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Recombinant α-galactosidase A (ERT)

Agalsidase beta

Brand names: Fabrazyme

Adult dose

Dose: 1 mg/kg every 2 weeks
Route: IV infusion
Frequency: Fortnightly

Clinical pearls

  • Fabry disease ERT; pre-medicate per protocol; commissioned through specialist LSDU service

Contraindications

  • Life-threatening hypersensitivity

Side effects

  • Infusion reactions (fever, rigors, chest tightness)
  • Headache

Interactions

  • Avoid chloroquine/amiodarone (theoretical)

Monitoring

  • Renal function (eGFR, proteinuria)
  • Cardiac function
  • Antibodies

Reference: BNF; NHS England LSDU; SmPC Fabrazyme. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.