Trade Names:Aldurazyme- Injection solution, concentrate laronidase 2.9 mg/5 mL
Provides exogenous enzyme for uptake into lysosomes and increases the catabolism of glycosaminoglycans.
Mean AUC is 4.5 to 6.9 mcg•h/mL. Mean plasma C max ranges from 1.2 to 1.7 mcg/mL.
Mean Vd is 0.24 to 0.6 L/kg.
Mean plasma Cl is 1.7 to 2.7 mL/min/kg and elimination half-life is 1.5 to 3.6 h.
Treatment of patients with Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I); patients with Scheie form who have moderate to severe symptoms.
IV 0.58 mg/kg once/wk delivered over 3 to 4 h.
Store vials of concentrated solution in refrigerator (36° to 46°F). Do not freeze or shake. Use diluted infusion solution immediately. If immediate use is not possible, store the diluted infusion solution in refrigerator (36° to 46°F). Do not store diluted infusion solution longer than 36 h from time of preparation to completion of infusion.
None well documented.
Do not mix with other medicinal products in the same infusion.
None well documented.
Vein disorder (14%); dependent edema, hypotension (9%); increased blood pressure, tachycardia (postmarketing).
Hyperreflexia, paresthesia (14%).
Corneal opacity (9%).
Diarrhea, nausea, vomiting (postmarketing).
Injection-site reaction (18%); injection-site pain (9%).
Upper respiratory tract infection (32%); decreased oxygen saturation (postmarketing).
Antibodies to laronidase (91%); infusion-related reactions, including fever, flushing, headache, and rash (32%); abscess, chest pain, facial edema (9%); abdominal pain, chills (postmarketing).
Anaphylaxis: Life-threatening anaphylactic reactions have occurred during laronidase infusions. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise because of infusion reactions and require additional monitoring. Appropriate medical support should be readily available.
Category B .
Safety and efficacy not studied in children younger than 5 yr of age.
Infusion-related hypersensitivity reactions may occur. Because of increased prevalence of coronary artery disease in patients with MPS I, use epinephrine with caution. Prior to infusion, patients should receive antipyretics and/or antihistamines. If an infusion reaction occurs, decreasing the infusion rate, temporarily stopping the infusion, and/or administering additional antipyretics and/or antihistamines may ameliorate the symptoms.
No experience with overdosage.
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