Inborn errors of metabolism:
Indications for: AMMONUL
Acute hyperammonemia with encephalopathy in urea cycle disorders, as adjunctive therapy.
Adults and Children:
Discontinue sodium phenylbutyrate first. Give by IV infusion through central venous catheter. Loading dose: Infuse over 90–120 minutes. Maintenance dose: Infuse over 24 hours; continue until ammonia levels normalize or patient can tolerate oral nutrition and medications. 0–20kg: 2.5mL/kg. >20kg: 55mL/m2. Discontinue if extravasation occurs; may resume infusion at different site. Concomitant L-arginine dose dependent upon specific enzyme defiency. See full labeling.
Do not administer undiluted product. Uncontrolled hyperammonemia (may require hemodialysis). Renal or hepatic insufficiency. CHF. Sodium retention with edema. Perform blood chemistry profiles. Monitor ammonia levels, blood pH and pCO2, electrolytes, neurological status, and clinical response. May give antiemetic to reduce nausea and vomiting. Restrict dietary protein. Maintain adequate caloric intake. Labor & delivery. Pregnancy. Nursing mothers.
Corticosteroids may increase ammonia levels. May be antagonized by drugs that compete for active renal tubule secretion (eg, penicillin), probenecid, valproic acid.
GI upset, convulsions, mental impairment, hyperglycemia, hypokalemia.