Finished product reference
Agalsidase Beta
Injection, Powder, for Solution · Parenteral · 35 mg
- List code
- 52523
- Pharmacologic class
- Hydrolytic Lysosomal Neutral Glycosphingolipid-specific Enzyme
WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy.
What it is and how it works
Mechanism of Action FABRAZYME (agalsidase beta) provides an exogenous source of α-galactosidase A in Fabry disease patients. Agalsidase beta is internalized and transported into lysosomes where it exerts enzymatic activity and reduces accumulated GL-3.
Indications
® is indicated for the treatment of adult and pediatric patients 2 years of age and older with confirmed Fabry disease. FABRAZYME is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme indicated for the treatment of adult and pediatric patients 2 years of age and older with confirmed Fabry disease.
Dosage and administration (summary)
Administration of FABRAZYME should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. The recommended dosage is 1 mg/kg body weight given every two weeks as an intravenous infusion. See the full prescribing information for rechallenge, preparation, storage, and administration instructions. ( 2.3 , 2.4 , 2.5 , 2.6 ) Recommendations Prior to FABRAZYME Treatment Administration of FABRAZYME should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis .
Contraindications
None. None.
Warnings and precautions
Infusion-Associated Reactions : If a severe infusion-associated reaction occurs, discontinue FABRAZYME immediately and initiate appropriate medical treatment. Hypersensitivity Reactions Including Anaphylaxis Life-threatening hypersensitivity reactions, including anaphylaxis, have been reported in FABRAZYME-treated patients. In clinical trials and postmarketing safety experience with FABRAZYME, approximately 1% of patients developed anaphylaxis or severe hypersensitivity reactions.
Pregnancy
Pregnancy Risk Summary Available data from a pregnancy sub-study within the Fabry Disease registry, post-marketing case reports, and case series with FABRAZYME use during pregnancy have not identified a drug-associated risk of major birth defects, miscarriage or other adverse maternal or fetal outcomes (see Data ).
Storage and supply
Refrigerate vials of FABRAZYME at 2°C to 8°C (36°F to 46°F). Do not use FABRAZYME after the expiration date on the vial. This product contains no preservatives. Reconstituted and diluted solutions of FABRAZYME should be used immediately.
Source: Agalsidase Beta (Fabrazyme) — US FDA prescribing information, 2025-08-22, DailyMed. Summarised and shortened; the full label governs. Iranian registration, indications and labelling may differ.
Reference information for professional sourcing and registration enquiries, not medical advice or a promotion. Drug information is condensed from US FDA prescribing information (public domain, via openFDA and DailyMed) and does not replace the product’s approved labelling in Iran. Availability, origin and documentation are confirmed per enquiry.
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