The Medical Letter on Drugs and Therapeutics
Glycerol Phenylbutyrate (Ravicti) for Urea Cycle Disorders
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The FDA has approved an oral liquid formulation of glycerol phenylbutyrate (Ravicti – Hyperion) for chronic management of patients ≥2 years old with urea cycle disorders that cannot be adequately managed by a protein- restricted diet. Sodium phenylbutyrate (Buphenyl, and generics), another oral drug approved by the FDA for this indication, has a bitter taste. The new product, which contains no sodium, has little or no taste. Either drug must be used in addition to a protein-restricted diet and, if needed, dietary supplementation with amino acids and other nutrients.

UREA CYCLE DISORDERS — Deficiencies in the enzymes that convert nitrogen from ammonia into urea are the cause of urea cycle disorders, which can present at birth. Accumulation of ammonia can lead to neurologic toxicity ranging from mild cognitive impairment to cerebral edema, seizures, coma, and death. ... more      

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Title: Glycerol Phenylbutyrate (Ravicti) for Urea Cycle Disorders
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