RAVICTI (Glycerol Phenylbutyrate) – Reduce Blood Ammonia Levels | DengYue

Glycerol Phenylbutyrate Application Scope

• Used for the chronic management of urea cycle disorders (UCDs) in patients who cannot be controlled by dietary protein restriction and/or amino acid supplementation alone.
• Helps reduce blood ammonia levels by providing an alternative pathway for nitrogen excretion.

Glycerol Phenylbutyrate Characteristics

Ingredients:

• Active ingredient: Glycerol phenylbutyrate (1,2,3-Propanetriyl tris(4-phenylbutanoate).
• Contains 1.1 g/mL of glycerol phenylbutyrate, delivering 1.02 g/mL of phenylbutyrate.

Properties:

• Tasteless and odorless liquid formulation.
• No sodium content, unlike sodium phenylbutyrate alternatives.
• Slow-release mechanism, providing stable ammonia control over 24 hours.

Specification:

• Oral liquid (1.1 g/mL).

Packaging Specification:

• Typically supplied in bottles with an oral syringe or dosing cup for accurate measurement.

Storage:

• Store at room temperature (away from moisture, heat, and direct light).
• Do not freeze.

Expiry Date:

• Refer to the manufacturer’s labeling (not explicitly mentioned in search results).

Executive Standard:

• Approved by FDA (U.S.), EMA (Europe), and other regulatory bodies.

Approval Number:

• FDA NDA #203284.

Date of Revision:

• Last updated in prescribing information as of 2024.

Manufacturer:

• Originally developed by Hyperion Therapeutics, now marketed by various pharmaceutical companies globally.

Guidelines for the Use of Glycerol Phenylbutyrate

Dosage and Administration:

• Adults & children ≥2 years:
  • Initial dose: 4.5–11.2 mL/m²/day, divided into 3 equal doses (rounded to nearest 0.5 mL).
  • Max dose: 17.5 mL/day.
• Infants <2 years:
  • Similar dosing but adjusted for body size, rounded to nearest 0.1 mL.
• Switching from sodium phenylbutyrate:
  • Powder form: Multiply dose by 0.81.
  • Tablet form: Multiply dose by 0.86.
• Administration:
  • Take with food or infant formula.
  • Can be given via nasogastric or gastrostomy tube (flush with water after).

Adverse Reactions:

• Common:
  • Gastrointestinal issues (diarrhea, vomiting, stomach pain).
  • Headache, fatigue, rash.
• Serious (require medical attention):
  • Hyperammonemia symptoms (confusion, seizures, lethargy).
  • Neurological effects (numbness, tingling, hearing loss).

Contraindications:

• Hypersensitivity to glycerol phenylbutyrate.
• Infants <2 months old (unless under strict medical supervision due to immature pancreatic enzymes).

Precautions:

• Monitor ammonia levels regularly.
• Use caution in liver/kidney disease (may increase drug exposure).
• Avoid abrupt discontinuation (risk of hyperammonemia).

Glycerol Phenylbutyrate Interactions

Drug Interactions:

• Valproic acid, corticosteroids, and haloperidol (may increase ammonia levels).
• Probenecid (may affect excretion of phenylacetylglutamine).

Generic Name / Brand Name:

• Generic: Glycerol phenylbutyrate
• Brand: Ravicti®.

Indications:

• Chronic management of UCDs (e.g., OTC, CPS1 deficiency).

Dosage Form:

• Oral liquid (1.1 g/mL).

Specification:

• 1.1 g/mL glycerol phenylbutyrate.

Note:

• If there is a new packaging for the drug, the new packaging shall prevail. The above information is sourced from DengYue Medicine.
• It is only for internal discussion among medical staff and does not serve as a basis for medication. For specific medication guidelines, please consult the attending physician.