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Urea Cycle Disorders News

FDA Approves Ravicti for the Chronic Management of Some Urea Cycle Disorders

Posted 3 Feb 2013 by Drugs.com

February 1, 2013 – The U.S. Food and Drug Administration today approved Ravicti (glycerol phenylbutyrate) for the chronic management of some urea cycle disorders (UCDs) in patients ages 2 years and older. UCDs are genetic disorders that involve deficiencies of specific enzymes involved in the urea cycle, a series of biochemical steps normally required to remove ammonia from the blood. When protein is absorbed and broken down by the body, it produces nitrogen as a waste product. The urea cycle removes nitrogen from the blood and converts it to urea, which is removed from the body through urine. In people with UCDs, nitrogen accumulates and remains in the body as ammonia, which can travel to the brain and cause brain damage, coma or death. Ravicti, a liquid taken three times a day with meals, helps dispose of ammonia in the body. It is intended for patients whose UCD cannot be managed ... Read more

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Metabolic Disorder Including Congenital

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sodium phenylbutyrate, Buphenyl, Ravicti, glycerol phenylbutyrate