1. Indications and Usage
\nFabry Disease:
\nIndicated for long-term enzyme replacement therapy in patients with confirmed Fabry disease (alpha-galactosidase A deficiency) to clear globotriaosylceramide (GL-3) from vascular endothelial cells and other cell types.
\n2. Dosage and Administration
\nRoute of Administration:
\nFor Intravenous (IV) Infusion ONLY. Must be administered by a healthcare professional.
\nRecommended Dosage:
\nThe recommended dose is 0.2 mg\/kg body weight administered every other week.
\nAdministration Instructions:
\nPreparation: The concentrated solution must be diluted with 0.9% Sodium Chloride Injection, USP prior to administration. Do not mix with other medications in the same infusion line.
\nInfusion Rate: Administer as an IV infusion over approximately 40 minutes.
\nPremedication: Antipyretics or antihistamines may be considered prior to infusion to reduce the risk of hypersensitivity reactions.
\nMissed Dose: If a dose is missed, administer as soon as possible. Subsequent doses should be scheduled at 2-week intervals from the date of the last administered dose.
\n3. Mechanism of Action
\nEnzyme Replacement Therapy:
\nAgalsidase alfa is a purified form of the lysosomal enzyme alpha-galactosidase A. In patients with Fabry disease, this enzyme is deficient, leading to the accumulation of glycosphingolipids, primarily globotriaosylceramide (GL-3), in various tissues.
\nSubstrate Clearance:
\nExogenously administered agalsidase alfa is taken up into cellular lysosomes via mannose receptors, where it catalyzes the hydrolysis of GL-3 into lactosylceramide and galactose, thereby reducing GL-3 storage.
\n4. Safety and Warnings
\nHypersensitivity Reactions (Including Anaphylaxis):
\nSevere hypersensitivity reactions, including anaphylaxis, have been reported during or shortly after infusion. Patients should be closely monitored during and after administration. Appropriate medical support must be readily available.
\nAntibody Formation:
\nPatients may develop IgG antibodies against agalsidase alfa. Antibody formation may be associated with hypersensitivity reactions and potentially reduced clinical efficacy.
\nAcute Renal Failure:
\nCases of acute renal failure have been reported in patients receiving agalsidase alfa. Monitor renal function periodically.
\nInfusion-Associated Reactions:
\nFever, chills, nausea, vomiting, headache, and fatigue are common during infusion. Slowing the infusion rate or temporarily stopping the infusion may alleviate symptoms.
\n5. Adverse Reactions
\nMost Common:
\nChills, fever, headache, nausea, vomiting, fatigue, paresthesia, and pruritus. These events typically occur during or within hours of the infusion.
\nOther Reported Reactions:
\nHypersensitivity reactions (rash, urticaria, dyspnea, hypotension, angioedema), elevated liver enzymes, and injection site reactions.
\n6. Drug Interactions
\nNo formal drug-drug interaction studies have been conducted with agalsidase alfa. Because it is an enzyme replacement therapy, it is not expected to interact with drugs metabolized by hepatic cytochrome P450 enzymes. However, caution is advised when co-administering with nephrotoxic agents due to the underlying risk of renal impairment in Fabry disease.
\n7. Pharmaceutical Information
\nChemical Composition:
\nActive Ingredient: Agalsidase alfa (recombinant human alpha-galactosidase A produced in human fibroblast cells).
\nAvailable Strengths: Vials containing 3.5 mg\/3.5 mL (1 mg\/mL) of agalsidase alfa.
\nExcipients: Polysorbate 20, sodium chloride, sodium dihydrogen phosphate monohydrate, disodium hydrogen phosphate dihydrate, and water for injections.
\nStorage:
\nStore in a refrigerator at 2\u00b0C to 8\u00b0C (36\u00b0F to 46\u00b0F) in the original carton to protect from light. Do not freeze. Do not shake.<\/p>\n","protected":false},"excerpt":{"rendered":"
Brand Name: \u745e\u666e\u4f73\u00ae(Replagal\u00ae)
\nGeneric Name: Agalsidase Alfa
\nStrength: 3.5mg\/3.5mL per vial
\nManufacturer: Vetter Pharma-Fertigung GmbH & Co.KG
\nMarketing Authorization Holder: Shire Human Genetic Therapies AB
\nApproval Date in China: 2018
\nRegistration Number: \u56fd\u836f\u51c6\u5b57 HJ20180029
\nStorage: Store at 2\u2103\uff5e8\u2103, avoid freezing and direct sunlight; keep out of reach of children.<\/p>\n
Price&Cost:Contact Us now for the best price of Agalsidase Alfa \uff08Replagal\uff09<\/p>\n","protected":false},"featured_media":3549,"comment_status":"open","ping_status":"closed","template":"","meta":[],"product_brand":[],"product_cat":[25],"product_tag":[],"class_list":["post-3548","product","type-product","status-publish","has-post-thumbnail","product_cat-injection","first","instock","shipping-taxable","product-type-simple"],"yoast_head":"\n