Classifications: enzyme replacement therapy; Therapeutic: enzyme replacement therapy
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.
Route & Dosage
Adult/Child (>5 y): IV 0.58 mg/kg infused over 34 h once/wk
- Pretreatment with antipyretics and/or antihistamines 60 min prior to infusion is recommended.
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.
- Store at 2°8° C (36°46° F). Do not freeze or shake. Discard any unused drug.
Adverse Effects (≥1%)Body as a Whole:
Infusion reactions (flushing, fever, headache, rash), injection site pain, hypersensitivity reactions. CNS:
Hyperreflexia, paresthesias. CV:
Chest pain, hypotension, edema. Hematologic: Thrombocytopenia
Upper respiratory tract infection
Assessment & Drug Effects
- Monitor for infusion-related reactions. Slow or stop infusion and notify physician for any of the following: cough, bronchospasm,
dyspnea, urticaria, angioedema, pruritus, or other signs of hypersensitivity.
- Lab tests: Periodic platelet count.
Patient & Family Education
- Report promptly difficulty breathing, rash, or itching.