AMMONUL^®

AMMONUL^® is indicated as adjunctive therapy for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle. In acute neonatal hyperammonemic coma, in moderate to severe episodes of hyperammonemic encephalopathy, and in episodes of hyperammonemia which fail to respond to an initial course of AMMONUL^® therapy, hemodialysis is the most rapid and effective technique for removing ammonia. In such cases, the concomitant administration of AMMONUL^® can help prevent the re-accumulation of ammonia by increasing waste nitrogen excretion. Treatment of hyperammonemia also requires caloric supplementation and restriction of dietary protein; intravenous arginine is an essential component of therapy for patients with CPS, OTC, ASS, or ASL deficiency.

Safety Information
The most common adverse reactions are vomiting (9%), hypokalemia (7%), hyperglycemia (7%), convulsions (6%), and mental impairments (6%). Do not administer to patients with known hypersensitivity to sodium phenylacetate or sodium benzoate. Acute symptomatic hyperammonemia should be treated as life-threatening. Uncontrolled hyperammonemia can result in brain damage or death. Dialysis may be required, preferably hemodialysis, to remove a large burden of ammonia. Administration must be through a central line; use of a peripheral line may cause burns. Do not administer undiluted product. Because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies, repeat loading doses should not be administered. Use caution when administering to patients with hepatic or renal insufficiency. AMMONUL^® may cause nausea and vomiting. An antiemetic may be administered during infusion. See the prescribing information for a complete listing of warnings, precautions, and drug interactions.

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About Urea Cycle Disorders
A urea cycle disorder (UCD) is an inherited, inborn error of metabolism. Patients with a UCD lack or are deficient in one of the key enzymes that comprise the urea cycle, the body’s primary vehicle for removing ammonia, a potent neurotoxin, from the bloodstream. Left untreated, UCDs can cause dangerously heightened levels of ammonia in the bloodstream (hyperammonemia) resulting in brain damage, coma, and/or death.

Additional information on urea cycle disorders is available from The National Urea Cycle Disorders Foundation (NUCDF), a nonprofit organization dedicated to the identification, treatment and cure of urea cycle disorders, and The Urea Cycle Disorders Consortium (UCDC) of the Rare Diseases Clinical Research Network, a National Institutes of Health (NIH)-funded research initiative.

AMMONUL is a registered trademark of Ucyclyd Pharma, Inc.

© 2008 Ucyclyd Pharma, Inc.