LARONIDASE (la-ron'i-dase) Aldurazyme Classifications: enzyme replacement therapy; Therapeutic: enzyme replacement therapy Prototype: Pancrelipase Pregnancy Category: B |
2.9 mg/5 mL injection
Laronidase is a recombinant form of human alpha-L-iduronidase used for enzyme replacement therapy in individuals with mucopolysaccharidosis I (MPSI). This is an inherited lysosomal storage disease caused by deficiency of the enzyme alpha-L-iduronidase.
Replacement therapy for individuals lacking the enzyme alpha-L-iduronidase in mucopolysaccharidosis.
Treatment of Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I); treatment of moderate to severe Scheie form of MPS I.
Hypersensitivity to laronidase; children <5 y.
Renal or hepatic dysfunction; history of allergies, asthma; hypersensitivity to drugs, especially recombinant forms; pregnancy (category B), lactation.
Mucopolysaccharidosis Adult/Child (>5 y): IV 0.58 mg/kg infused over 34 h once/wk |
Intravenous
PREPARE: IV Infusion: Determine volume of infusion based on the patient's body weight (100 mL if ≤20 kg or 250 mL if >20 kg). Prepare IV infusion of 0.1% albumin (human) in NS injection as follows: 1) remove and discard a volume of NS injection equal to the volume of albumin (human) to be added to the IV bag (for 100 mL infusion use 2 mL of 5% albumin of 0.4 mL or 25% albumin, for 250 mL infusion use 5 mL of 5% albumin or 1 mL of 25% albumin); 2) add the appropriate volume of albumin to the IV bag and gently rotate; 3) add the albumin to IV bag and gently rotate to mix; 4) withdraw and discard a volume of the 0.1% albumin in NS injection from the IV bag equal to the volume of laronidase concentrate to be added; 5) slowly withdraw the required amount of laronidase from vials (avoid excessive agitation), then slowly add laronidase to the IV solution. Use immediately. ADMINISTER: IV Infusion: Infuse initially at 10 mcg/kg/h; may increase q15min during first h, as tolerated, to a max rate of 200 mcg/kg/hr. Maintain max for remainder of the infusion (23 h). INCOMPATIBILITIES Solution/additive: No compatibility data available. Do not recommend mixing or infusing with other drugs. |
Assessment & Drug Effects
Patient & Family Education