About phenylbutyrate sodium
Nitrogen-scavenging agent
Used for
- Long-term management of urea-cycle disorders involving carbamoyl phosphate synthetase, ornithine transcarbamylase, or argininosuccinate synthetase deficiency.
- Used with dietary protein restriction and other treatments to reduce ammonia buildup.
Usual adult dose
Usually 9.9–13.0 g/m² daily, divided into three to six doses; dosing is individualized by a specialist.
Usual dose in children
Usually 450–600 mg/kg daily for children weighing under 20 kg; for larger children, dosing is based on body surface area. Divide into three to six doses; specialist-directed.
Common side effects
- Poor appetite, nausea, vomiting, or abdominal pain
- Unpleasant taste or body odor
- Changes in blood counts or electrolyte levels
- Menstrual changes or reduced appetite and weight
Important warnings
- Requires specialist monitoring of blood ammonia, electrolytes, blood counts, and nutritional status.
- Not suitable as the only treatment during acute hyperammonemia.
- Use caution with liver or kidney impairment.
- Compounded powder must be measured and prepared according to pharmacy instructions.
Pregnancy
Use only when clearly needed; pregnancy data are limited, and uncontrolled urea-cycle disease also poses serious risks.
Breastfeeding
It is not known whether phenylbutyrate enters breast milk; use requires individualized assessment.
Interactions
- Probenecid may affect removal of phenylbutyrate metabolites.
- Corticosteroids, valproate, or haloperidol may increase ammonia levels.
AI-generated from general medical knowledge, not from this product's official leaflet. It may be incomplete or out of date. Not medical advice — ask a pharmacist or doctor before making any decision.