Generic Medicine Info
Indications and Dosage
Hunter syndrome
Adult: 0.5 mg/kg once weekly, given via infusion over 1-3 hours at an initial rate of 8 mL/hr for the first 15 minutes, may be increased by 8 mL/hr increments every 15 minutes, if tolerated. Max infusion rate: 100 mL/hr, based on individual tolerability.
Child: >16 months Same as adult dose.
Dilute in 100 mL NaCl 0.9%. Mix gently, do not shake.
Special Precautions
Patient with compromised cardiac and/or respiratory function, acute febrile or respiratory illness, risk of fluid overload, severe genetic mutations. Children. Pregnancy and lactation.
Adverse Reactions
Significant: IgG antibody formation, infusion reactions (e.g. rash, pruritus, urticaria).
Cardiac disorders: Arrhythmia, tachycardia.
Ear and labyrinth disorders: Otitis.
Gastrointestinal disorders: Dyspepsia, vomiting, nausea, diarrhoea, abdominal pain.
General disorders and administration site conditions: Fatigue, pyrexia.
Immune system disorders: Bronchospasm.
Infections and infestations: Pneumonia.
Injury, poisoning and procedural complications: Infusion site reactions (e.g. oedema, cutaneous reaction).
Metabolism and nutrition disorders: Peripheral oedema.
Musculoskeletal and connective tissue disorders: Chills, arthralgia, musculoskeletal pain.
Nervous system disorders: Headache, dizziness, tremor.
Respiratory, thoracic and mediastinal disorders: Wheezing, cough, tachypnoea, chest pain, pulmonary embolism.
Skin and subcutaneous tissue disorders: Erythema.
Vascular disorders: Hypertension, hypotension, flushing, cyanosis.
Potentially Fatal: Severe hypersensitivity/anaphylactoid reactions (e.g. respiratory distress, hypoxia, hypotension, urticaria, angioedema, hypoxia).
IV/Parenteral: C
Monitoring Parameters
Monitor infusion-related reactions, pulmonary function, oxygen saturation, blood pressure and measure weight prior to each infusion.
Description: Idursulfase is a recombinant form of iduronate-2-sulfatase, an enzyme that catalyses the hydrolysis of mucopolysaccharides glycosaminoglycans dermatan sulfate and heparan sulfate. It is used as enzyme replacement to prevent accumulation of polysaccharides in lysosomes which can lead to physical changes, CNS involvement, cardiac, respiratory and mobility dysfunction.
Distribution: Volume of distribution: <27 years: 213-254 mL/kg.
Excretion: Elimination half-life: <27 years: 44-48 minutes.
Store between 2-8°C. Protect from light. Do not freeze or shake.
Any unused portions should be disposed of in accordance with local requirements.
MIMS Class
Other Agents Affecting Metabolism
ATC Classification
A16AB09 - idursulfase ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
Anon. Idursulfase. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. Accessed 05/12/2017.

Buckingham R (ed). Idursulfase. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. Accessed 05/12/2017.

Elaprase Solution, Concentrate (Shire US Manufacturing Inc.). DailyMed. Source: U.S. National Library of Medicine. Accessed 05/12/2017.

Joint Formulary Committee. Idursulfase. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. Accessed 05/12/2017.

McEvoy GK, Snow EK, Miller J et al (eds). Idursulfase. AHFS Drug Information (AHFS DI) [online]. American Society of Health-System Pharmacists (ASHP). Accessed 05/12/2017.

Disclaimer: This information is independently developed by MIMS based on Idursulfase from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2022 MIMS. All rights reserved. Powered by
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