BUPHENYL^®

BUPHENYL^® is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). It is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy. It is important that the diagnosis be made early and treatment initiated immediately to improve survival. Any episode of acute hyperammonemia should be treated as a life-threatening emergency. BUPHENYL^® must be combined with dietary protein restriction and, in some cases, essential amino acid supplementation.

Safety Information
BUPHENYL^® should not be administered to patients with known hypersensitivity to sodium phenylbutyrate or any component of this preparation. The most common adverse reactions associated with BUPHENYL^® were amenorrhea dysfunction, decreased appetite, body odor (probably caused by its metabolite phenylacetate) and bad taste or taste aversion. Patients with urea cycle disorders should not take valproic acid, haloperidol or steroids as these drugs have been reported to increase blood ammonia levels, and probenecid may affect the kidneys’ excretion. Use with great care, if at all, in patients with congestive heart failure or severe renal insufficiency, and in clinical states where there is sodium retention with edema. Use caution when administering to patients with hepatic or renal insufficiency or inborn errors of beta oxidation. The safety or efficacy of doses in excess of 20 grams (40 tablets) per day has not been established.

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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.

BUPHENYL is a registered trademark of Ucyclyd Pharma, Inc.

© 2008 Ucyclyd Pharma, Inc.