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Aldurazyme(Laronidase)

Acetylcysteine Injection, FLUIMUCIL, Acetylcysteine InjectionAcetylcysteine Injection, FLUIMUCIL, Acetylcysteine InjectionAcetylcysteine Injection, FLUIMUCIL, Acetylcysteine Injection

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INJECTION

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Reminder: The outer packaging is for reference only, please purchase and use under the guidance of a pharmacist. For read by medical and pharmaceutical professionals only.

ALDURAZYME® (laronidase) is a recombinant enzyme replacement therapy used for treating mucopolysaccharidosis type I (MPS I), a rare genetic disorder caused by the deficiency of the enzyme α-L-iduronidase. This condition leads to the accumulation of glycosaminoglycans (GAGs) in various organs and tissues, causing progressive damage. ALDURAZYME® helps to replace the deficient enzyme and prevent the buildup of GAGs, thereby improving symptoms and quality of life in patients, particularly those with the severe Hurler syndrome and mixed Hurler-Scheie syndrome.

The drug is administered intravenously once a week, with the dose tailored to the individual patient’s needs. While it provides significant clinical benefits, careful monitoring is required due to potential infusion reactions and the need for dose adjustments based on patient response.

Generic name

Aldurazyme(Laronidase)

English name

Laronidase

Alternative Names

Aldurazyme

Drug prices

Indications

ALDURAZYME® is indicated for:

Adult and pediatric patients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I).

Patients with the Scheie form of MPS I who exhibit moderate to severe symptoms.

Limitations:

Safety/efficacy in mild Scheie form patients is unestablished.

No demonstrated effect on central nervous system (CNS) manifestations of MPS I

Therapeutic Target

Mucopolysaccharidosis I (MPS I): A lysosomal storage disorder caused by α-L-iduronidase deficiency, leading to accumulation of glycosaminoglycans (GAGs: dermatan sulfate and heparan sulfate) in tissues.

Active Ingredients

Laronidase: Recombinant human α-L-iduronidase (specific activity: ~172 U/mg).

Dosage form

INJECTION

specifications

Single-dose vial: 2.9 mg/5 mL (0.58 mg/mL) laronidase.

Description

Molecular Weight: ~83 kD glycoprotein with 628 amino acids.

Formulation: Contains sodium chloride, sodium phosphate buffers, and polysorbate 80. pH ~5.5.

Storage: Refrigerate at 2–8°C (36–46°F). Do not freeze or shake. Protect from light.

Dosage and Administration

Recommended Dosage

0.58 mg/kg actual body weight administered once weekly via IV infusion.

Preparation

Diluent: 0.9% Sodium Chloride Injection.

Infusion Volume:

≥2 to <4 kg: 50 mL

≥4 to ≤20 kg: 100 mL

20 kg: 250 mL

Infusion Rate:

Start at 10 mcg/kg/hr, escalate every 15 minutes to max 200 mcg/kg/hr (Table 1).

Total infusion time: 3–4 hours.

Administration Modifications

Severe hypersensitivity/IAR: Discontinue infusion, initiate emergency care.

Mild/moderate reactions: Temporarily hold infusion or reduce rate by 25–50%.

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