RAVICTI is indicated for use as a nitrogen-binding agent for chronic management of
patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein
restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary
protein restriction and, in some cases, dietary supplements (e.g., essential amino acids,
arginine, citrulline, protein-free calorie supplements).
RAVICTI is indicated for use as a nitrogen-binding agent for chronic management of
patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein
restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary
protein restriction and, in some cases, dietary supplements (e.g., essential amino acids,
arginine, citrulline, protein-free calorie supplements).
The recommended dosage range, based upon body surface area, in patients naïve to
phenylbutyrate (PBA) is 4.5 to 11.2 mL/m2
/day (5 to 12.4 g/m2
/day). For patients with some
residual enzyme activity who are not adequately controlled with protein restriction, the
recommended starting dosage is 4.5 mL/m2
/day.
In determining the starting dosage of RAVICTI in treatment-naïve patients, consider the
patient’s residual urea synthetic capacity, dietary protein requirements, and diet adherence.
Dietary protein is approximately 16% nitrogen by weight. Given that approximately 47% of
dietary nitrogen is excreted as waste and approximately 70% of an administered PBA dose
will be converted to urinary phenylacetylglutamine (U-PAGN), an initial estimated
RAVICTI dose for a 24-hour period is 0.6 mL RAVICTI per gram of dietary protein ingested
per 24-hour period. The total daily dosage should not exceed 17.5 mL.
• Neurotoxicity [see Warnings and Precautions (5.1)]
• Pancreatic insufficiency or Intestinal Malabsorption [