Xiyouyi Stiripentol for Suspension

1. Indications and Usage
Dravet Syndrome: Indicated for the treatment of seizures associated with Dravet syndrome in adult and pediatric patients aged 6 months and older weighing ≥7 kg who are on clobazam therapy.
Usage Restrictions: Safety and efficacy as monotherapy have not been established.
2. Dosage and Administration
Route of Administration: Oral administration ONLY.
Recommended Dosage: The recommended dosage is 50 mg/kg/day, administered in 2 or 3 divided doses. The maximum recommended total daily dosage is 3,000 mg.
Critical Administration Instructions:
Food: Must be administered with meals to improve absorption and reduce gastrointestinal irritation.
Administration: Shake the suspension well immediately before use. Administer the suspension immediately after mixing with water; do not store for later use.
3. Mechanism of Action
GABA Enhancement: The exact mechanism of action is unknown but may involve the enhancement of γ-aminobutyric acid (GABA)-mediated inhibitory neurotransmission.
Metabolic Inhibition: It acts as a potent inhibitor of cytochrome P450 enzymes (particularly CYP2C19 and CYP3A4), thereby increasing the plasma concentrations of co-administered drugs like clobazam and its active metabolite.
4. Safety and Warnings
Hematologic Monitoring: Neutropenia and leukopenia have been reported. Complete blood counts should be monitored regularly.
Hepatotoxicity: Liver function abnormalities may occur. Liver function tests should be performed periodically, especially when used with valproate.
CNS Depression: May cause somnolence, ataxia, and psychomotor retardation.
Behavioral Changes: Aggression, irritability, and behavioral disorders have been observed.
5. Adverse Reactions and Clinical Research
Most Common Adverse Reactions: Anorexia, weight loss, somnolence, insomnia, ataxia, psychomotor hyperactivity, and agitation.
Clinical Research Highlights: Clinical trials demonstrated that as an add-on therapy to clobazam and valproate, stiripentol significantly reduced the frequency of convulsive seizures in patients with Dravet syndrome.
6. Drug Interactions
CYP2C19/CYP3A4 Substrates: Stiripentol significantly increases the steady-state plasma concentrations of clobazam and N-desmethylclobazam. The clobazam dose may need to be reduced by 25% weekly upon stiripentol initiation.
Valproate: Concomitant use may increase the risk of gastrointestinal adverse reactions and hematologic toxicity.
CNS Depressants: Concomitant use with alcohol or other CNS depressants may potentiate the sedative effects.
7. Pharmaceutical Information
Chemical Composition: Active Ingredient: Stiripentol.
Appearance: White to pale yellow suspension.
Packaging: Single-dose bottles or sachets.
Storage: Store at controlled room temperature, protected from moisture.