Agalsidase beta


Generic Medicine Info
Indications and Dosage
Intravenous
Fabry disease
Adult: As long-term enzyme replacement therapy: 1 mg/kg once every 2 weeks given via infusion at an initial rate of not more than 0.25 mg/min (15 mg/hour). For patients weighing ≥30 kg, after tolerance to the infusion is established, infusion rate may be gradually increased in increments of 0.05-0.08 mg/min (3-5 mg/hour) with each subsequent infusion; minimum infusion duration: 1.5 hours (based on individual tolerability). For patients weighing <30 kg, the Max infusion rate is 0.25 mg/min (15 mg/hour). Premedicate with antipyretics, antihistamines, and/or corticosteroids prior to infusion.
Child: ≥8 years Same as adult dose.
Special Patient Group
Rechallenge in patients with IgE antibodies or positive skin test result to agalsidase beta: 0.5 mg/kg once every 2 weeks via IV infusion at an initial Max rate of 0.01 mg/min. Dose may be gradually increased to 1 mg/kg, and infusion rate (doubled every 30 minutes up to the Max rate of 0.25 mg/min), as tolerated.
Reconstitution
Reconstitute a vial labelled as 5 mg and 35 mg with 1.1 mL and 7.2 mL of sterile water for inj (SWFI) respectively, to make a solution containing 5 mg/mL. Slowly add the SWFI down on the inside wall of the vial. Gently roll and tilt the vial; do not invert, swirl or shake. Before adding the volume of reconstituted solution required for the patient dose, remove an equal volume of 0.9% NaCl solution for inj from the infusion bag. Further dilution may be done by slowly adding the required dose of reconstituted solution directly into the 0.9% NaCl solution for inj (not in the airspace) to prepare a final concentration between 0.05-0.7 mg/mL. Gently invert or lightly massage the infusion bag to mix. Avoid vigorous shaking or agitation. Do not use filter needles during preparation.
Contraindications
Hypersensitivity.
Special Precautions
Patient with compromised cardiac function. Children. Pregnancy and lactation.
Adverse Reactions
Significant: IgG and IgE antibody development, skin test reactivity, infusion-associated reactions (e.g. chills, vomiting, paraesthesia).
Cardiac disorders: Tachycardia, bradycardia, palpitation, chest pain.
Ear and labyrinth disorders: Tinnitus, hypoacusis, vertigo.
Eye disorders: Increased lacrimation.
Gastrointestinal disorders: Nausea, abdominal pain or discomfort, diarrhoea.
General disorders and administration site conditions: Fever, fatigue, lethargy, asthenia, pain, feeling hot or cold, hyperthermia.
Infections and infestations: Fungal or viral infection.
Investigations: Increased serum creatinine.
Musculoskeletal and connective tissue disorders: Arthralgia, myalgia, back pain, muscle spasm or tightness, pain in extremity, musculoskeletal stiffness.
Nervous system disorders: Headache, dizziness, somnolence, hypoaesthesia, burning sensation.
Psychiatric disorders: Anxiety, depression.
Respiratory, thoracic and mediastinal disorders: Cough, pharyngitis, rhinitis, upper or lower respiratory tract infection, wheezing.
Skin and subcutaneous tissue disorders: Skin excoriation, generalised pruritus, erythema, maculopapular rash.
Vascular disorders: Hypertension, syncope, hot flush, pallor.
Potentially Fatal: Anaphylactic and severe hypersensitivity reactions (e.g. angioedema, bronchospasm, dyspnoea, chest discomfort, hypotension, nasal congestion, flushing, dysphagia, pruritus, rash, generalised urticaria).
Monitoring Parameters
Monitor for the development of IgG or IgE antibodies in patients with suspected allergic reactions; signs of infusion-associated reactions.
Drug Interactions
Amiodarone, monobenzone, chloroquine, or gentamicin may diminish the therapeutic effect of agalsidase beta.
Action
Description:
Mechanism of Action: Agalsidase beta is a recombinant form of α-galactosidase A, an enzyme that catalyses the hydrolysis of globotriaosylceramide (GL-3) and other α-galactyl-terminated neutral glycosphingolipids. These substrates accumulate within the tissues of patients with Fabry disease, resulting in renal and CV complications. Agalsidase beta reduces the tissue inclusions of GL-3 and restores the level of enzymatic action sufficient to clear the accumulation of substrates in organ tissues, thereby preventing and stabilising the progressive decline of organ functions.
Pharmacokinetics:
Distribution: Volume of distribution: 81-570 mL/kg.
Metabolism: Metabolised via peptide hydrolysis.
Excretion: Elimination half-life (dose-dependent): Mean range: 45-119 minutes.
Storage
Intact vial: Store between 2-8°C. Diluted solutions: Store between 2-8°C for up to 24 hours.
MIMS Class
Other Agents Affecting Metabolism
ATC Classification
A16AB04 - agalsidase beta ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
References
Anon. Agalsidase Beta. AHFS Clinical Drug Information [online]. Bethesda, MD. American Society of Health-System Pharmacists, Inc. https://www.ahfscdi.com. Accessed 01/03/2022.

Anon. Agalsidase Beta. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 01/03/2022.

Buckingham R (ed). Alpha Galactosidase A. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 01/03/2022.

Fabrazyme (Sanofi-Aventis Singapore Pte. Ltd.). MIMS Singapore. http://www.mims.com/singapore. Accessed 01/03/2022.

Fabrazyme 35 mg Powder for Concentrate for Solution for Infusion (Aventis Pharma Ltd Trading as: Sanofi Genzyme). MHRA. https://products.mhra.gov.uk. Accessed 01/03/2022.

Fabrazyme 5 mg Powder for Concentrate for Solution for Infusion (Aventis Pharma Ltd Trading as: Sanofi Genzyme). MHRA. https://products.mhra.gov.uk. Accessed 01/03/2022.

Fabrazyme Injection, Powder, Lyophilized, for Solution (Genzyme Corporation). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 01/03/2022.

Joint Formulary Committee. Agalsidase Beta. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 01/03/2022.

Disclaimer: This information is independently developed by MIMS based on Agalsidase beta from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2024 MIMS. All rights reserved. Powered by MIMS.com
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