Alglucosidase Alfa Application Scope

Alglucosidase alfa is an enzyme replacement therapy used for treating Pompe disease, a rare genetic disorder characterized by the buildup of glycogen in cells due to the deficiency of the enzyme acid alpha-glucosidase.

Alglucosidase Alfa Characteristics

Ingredients: Each vial contains 50 mg of alglucosidase alfa, along with inactive ingredients such as mannitol, polysorbate 80, sodium phosphate dibasic heptahydrate, and sodium phosphate monobasic monohydrate.
Properties: A recombinant form of the human enzyme acid alpha-glucosidase. It binds to mannose-6-phosphate receptors on the cell surface, is internalized, and transported to lysosomes, leading to increased enzymatic activity that helps break down glycogen.
Specification: Supplied as a sterile, nonpyrogenic, white to off-white lyophilized powder for reconstitution. Each single-use vial contains 50 mg of alglucosidase alfa.
Packaging Specification: Available in single-use vials containing 50 mg of lyophilized powder.
Storage: Store vials under refrigeration at 2°C to 8°C (36°F to 46°F). Do not freeze or shake.
Expiry Date: Refer to the expiration date printed on the vial label.
Executive Standard: Approved by the U.S. Food and Drug Administration (FDA).
Approval Number: Biologics License Application (BLA) #125141.
Date of Revision: Refer to the date on the product’s prescribing information.
Manufacturer: Sanofi Genzyme.

Guidelines for the Use of Alglucosidase Alfa

Dosage and Administration: The recommended dosage is 20 mg/kg of body weight administered every two weeks as an intravenous infusion. The infusion should begin at a low rate and may be increased gradually based on patient tolerance.
Adverse Reactions: Common adverse reactions include infusion-associated reactions such as fever, chills, rash, and respiratory symptoms. Serious allergic reactions, including anaphylaxis, have been reported.
Contraindications: There are no known contraindications for alglucosidase alfa.
Precautions: Patients should be monitored closely for infusion-associated reactions. Pre-medication with antihistamines, antipyretics, and/or corticosteroids may be considered to reduce the risk of such reactions.

Interactions

Drug Interactions: No formal drug interaction studies have been conducted with alglucosidase alfa. However, caution should be exercised when administering other medications concomitantly, and patients should be monitored for potential interactions.

Note:

If there is a new packaging for the drug, the new packaging shall prevail. The above information is sourced from DengYue Medicine.
It is only for internal discussion among medical staff and does not serve as a basis for medication. For specific medication guidelines, please consult the attending physician.