A Clinical Study of Glycerol Phenylbutyrate in Chinese Patients With Urea Cycle Disorders

"Administration and dosage: The recommended total daily dose of glycerol phenylbutyrate is calculated based on the patient's body surface area, ranging from 4.5 mL/m2/d to 11.2 mL/m2/d:~1. Recommended initial dose in phenylbutyrate-naïve patients~ * Patients with body surface area (BSA) \< 1.3 m2: 8.5 mL/m2/day~ * Patients with body surface area (BSA) ≥ 1.3 m2: 7 mL/m2/day~2. Initial dose for patients switching from sodium phenylbutyrate to glycerol phenylbutyrate: Total daily dose of glycerol phenylbutyrate (ml) = total daily dose of sodium phenylbutyrate granules (g) ×0.81 Medication Schedule: The daily total dose should be divided into equal amounts and administered with each meal or feeding (such as three to six times daily)."