(la-ron-i-dase) ,


(trade name)


Therapeutic: replacement enzyme
Pharmacologic: enzymes
Pregnancy Category: B


Mucopolysaccharidosis 1 (MPS 1; specifically Hurler and Hurler-Scheie form or Scheie form) with moderate to severe symptoms.


Replaces the naturally occurring enzyme α-L-iduronidase which is deficient in MPS 1. Without replacement, the glucosaminoglycans dermatan and heparan accumulate in tissues.

Therapeutic effects

Decreased cellular, tissue and organ damage due to mucopolysaccharide accumulation resulting in improved pulmonary function and walking capacity.


Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: Unknown.
Half-life: 1.5–3.6 hr.

Time/action profile

IVunknownunknown1 wk


Contraindicated in: None known.
Use Cautiously in: Obstetric: Use during pregnancy only if clearly needed; Lactation: Safety not established.

Adverse Reactions/Side Effects

Central nervous system

  • fatigue


  • edema


  • respiratory tract infections


  • rash


  • injection site reactions


  • allergic reactions including anaphylaxis (life-threatening)
  • infusion related reactions


Drug-Drug interaction

None known.


Intravenous (Adults and Children) 0.58 mg/kg once weekly, pre-treat with antipyretics and/or antihistamines.


Solution for injection: 0.58 mg/mL

Nursing implications

Nursing assessment

  • Assess vital signs every 15 min during the first hr of the infusion and frequently during remainder of infusion.
  • Monitor for signs of anaphylaxis (dyspnea, rash, urticaria) during and for up to 3 hrs following infusion. May be treated by slowing infusion rate, additional antipyretics and antihistamines. If severe reaction occurs, discontinue infusion and institute treatment. Use caution when considering epinephrine; these patients have an increased prevalence of coronary artery disease.
  • Monitor for infusion-related reaction (flushing, fever, headache, rash). Treat by slowing infusion rate, temporarily stopping infusion, and/or administering antipyretics and antihistamines.

Potential Nursing Diagnoses

Impaired gas exchange (Indications)


  • Administer antipyretics and antihistamines 60 min prior to infusion to prevent hypersensitivity reactions.
  • Intravenous Administration
  • Intermittent Infusion: Diluent: Dilute concentrated solution with 0.1% albumin and 0.9% NaCl using PVC containers. Determine number of vials needed based on patient weight and round to nearest whole vial. Remove required number of vials from refrigerator and allow to reach room temperature; do not heat or microwave vials. Solution in vials should be slightly opalescent and colorless to pale yellow and may contain a few translucent particles; do not use if discolored or contain particulate matter. Refrigerate, do not freeze. Laronidase administration must be completed within 36 hr of preparation. Do not use after expiration date.
    • Determine total volume of infusion based on weight. Final volume should be 100 mL if patient weighs ≤20 kg and 250 mL if patient weighs >20 kg.
    • To prepare infusion, remove and discard amount of 0.9% NaCl from infusion bag equal to amount of albumin to be added. If total volume is 100 mL, add 2 mL of albumin 5% or 0.4 mL of albumin 25%; if total volume is 250 mL, add 5 mL of albumin 5% or 1 mL of albumin 25%. Gently rotate infusion bag to ensure distribution of albumin.
    • Withdraw and discard a volume of 0.1% albumin in 0.9% NaCl equal to the volume of laronidase concentrate to be added. Slowly withdraw volume of laronidase from vial; avoid excessive agitation. Do not use a filter needle; may cause agitation and denature laronidase rendering it biologically inactive. Slowly add laronidase to 0.1% albumin in 0.9% NaCl solution. Gently rotate infusion bag to ensure distribution; do not shake.
  • Rate: Administer with a low-protein-binding infusion set equipped with an in-line, low-protein-binding 0.2 micrometer filter over 3–4 hr. Initial infusion rate of 10 mcg/kg/hr can be increased every 15 min during first hr, as tolerated, until a maximum rate of 200 mcg/kg/hr is reached; then maintain maximum infusion rate for remainder of infusion (2–3 hr).
  • Additive Incompatibility: Do not mix with other medications.

Patient/Family Teaching

  • Explain MPS I registry to patient and encourage participation. Purpose is to better understand the variability and progression of MPS I disease and to continue to monitor and evaluate treatments. Advise patients that participation may involve long-term follow-up. Information regarding the registry program can be found at or by calling (800) 745-4447.
  • Advise patient of risk of anaphylaxis. Caution patient to inform health care professional immediately if side effects occur.
  • Advise female patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

Evaluation/Desired Outcomes

  • Improvement in pulmonary function and walking capacity. Full benefits may not be evident for several months to several years.
Drug Guide, © 2015 Farlex and Partners


A recombinant DNA enzyme used as replacement therapy in Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I).
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
Mentioned in ?
References in periodicals archive ?
There are potential infusion related adverse events(pyrexia, chills, hypertension,tachycardia, cutaneous reactions, headache, nausea, fatigue, malaise, joint pain, dyspnea, facial edema, dizziness and bronchospasm) for all seven FDA approved ERT products and the black box warnings (life-threatening anaphylactic reactions) for four FDA approved ERT products alglucosidasealfa - MyozymeA(r) and LumizymeA(r), idursulfase - ElapraseA(r), laronidase -AldurazymeA(r)).17 Early infusions must be given by physicians experienced in dealing the ERT.
The Mucopolysaccharidosis I (MPS I) Registry, Genzyme Corp (617591-5500 /, is studying the use of laronidase (Aldurazyme) for Hurler syndrome, Scheie syndrome, and Hurler-Scheie syndrome.
Ha onze tipos de MPS, mas medicamentos para apenas tres delas, a MPS I (laronidase), a MPS II (idursulfase) e a MPS VI (galsulfase).
Approved products include Naglazyme (galsulfase) for mucopolysaccharidosis VI, Kuvan (sapropterin dihydrochloride) for phenylketonuria, Aldurazyme (laronidase) for mucopolysaccharidosis I and Firdapse (amifampridine phosphate) for the treatment of Lambert Eaton Myasthenic Syndrome.
Then, in November, the FDA warned of the potential for foreign particle contamination in all lots of the two aforementioned therapies plus Myozyme (alglucosidase alpha) for Pompe disease, Aldurazyme (laronidase) for mucopolysaccharidosis type I and Thyrogen (thyrotropin alpha) for thyroid cancer.
The company markets Kuvan (sapropterin dihydrochloride) for phenylketonuria, Naglazyme (galsulfase) for mucopolysaccharidosis VI and Aldurazyme (laronidase), with partner Genzyme Corp., for mucopolysaccharidosis I.
NHS Greater Glasgow and Clyde initially said they would not pay for laronidase, a costly enzyme-replacement therapy available in England.
Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled multinational study of recombinant human a-L-iduronidase (Laronidase).
(Novato, CA) announced that Aldurazyme (laronidase) is now available in the United States as the first specific treatment for people with the rare genetic disease mucopolysaccharidosis I (MPS I).
and Genzyme General have announced detailed results from the six-month double-blind Phase 3 clinical trial of Aldurazyme(TM) (laronidase) and preliminary six-month findings from the trial's ongoing open-label extension study.