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Hyperion Therapeutics Completes Enrollment in Phase 1/2 Clinical Trial in Patients With Urea Cycle Disorders
Date:7/22/2008

liquid form, provides an alternative pathway to the urea cycle for the disposal of waste nitrogen through the renal excretion of phenylacetylglutamine, which is formed from PAA and glutamine.

About BUPHENYL(R)

BUPHENYL(R) 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). BUPHENYL(R) 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(R) 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.

About Hyperion Therapeutics

Hyperion Therapeutics is a specialty therapeutics company focused on the development of therapies that address orphan or underserved patient populations with unmet medical needs. Hyperion is headquartered in South San Francisco, CA. For additional information, visit: http://www.hyperiontx.com.

BUPHENYL is a registered trademark of Ucyclyd Pharma, Inc.

Full Prescribing Information for BUPHENYL(R) is available at http://www.
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SOURCE Hyperion Therapeutics, Inc.
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