Medical Uses
Pheburane (sodium phenylbutyrate) is a nitrogen-binding agent indicated as adjunctive therapy to standard of care, which includes dietary management, for the chronic management of adult and pediatric patients with urea cycle disorders (UCDs), involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase.
Limitations of Use: This therapeutic drug is not indicated for the treatment of acute hyperammonemia.
Recommended Dosage:
The recommended dosage of Pheburane (measured as sodium phenylbutyrate) for patients with urea cycle disorders is:
- Patients weighing less than 20kg: 450 – 600 mg/kg/day of sodium phenylbutyrate orally. Divide the calculated total daily dose into three to six doses. Administer three to six divided doses and take with food.
- Patients weighing greater than or equal to 20 kg: 9.9 – 13 g/m2/day of sodium phenylbutyrate orally. Divide the calculated total daily dose into three to six doses. Administer as three to six divided doses and take with food.
The maximum dosage is 20 grams per day. Combine this treatment with dietary protein restriction and, in some cases, amino acid supplementation (e.g., essential amino acids, arginine, citrulline, and protein-free calorie supplements). Measure the dose using only the calibrated spoon provided in the packaging. This calibrated dosing spoon directly measures Pheburane oral pellets as sodium phenylbutyrate.
If a dose is missed, take the missed dose as quickly as possible. There should be at least 3 hours between two doses, and doses should not be doubled to make up for the missed dose.
