Alglucosidase alfa

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What is Alglucosidase alfa?[edit | edit source]


What are the uses of this medicine?[edit | edit source]

  • LUMIZYME (alglucosidase alfa) used for the treatment of patients with Pompe disease (acid α-glucosidase [GAA] deficiency).

How does this medicine work?[edit | edit source]

  • Pompe disease (acid maltase deficiency, glycogen storage disease type II, GSD II, glycogenosis type II) is an inherited disorder of glycogen metabolism caused by the absence or marked deficiency of the lysosomal enzyme GAA.
  • Alglucosidase alfa provides an exogenous source of GAA.
  • Binding to mannose-6-phosphate receptors on the cell surface has been shown to occur via carbohydrate groups on the GAA molecule, after which it is internalized and transported into lysosomes, where it undergoes proteolytic cleavage that results in increased enzymatic activity.
  • It then exerts enzymatic activity in cleaving glycogen.

Who Should Not Use this medicine ?[edit | edit source]

  • This medicine have no usage limitations.

What drug interactions can this medicine cause?[edit | edit source]

  • No formal drug interaction studies have been conducted with LUMIZYME.

Is this medicine FDA approved?[edit | edit source]

  • It was approved for medical use in the United States in May 2010, as Lumizyme

How should this medicine be used?[edit | edit source]

Recommended dosage:

  • The recommended dosage of alglucosidase alfa is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion.

Administration:

  • Alglucosidase alfa should be reconstituted, diluted, and administered by a healthcare professional.
  • Use aseptic technique during preparation. Do not use filter needles during preparation.
  • Add the reconstituted alglucosidase alfa solution slowly and directly into the sodium chloride solution. Do not add directly into airspace that may remain within the infusion bag. Avoid foaming in the infusion bag.
  • Gently invert or massage the infusion bag to mix. Do not shake.
  • Administer alglucosidase alfa using an in-line low protein binding 0.2 µm filter.
  • Do not infuse alglucosidase alfa in the same intravenous line with other products.

What are the dosage forms and brand names of this medicine?[edit | edit source]

This medicine is available in fallowing doasage form:

  • As injection: 50 mg of alglucosidase alfa as lyophilized powder in a single-dose vial for reconstitution.

This medicine is available in fallowing brand namesː

  • LUMIZYME

What side effects can this medication cause?[edit | edit source]

The most common side effects of this medicine include:

What special precautions should I follow?[edit | edit source]

  • Life-threatening anaphylaxis and hypersensitivity reactions have been observed in some patients during and after treatment with alglucosidase alfa. Ensure that appropriate medical support measures, including cardiopulmonary resuscitation equipment, are readily available. If anaphylaxis or severe hypersensitivity reactions occur, immediately discontinue infusion and initiate appropriate medical treatment.
  • Immune-mediated cutaneous reactions have been reported with alglucosidase alfa including necrotizing skin lesions. Monitor patients for the development of systemic immune-mediated reactions involving skin and other organs.
  • Patients with compromised cardiac or respiratory function may be at risk of acute cardiorespiratory failure. Caution should be exercised when administering alglucosidase alfa to patients susceptible to fluid volume overload. Appropriate medical support and monitoring measures should be available during infusion.
  • Administration of general anesthesia can be complicated by the presence of severe cardiac and skeletal (including respiratory) muscle weakness. Caution should be used when administering general anesthesia for the placement of a central venous catheter intended for alglucosidase alfa infusion.
  • Patients with infantile-onset Pompe disease should have a cross-reactive immunologic material (CRIM) assessment early in their disease course and be managed by a clinical specialist knowledgeable in immune tolerance induction in Pompe disease to optimize treatment.
  • Patients should be monitored for IgG antibody formation every 3 months for 2 years and then annually thereafter.


What to do in case of emergency/overdose?[edit | edit source]

  • In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

Can this medicine be used in pregnancy?[edit | edit source]

  • Data from postmarketing reports and published case reports with alglucosidase alfa use in pregnant women have not identified a LUMIZYME-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes.

Can this medicine be used in children?[edit | edit source]

  • The safety and effectiveness of alglucosidase alfa have been established in pediatric patients with Pompe disease.

What are the active and inactive ingredients in this medicine?[edit | edit source]

Active ingredients: ALGLUCOSIDASE ALFA

Inactive ingredients: MANNITOL SODIUM PHOSPHATE, MONOBASIC, MONOHYDRATE SODIUM PHOSPHATE, DIBASIC, HEPTAHYDRATE POLYSORBATE 80

Who manufactures and distributes this medicine?[edit | edit source]

  • LUMIZYME is manufactured and distributed by:
  • Genzyme Corporation
  • Cambridge, MA

What should I know about storage and disposal of this medication?[edit | edit source]

  • Store LUMIZYME under refrigeration between 2°C and 8°C (36°F and 46°F).
  • Do not use LUMIZYME after the expiration date on the vial.
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