INDICATIONS AND USAGE（适应症）

DOSAGE（服用剂量）

For oral use only.

The use of BUPHENYL Tablets is indicated for children weighing more than 20 kg and for adults.

The usual total daily dose of BUPHENYL Tablets and Powder for patients with urea cycle disorders is 450–600 mg/kg/day in patients weighing less than 20 kg, or 9.9–13.0 g/m2/day in larger patients. The tablets and powder are to be taken in ­equally divided amounts with each meal or feeding (i.e., three to six times per day).

BUPHENYL Powder is indicated for oral use (via mouth, gastrostomy, or nasogastric tube) only. The powder is to be mixed with food (solid or liquid) for immediate use; however, when dissolved in water, BUPHENYL Powder has been shown to be stable for up to one week at room temperature or refrigerated. Sodium phenylbutyrate is very soluble in water (5 grams per 10 mL). When BUPHENYL Powder is added to a liquid, only sodium phenylbutyrate will dissolve, the excipients will not. The effect of food on sodium phenylbutyrate has not been determined.

Each level teaspoon (enclosed) dispenses 3.2 grams of powder and 3.0 grams of sodium phenylbutyrate. Each level tablespoon (enclosed) dispenses 9.1 grams of powder and 8.6 grams of sodium phenylbutyrate.

Shake lightly before use.

ADVERSE REACTIONS（不良反应）

Acidosis

Alkalosis

Hyperchloremia

Hypophosphatemia

Anemia

Leukopenia

Leukocytosis

Thrombocytopenia

Thrombocytosis

For full information, please refer to:

https://nctr-crs.fda.gov/fdalabel/services/spl/set-ids/463a36fa-3eb2-4326-8bd0-c8c7a11bca3a/spl-doc?hl=Buphenyl

Buphenylinformation