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Nitrogen-binding agent

Glycerol phenylbutyrate

Brand names: Ravicti

Adult dose

Dose: Per BNF/SmPC; weight-based
Route: PO
Frequency: divided

Clinical pearls

  • Chronic urea cycle disorders; specialist metabolic service

Contraindications

  • Acute hyperammonaemic crisis (use IV)
  • Hypersensitivity

Side effects

  • GI upset
  • Hyperammonaemia (if dose insufficient)
  • Hypokalaemia
  • Headache

Interactions

  • Probenecid
  • Corticosteroids (raise ammonia)
  • Valproate (raise ammonia)

Monitoring

  • Ammonia
  • U&E
  • LFTs
  • Phenylacetate/phenylbutyrate

Reference: BNF; SmPC; https://bnf.nice.org.uk/drugs/glycerol-phenylbutyrate/. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.