Drug reference

Idursulfase

BNF 80 (2020–2021)

Source: BNF 80 (2020–2021)
PDF pages: 1150

Review source pages for extracted sections and age labels. BNF dose snippets show only their first line.

Overview

Drug Action

Idursulfase is an enzyme produced by recombinant DNA technology licensed for long-term replacement therapy in mucopolysaccharidosis II (Hunter syndrome), a lysosomal storage disorder caused by deﬁciency of iduronate-2-sulfatase.

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Indications And Dose

Mucopolysaccharidosis II (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 500 micrograms/kg once weekly

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Cautions

Acute febrile respiratory illness (consider delaying treatment). infusion-related reactions can occur. Blood and nutrition severe respiratory disease

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Safety

Side Effects

▶Common or very common Arrhythmias. arthralgia. chest pain. cough. cyanosis. diarrhoea. dizziness. dyspnoea. fever. ﬂushing. gastrointestinal discomfort. headache. hypertension. hypotension. hypoxia. infusion related reaction. nausea. oedema. respiratory disorders. skin reactions. tongue swelling. tremor. vomiting ▶Frequency not known Hypersensitivity SIDE-EFFECTS, FURTHER INFORMATION Infusion-related reactions often occur, they can be managed by slowing the infusion rate or interrupting the infusion, and can be minimised by pre-treatment with an antihistamine and an antipyretic. Recurrent infusion-related reactions may require pre-treatment with a corticosteroid—consult product literature for details.

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Conception And Contraception

Contra-indicated in women of child-bearing potential.

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Pregnancy

Manufacturer advises avoid.

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Breast Feeding

Manufacturer advises avoid—present in milk in animal studies.

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Dose and formulations

Directions For Administration

For intravenous infusion (Elaprase ®), give intermittently in Sodium chloride 0.9%; dilute requisite dose in 100 mL infusion ﬂuid and mix gently (do not shake); give over 3 hours (gradually reduced to 1 hour if no infusion-related reactions).

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Medicinal Forms

There can be variation in the licensing of different medicines containing the same drug. Solution for infusion ▶Elaprase (Shire Pharmaceuticals Ltd) A Idursulfase 2 mg per 1 ml Elaprase 6mg/3ml concentrate for solution for infusion vials | 1 vial P £1,985.00

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Dose text by age group

Adult

500 micrograms/kg once weekly

First-line fragment. Read the complete Indications and Dose section above.

PDF page 1150

Age labels are extracted for navigation. Check the complete source section and current local guidance before clinical use.

Drug reference

Idursulfase

BNF 80 (2020–2021)

Source: BNF 80 (2020–2021)
PDF pages: 1150

Review source pages for extracted sections and age labels. BNF dose snippets show only their first line.

Overview

Drug Action

PDF page 1150

Indications And Dose

Mucopolysaccharidosis II (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 500 micrograms/kg once weekly

PDF page 1150

Cautions

Acute febrile respiratory illness (consider delaying treatment). infusion-related reactions can occur. Blood and nutrition severe respiratory disease

PDF page 1150

Safety

Side Effects

PDF page 1150

Conception And Contraception

Contra-indicated in women of child-bearing potential.

PDF page 1150

Pregnancy

Manufacturer advises avoid.

PDF page 1150

Breast Feeding

Manufacturer advises avoid—present in milk in animal studies.

PDF page 1150

Dose and formulations

Directions For Administration

PDF page 1150

Medicinal Forms

PDF page 1150

Dose text by age group

Adult

500 micrograms/kg once weekly

First-line fragment. Read the complete Indications and Dose section above.

PDF page 1150

Age labels are extracted for navigation. Check the complete source section and current local guidance before clinical use.