of the following enzymes: -acetylglutamate synthetase (NAGS), carbamyl phosphate synthetase (CPS), argininosuccinate synthetase (ASS), ornithine transcarbamylase (OTC), argininosuccinate lyase (ASL), or arginase (ARG).
Sodium phenylacetate and sodium benzoate are metabolically active compounds that can serve as alternatives to urea for the excretion of waste nitrogen. Figure is schematic illustrating how the components of AMMONUL, phenylacetate and benzoate, provide an alternative pathway for nitrogen disposal in patients without fully functioning urea cycle. Phenylacetate conjugates with glutamine in the liver and kidneys to form phenylacetylglutamine, via acetylation. Phenylacetylglutamine is excreted by the kidneys via glomerular filtration and tubular secretion
INDICATIONS AND USAGE
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
DOSAGE AND ADMINISTRATION
2.1 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
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.
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
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3 DOSAGE FORMS AND STRENGTHS
AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10% per 10% is sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate.
HOW SUPPLIED/STORAGE AND HANDLING
AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10% per 10% is supplied in 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?-
Processed in the USA with Foreign Ingredients
04100281
PRINCIPAL DISPLAY PANEL 50 mL Vial Carton
Ucyclyd
Pharma?
NDC 62592-720-50 Rx Only
AMMONUL ?
(sodium phenylacetate and
sodium benzoate) Injection
10% 10%
50 mL For IV use only