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Idursulfase parenteral

Updated 2 Feb 2023 | Mucopolysaccharidosis


Infusions of idursulfase.

Drugs List

  • ELAPRASE 6mg/3ml concentrate for solution for infusion
  • idursulfase 6mg/3ml concentrate for solution for infusion
  • Therapeutic Indications


    Mucopolysaccharidosis II (Hunter syndrome): long term treatment



    500 micrograms/kg body weight once weekly by intravenous infusion over 3 hours.

    The infusion duration may be reduced gradually to 1 hour if no infusion-associated adverse reactions occur.


    Children aged 16 months to 18 years

    500 micrograms/kg body weight once weekly by intravenous infusion over 3 hours.

    The infusion duration may be reduced gradually to 1 hour if no infusion-associated adverse reactions occur.

    Additional Dosage Information

    Home treatment with idursulfase may be considered for patients who have received several months of treatment in the clinic and who are tolerating their infusions well. These should be performed under the surveillance of a physician or other healthcare professional.



    Precautions and Warnings

    Children under 16 months
    Restricted sodium intake
    Severe respiratory disease

    Sodium content of formulation may be significant
    Consider pre-medication with antihistamines and/or antipyretics
    Treatment to be initiated and supervised by a specialist
    Delay treatment if acute febrile illness
    Delay treatment if respiratory illness
    Dilute and use as an infusion
    If adverse reactions occur, reduce rate or temporarily stop infusion
    Record name and batch number of administered product
    Resuscitation facilities must be immediately available
    Monitor patient for infusion-associated reactions (IARs)
    Monitor patients with risk factors for respiratory symptoms
    May cause anaphylactic / anaphylactoid reactions
    Discontinue if serious allergic or anaphylactic reaction occurs

    In patients who present with an acute febrile respiratory illness, consider delaying the infusion. Patients receiving supplemental oxygen should have their oxygen readily available during the infusion, in case of an infusion-related reaction.

    Paediatric patients with the complete deletion or large rearrangement genotype have a high probability of developing antibodies, including neutralising antibodies, in response to exposure to idursulfase. Patients with this genotype have a higher probability of developing infusion-related adverse effects and tend to show a muted response as assessed by decrease in urinary output of glycosaminoglycans, liver size and spleen volume compared to patients with the missense genotype.

    Anaphylactoid/anaphylatic reactions, have been observed in some patients up to several years after initiating treatment. Late emergent symptoms and signs of anaphylactoid/anaphylatic reactions have been observed as long as 24 hours after an initial reaction.

    Pregnancy and Lactation


    Idursulfase is contraindicated during pregnancy.

    The manufacturer does not recommend using idursulfase during pregnancy. At the time of writing there is limited published information regarding the use of idursulfase during pregnancy. Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy, embryonal/foetal development, parturition or postnatal development. The potential risk is unknown.


    Idursulfase is contraindicated during breastfeeding.

    The manufacturer does not recommend breastfeeding whilst taking idursulfase. It is not known whether idursulfase is excreted in human breast milk. Available data in animals have shown excretion of idursulfase in breast milk. A risk to the newborn/infant can not be excluded.

    Side Effects

    Abdominal pain
    Anaphylactoid reaction
    Antibody formation
    Chest pain
    Facial oedema
    Infusion-related symptoms
    Peripheral oedema
    Pulmonary embolism
    Swelling (injection site)
    Tongue swelling


    It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.

    The following number will direct the caller to the relevant local centre (0844) 892 0111

    Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( ) or if this is unavailable at the backup site ( ).

    Further Information

    Last Full Review Date: June 2020

    Reference Sources

    Summary of Product Characteristics: Elaprase 2mg/ml concentrate for solution for infusion. Shire Pharmaceuticals Limited. Revised October 2019.

    NICE Evidence Services Available at: Last accessed: 12 June 2020

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