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New drugs and dosage forms.

690 Am J Health-Syst Pharm—Vol 67 May 1, 2010 Carglumic acid approved to treat genetic hyperammonemia FDA on March 18 approved the marketing of carglumic acid, or Carbaglu, for the treatment of a rare genetic enzyme deficiency that results in elevated levels of ammonia in the blood and can be fatal. N-acetylglutamate synthase deficiency is the rarest disorder of the urea cycle, according to Paris-based Orphan Europe, manufacturer of the new treatment. Without that enzyme, N-acetylglutamate does not form and activate the first enzyme of the urea cycle. The consequence is a buildup of ammonia, which can eventually cross the blood–brain barrier and cause neurologic problems, cerebral edema, coma, and death. Carglumic acid, the company said, resembles N-acetylglutamate sufficiently to activate the first enzyme of the urea cycle and quickly reduce plasma ammonia levels to normal in patients deficient in N-acetylglutamate synthase. The FDA-approved labeling for carglumic acid describes the drug as efficacious in helping to treat acute hyperammonemia due to N-acetylglutamate synthase deficiency and in treating chronic hyperammonemia due to the disorder. Children and adults with acute hyperammonemia, the labeling states, should initially receive 100–250 mg/kg/day of carglumic acid. The dosage should be adjusted on the basis of the patient’s plasma ammonia concentration and clinical symptoms. For the treatment of chronic hyperammonemia, patients should receive a maintenance dosage that targets the normal plasma ammonia concentration for their age. A company-conducted longterm follow-up of 22 patients treated with carglumic acid found that most received a maintenance dosage of less than 100 mg/kg/day. Patients should not swallow whole or crushed carglumic acid tablets. The labeling states that all doses are to be prepared as a liquid. Instructions for preparing and administering the liquid are provided in the labeling. The most common adverse events revealed in the long-term follow-up of patients were vomiting, abdominal pain, pyrexia, and tonsillitis. Orphan Europe will market Carbaglu in bottles containing 5 or 60 200-mg tablets. Until opened, the bottles must be stored in a refrigerator at 2–8 °C. Once opened, bottles are to be stored at room temperatures not exceeding 30 °C and the contents discarded in one month. The company had not responded by presstime to a query about distribution in the United States. —Cheryl A. Thompson DOI 10.2146/news100031