Agalsidase alfa


Generic Medicine Info
Indications and Dosage
Intravenous
Fabry disease
Adult: As long-term enzyme replacement therapy: 0.2 mg/kg every other week via infusion over 40 minutes using an IV line with integral filter. Premedicate with antihistamines and/or corticosteroids 1-24 hours before infusion if required. If mild or moderate acute infusion reactions occur, infusion may be temporarily interrupted (5-10 minutes) until symptoms subside.
Child: ≥7 years Same as adult dose.
Reconstitution
Prior to infusion, dilute the required total volume in 100 mL 0.9% NaCl solution for infusion. Gently mix the solution. Do not shake.
Contraindications
Hypersensitivity.
Special Precautions
Patient with compromised cardiac function due to Fabry disease. Renal impairment (eGFR <60 mL/min). Children. Pregnancy and lactation.
Adverse Reactions
Significant: Idiosyncratic infusion-related reactions (e.g. headache, nausea, rigors, pyrexia, flushing, fatigue), hypersensitivity reactions (including anaphylactic reactions), IgG antibody development.
Cardiac disorders: Palpitations, atrial fibrillation, tachycardia, chest pain or tightness.
Ear and labyrinth disorders: New-onset or aggravated tinnitus.
Eye disorders: Lacrimation increased, corneal reflex decreased.
Gastrointestinal disorders: Vomiting, abdominal pain or discomfort, diarrhoea, dysgeusia.
General disorders and administration site conditions: Peripheral oedema, asthenia, pain or discomfort, aggravated fatigue, feeling hot or cold, influenza-like illness, malaise, inj site rash.
Musculoskeletal and connective tissue disorders: Arthralgia, myalgia, back pain, pain in limb, joint swelling, musculoskeletal discomfort, peripheral swelling.
Nervous system disorders: Dizziness, neuropathic pain, tremor, paraesthesia, hypoaesthesia.
Psychiatric disorders: Hypersomnia.
Respiratory, thoracic and mediastinal disorders: Cough, dyspnoea, nasopharyngitis, pharyngitis, throat tightness, rhinorrhoea, hoarseness.
Skin and subcutaneous tissue disorders: Rash, acne, urticaria, erythema, pruritus, hyperhidrosis, livedo reticularis.
Vascular disorders: Hypertension, hypotension.
Drug Interactions
Amiodarone, monobenzone, chloroquine, or gentamicin may potentially inhibit intra-cellular α-galactosidase activity; avoid concomitant use.
Action
Description: Agalsidase alfa is a recombinant form of α-galactosidase-A. It catalyses the hydrolysis of globotriaosylceramide (Gb3), the glycosphingolipid substrate that accumulates within the tissues of patients with Fabry disease, thereby cleaving the terminal galactose residue from the molecule. This action reduces the accumulation of Gb3 in many cell types including endothelial and parenchymal cells, resulting in stabilisation of or improvement in renal function and structure, reduction in pain, and decrease in cardiac mass with an associated improvement in cardiac function.
Pharmacokinetics:
Distribution: Volume of distribution: Male: 203 mL/kg (range: 89-6,778 mL/kg).
Metabolism: Metabolised in the plasma via peptide hydrolysis.
Excretion: Elimination half-life: Male: 54.7 minutes (range: 28.5-654.2 minutes).
Storage
Store between 2-8°C. Diluted solutions must be administered immediately; if not used immediately, diluted solutions are stable for 24 hours between 2-8°C.
ATC Classification
A16AB03 - agalsidase alfa ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
References
Anon. Agalsidase Alfa. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 09/08/2021.

Buckingham R (ed). Alpha Galactosidase A. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 09/08/2021.

Joint Formulary Committee. Agalsidase Alfa. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 09/08/2021.

Replagal 1 mg/mL Concentrate for Solution for Infusion (Shire Human Genetic Therapies AB). MHRA. https://products.mhra.gov.uk. Accessed 09/08/2021.

Replagal Concentrate for Solution for Infusion 3.5 mg/3.5 mL (Takeda Pharmaceuticals [Hong Kong] Ltd). MIMS Hong Kong. http://www.mims.com/hongkong. Accessed 09/08/2021.

Takeda New Zealand Limited. Replagal 3.5 mg/3.5 mL Concentrate for Infusion Vial data sheet 28 September 2020. Medsafe. http://www.medsafe.govt.nz. Accessed 09/08/2021.

Disclaimer: This information is independently developed by MIMS based on Agalsidase alfa 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 © 2021 MIMS. All rights reserved. Powered by MIMS.com
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