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Pronunciation: lar-ON-i-dase
Class: Endocrine and metabolic agent

Trade Names

- Injection solution, concentrate laronidase 2.9 mg/5 mL


Provides exogenous enzyme for uptake into lysosomes and increases the catabolism of glycosaminoglycans.

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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.

Indications and Usage

Treatment of patients with Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I); patients with Scheie form who have moderate to severe symptoms.


Standard considerations.

Dosage and Administration

Adults and Children 5 yr of age and older

IV 0.58 mg/kg once/wk delivered over 3 to 4 h.

General Advice

  • For IV administration only. Not for intradermal, IM, subcutaneous, or intra-arterial administration.
  • Follow manufacturer's instructions for diluting the concentrated solution for infusion.
  • Prepare infusion solution using polyvinyl chloride (PVC) containers and administer with a PVC infusion set equipped with an in-line, low protein-binding 0.2 mcm filter.
  • Do not administer if solution is cloudy, discolored, or contains particulate matter.
  • Do not shake or agitate vials during dilution. Do not use filter needles during preparation of infusion.
  • Discard any unused product. Vials are for single-use only. Do not save medication for future use.
  • Initial IV infusion rate should be no more than 10 mcg/kg/h and may be incrementally increased every 15 min during the first hour, as tolerated, until a max infusion rate of 200 mcg/kg/min is reached. The max rate is then maintained for remainder of the infusion.
  • Do not administer in same IV line with other products.
  • Patients with a body weight of 20 kg or less should receive a total volume of 100 mL.
  • Patients with a body weight more than 20 kg should receive a total volume of 250 mL.


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.

Drug Interactions

None well documented.


Do not mix with other medicinal products in the same infusion.

Laboratory Test Interactions

None well documented.

Adverse Reactions


Vein disorder (14%); dependent edema, hypotension (9%); increased blood pressure, tachycardia (postmarketing).


Hyperreflexia, paresthesia (14%).


Rash (36%).


Corneal opacity (9%).


Diarrhea, nausea, vomiting (postmarketing).


Bilirubinemia (9%).


Thrombocytopenia (9%).


Injection-site reaction (18%); injection-site pain (9%).


Arthralgia (postmarketing).


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.

Hypersensitivity/Infusion reactions

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.

Patient Information

  • Advise patient, family, or caregiver that medication will be prepared and administered by a health care provider in a health care setting.
  • Advise patient, family, or caregiver that an antipyretic (eg, acetaminophen) and antihistamine (eg, diphenhydramine) will be administered before each treatment to prevent or reduce the severity of infusion reactions.
  • Advise patient, family, or caregiver to report any signs or symptoms of infusion reaction (eg, fever, flushing, headache, rash).
  • Encourage patient to enroll and participate in the MPS I registry, as noted in the manufacturer's product information.

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