Pancreatic insufficiency or intestinal malabsorption may result in reduced or absent digestion of RAVICTI and/or absorption of phenylbutyrate and reduced control of plasma ammonia. Monitor ammonia levels closely in these patients.

Most common adverse reactions in 10% or more of patients are: diarrhea, flatulence, headache, nausea, vomiting, fatigue, decreased appetite, HA, dizziness, headache, upper abdominal (stomach) pain and rash.

Corticosteroids, valproic acid, or haloperidol may increase plasma ammonia levels; monitor ammonia levels closely when used concomitantly with RAVICTI. Probenecid may affect renal excretion of metabolites of RAVICTI including PAGN and PAA.

The use of RAVICTI in pregnant women may cause fetal harm. Breastfeeding is not recommended during RAVICTI treatment.

Please see full Prescribing Information for RAVICTI at www.hyperiontx.com .

About Hyperion Therapeutics

Hyperion Therapeutics, Inc. is a commercial stage biopharmaceutical company committed to developing and delivering life-changing treatments for orphan diseases and hepatology. For more information, please visit www.hyperiontx.com

Forward-Looking Statements

To the extent that statements contained in this press release are not descriptions of historical facts regarding Hyperion, they are forward-looking statements reflecting the current beliefs and expectations of management made pursuant to the safe harbor provisions of the Private Securities Litigation Reform Act of 1995. Words such as "may," "will," "expect," "anticipate," "estimate," "intend," and similar expressions (as well as other words or expressions referencing future events, conditions or circumstances) are intended to identify forward-looking statements. Examples of forward-looking statements contained in this press release include, among others, statements regarding our expectations regarding the timing of to establishing comprehensive payor coverage. Forward-looking statements in this release involve substantial risks and uncertainties that could cause future results, performance or achievements to differ significantly from those expressed or implied by the forward-looking statements. Such risks and uncertainties include, among others, risks related to: the ability to comply with requirements established by the FDA, including post-marketing requirements and label restrictions; the success and timing of commercialization efforts of Ravicti; the uncertain clinical development process; the satisfaction of the FDA with preclinical data, including carcinogenicity data; the fact that the patient population suffering from UCD is small and has not been established with precision; the ability to obtain adequate clinical supplies of Ravicti; and the ability to raise sufficient capital to complete the development of and commercialize Ravicti in UCD. Hyperion undertakes no obligation to update or revise any forward-looking statements. For a further description of the risks and uncertainties that could cause actual results to differ from those expressed in these forward-looking statements, as well as risks relating to the business of the company in general, see the section entitled "Risk Factors" in Hyperion's Annual Report on Form 10-K/A for the year ended December 31, 2012, and in subsequent filings with the Securities and Exchange Commission.
CONTACT: Shari Annes, Investor Relations         (650) 888-0902         sannes@annesassociates.com                  Kristie Kuhl, Media Relations         (203) 556-7417         kkuhl@makovsky.com

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