Ammonul

About Ammonul:

• In patients with hyperammonemia due to deficiencies in enzymes of the urea cycle, Ammonul has been shown to decrease elevated plasma ammonia levels and improve encephalopathy and outcome of survival.
• The phenylacetate and benzoate components provide an alternative pathway for nitrogen disposal in patients without a proper functioning urea cycle. 
• Concomitant administration of Ammonul can help prevent the re-accumulation of ammonia by inducing waste nitrogen excretion.

Strength: Ammonul is sodium phenylacetate and sodium benzoate injection 10% per 10% is a sterile, concentrated, aqueous solution. Single-use vial containing 50 mL of sodium phenylacetate and sodium benzoate injection 10% per 10%.

Recommended Dosage:

• Ammonul must be administered through a central line. Administration through a peripheral site can cause burns.
• Ammonul is used as an intravenous administration as a loading dose infusion over 90 to 120 minutes, followed by an equivalent maintenance dose infusion over 24 hours. 
• Administration of any analogous oral drugs, such as sodium phenylbutyrate, should be discontinued prior to Ammonul infusion.

Important:

• All patients should be promptly hemodialyzed.
• A target blood flow of 150 mL/min/m2 may be attained using a 7F catheter. 
• 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. 
• Attempts should be made to maintain a caloric intake of greater than 80 cal/kg/d. 
• Monitoring of neurological status, plasma ammonia levels, clinical laboratory values, and clinical responses is crucial to evaluate patient response to treatment. 
• Patients who are not responsive to Ammonul require aggressive therapy including hemodialysis.
• Ammonul must be diluted with sterile dextrose Injection, 10% before administration. 
• The dilution and dosage are determined based on weight for neonates, infants, and young children, and by body surface area for larger patients, including older children, adolescents, and adults. 
• Maintenance infusions may be continued until elevated plasma ammonia levels have been normalized or the patient can tolerate oral nutrition and medications.

Warnings & Precautions: 

• Do not administer undiluted product as it is a concentrated product.
• Uncontrolled hyperammonemia may require hemodialysis.
• Blood chemistry profiles to be performed. 
• Ammonia levels, blood pH and pCO2, electrolytes, neurological status, and clinical response to be monitored.
• May require antiemetic in the event of nausea and vomiting. 
• Dietary protein to be restricted and adequate calorie intake to be maintained

Common Ammonul Side Effects

• Nervous system disorders
• metabolism and nutrition disorders
• Respiratory, thoracic, and mediastinal disorders
• Vomiting 
• Hyperglycemia 
• Hypokalemia 
• Convulsions 
• Mental impairment
• Metabolic acidosis
• Injection-site reactions overdose
• Subdural hematoma
• Hyperammonemia
• Hypoglycemia

Use in Specific Population

• Gender: Bioavailability of both benzoate and phenylacetate was slightly higher in females than in males as per studies.
• Hepatic Insufficiency: Limited information is available on the metabolism and excretion of sodium phenylacetate and sodium benzoate in patients with impaired hepatic function. 
• Renal Impairment: Renal clearance of Ammonul metabolites is required, therefore, patients with impaired renal function should be monitored.
• Dialysis: Intravenous administration of Ammonul is complementary with the use of dialysis. It is used for ammonia-scavenging and suppresses the production of ammonia and dialysis eliminates the ammonia and ammonia conjugates.

Storage and Handling

• Ammonul is stored at 25°C (77°F); excursions permitted to 15°C to 30°C (59°F to 86°F)