IntravenousMucopolysaccharidosis IIAdult: 0.5 mg/kg once weekly via IV infusion at an initial rate of 8 mL/hr for the 1st 15 minutes; if tolerated, may increase by 8 mL/hour increments every 15 minutes up to Max infusion rate of 100 mL/hour. Initial infusion should be over 3 hours, which may be gradually reduced to 1 hour if tolerated. Total infusion time should not exceed 8 hours. Child: ≥5 years Same as adult dose.
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Dilute in 100 mL of 0.9% NaCl solution. Mix gently, do not shake.
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Patient with compromised respiratory function, acute febrile or respiratory illness; severe genetic mutations (e.g. large gene rearrangement, complete gene deletion; nonsense, frameshift or splice-site mutations), risk for fluid overload or in conditions where a fluid restriction is indicated (e.g. compromised cardiac and/or respiratory function, acute underlying respiratory illness). Children. Pregnancy and lactation.
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Significant: Anti-idursulfase IgG antibody formation; infusion-related reactions (e.g. cutaneous reaction [rash, pruritus], pyrexia, headache, hypertension, flushing).
Cardiac disorders: Tachycardia, arrhythmia, chest pain.
Gastrointestinal disorders: Nausea, vomiting, abdominal pain, diarrhoea, dyspepsia.
General disorders and administration site conditions: Infusion-site swelling, face oedema, peripheral oedema.
Musculoskeletal and connective tissue disorders: Arthralgia, musculoskeletal pain.
Nervous system disorders: Dizziness, tremor.
Respiratory, thoracic and mediastinal disorders: Wheezing, dyspnoea, cough, bronchospasm, tachypnoea.
Skin and subcutaneous tissue disorders: Erythema.
Vascular disorders: Cyanosis.
Potentially Fatal: Serious hypersensitivity or anaphylactic reactions (presenting as hypoxia, respiratory distress, urticaria, tongue or throat angioedema, hypotension), cardiorespiratory arrest, respiratory failure, cardiac failure, pneumonia.
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Monitor blood pressure, oxygen saturation, pulmonary function; signs of infusion-related and hypersensitivity reactions. Obtain weight before each infusion.
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Description: Idursulfase, a recombinant form of iduronate-2-sulfatase, is an enzyme responsible for the hydrolysis of the mucopolysaccharides dermatan sulfate and heparan sulfate in various cells. It is used as an enzyme replacement to prevent the accumulation of polysaccharides in lysosomes which can lead to physical changes, CNS involvement, cardiac, respiratory and mobility dysfunction. Pharmacokinetics: Excretion: Elimination half-life: 44-48 minutes (<27 years).
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Store between 2-8°C. Do not freeze. Protect from light. Diluted solution may be stored between 2-8°C for up to 24 hours.
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A16AB09 - idursulfase ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
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Anon. Idursulfase. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 29/04/2022. Buckingham R (ed). Idursulfase. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 29/04/2022. Elaprase 2 mg/mL Concentrate for Solution for Infusion (Shire Human Genetic Therapies AB). MHRA. https://products.mhra.gov.uk. Accessed 29/04/2022. Elaprase Solution for Intravenous Infusion (Sanofi-Aventis [Malaysia] Sdn. Bhd.). National Pharmaceutical Regulatory Agency - Ministry of Health Malaysia. https://www.npra.gov.my. Accessed 29/04/2022. Elaprase Solution, Concentrate (Takeda Pharmaceuticals America, Inc.). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 29/04/2022. Joint Formulary Committee. Idursulfase. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 29/04/2022. Sanofi-Aventis New Zealand Limited. Elaprase (Idursulfase) 6 mg/3 mL Concentrate for Intravenous Solution for Infusion data sheet 06 April 2021. Medsafe. http://www.medsafe.govt.nz. Accessed 29/04/2022.
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