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(i-dur-sul-fase) ,


(trade name)


Therapeutic: replacement enzyme
Pharmacologic: enzymes
Pregnancy Category: C


Treatment of Mucopolysaccharidosis II (MPS II, Hunter syndrome).


Replaces an enzyme not present in Hunter syndrome. Absence of the enzyme results in cellular accumulation of aminoglycans, organomegaly and organ dysfunction.

Therapeutic effects

Improved walking capacity.


Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: unknownnown.
Half-life: 44–48 min.

Time/action profile

IVrapidend of infusionunknown


Contraindicated in: No known contraindications.
Use Cautiously in: Compromised respiratory function/acute respiratory disease (increased risk of infusion reactions); Severe genetic mutations (↑ risk of serious adverse reactions)Patients at risk for fluid overload, acute respiratory disorder, cardiac or respiratory illness (↑ risk of compromised cardiac or respiratory function) Obstetric: Use in pregnancy only if clearly needed; Obstetric: Use cautiously in lactation; Pediatric: Children <16 mo (safety not established).

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)
  • malaise


  • hypertension


  • dyspepsia


  • cutaneous reactions (most frequent)


  • infusion site swelling


  • arthralgia (most frequent)


  • anaphylaxis (life-threatening)
  • fever (most frequent)


Drug-Drug interaction

None noted.


Intravenous (Adults and Children ≥5 yr) 0.5 mg/kg weekly


Solution for IV infusion (requires dilution): 2 mg/mL

Nursing implications

Nursing assessment

  • Observe for signs and symptoms of anaphylaxis (rash, pruritus, laryngeal edema, wheezing) during and for 24 hrs after infusion. Keep epinephrine, an antihistamine, corticosteroids, and resuscitation equipment close by in case of anaphylactic reaction.
  • Monitor for infusion-related reactions (headache, fever, rash, pruritus, erythema, urticaria, hypertension. Most reactions are managed with antihistamines and/or corticosteroids prior to or during infusions, slowing rate of infusion, and/or early discontinuation if reaction is serious.

Potential Nursing Diagnoses

Impaired physical mobility (Indications)


  • Patients with compromised respiratory function or acute respiratory disease are at increased risk of life-threatening infusion reactions. Consider delaying infusion in patients with concomitant acute respiratory and/or febrile illness.
  • Intermittent Infusion: Determine number of vials required for the dose ordered. If number of vials includes a fraction, round up to next whole number. Vials are for single use; discard remaining medication. Dilute each vial in 100 mL of 0.9% NaCl. Use an infusion set with a 0.2 micrometer filter for administration. Mix gently; do not shake solution. Solution is clear to slightly opalescent; do not administer solutions that are discolored or contain particulate matter. Solution may be stored in refrigerator for up to 48 hr. Discard solution of not administered or refrigerated within 8 hrs.
  • Rate: Administer at 8 mL/hr for first 15 min. If well tolerated, may increase by 8 mL/hr at 15 min intervals to administer total volume within desired time. Do not exceed 100 mL/hr. May slow, temporarily stop, or discontinue for that visit if infusion reactions occur. Administer total infusion over 1–3 hrs. May require longer times if infusion reactions occur; should not exceed 8 hrs.
  • Y-Site Incompatibility: Do not administer other medications or solutions in same tubing as idursulfase.

Patient/Family Teaching

  • Explain purpose of infusion to patient and parents.
  • Advise female patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
  • Inform patient that a Hunter Outcome Survey was established to evaluate long term treatments. For information, visit or call 1–866–888–0660.

Evaluation/Desired Outcomes

  • Improved walking capacity in patients with Hunter syndrome.
Drug Guide, © 2015 Farlex and Partners
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References in periodicals archive ?
Release date- 29072019 - BEIJING - CANbridge Pharmaceuticals Inc., a biopharmaceutical company developing innovative drug candidates to treat underserved medical conditions in China and other markets, announced that it has filed a New Drug Application (NDA) with the National Medical Products Administration (NMPA) for Hunterase (idursulfase beta), a human recombinant iduronate-2-sulfatase (IDS) enzyme replacement therapy, to treat Hunter Syndrome in China.
Two main therapies have been reported for MPS II patients: The first one is idursulfase as an enzyme replacement therapy (ERT) which is a recombinant form of human IDS.
He had been diagnosed with Hunter syndrome at the age of 40 years and had received enzyme replacement therapy with idursulfase infusion.
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.
Ha onze tipos de MPS, mas medicamentos para apenas tres delas, a MPS I (laronidase), a MPS II (idursulfase) e a MPS VI (galsulfase).
Therefore, comparison of the incidence of antibodies to Oncaspar[R] with the incidence of antibodies to other products may be misleading' Elaprase The data reflect the percentage of patients (idursulfase) whose test results were positive for antibodies to idursulfase in specific assays, and are highly dependent on the sensitivity and specificity of these assays.
Mecanismo Doencaalvo Nome do farmaco de acao Fabry Betagalsidase TRE Fabry Alfagalsidase TRE Fabry AT1001 Chaperona Gaucher Velaglucerase alfa (glicocerebrosidase TRE produzida em celulas humanas) Gaucher Glicocerebrosidase produzida em TRE celulas vegetais Gaucher Miglustate ISS Gaucher Genz-112638 ISS Gaucher AT2101 Chaperona MPS I Laronidase TRE MPS II Idursulfase TRE MPS VI Galsulfase TRE Niemann-Pick B Esfingomielinase recombinante TRE Niemann-Pick C Miglustate ISS Pompe Alglucosidase alfa([alpha]-glicosidase TRE acida produzida em celulas CHO) Pompe [alfa]-glicosidase acida recombinante TRE produzida a partir do leite de coelhos transgenicos Tay-Sachs (tardia) Miglustate ISS Tay-Sachs (tardia) Pirimetamina Chaperona Registro Doencaalvo Fase de desenvolvimento Anvisa?
The new Co-Pay Assistance Program will take effect immediately, and will apply to eligible ELAPRASE (idursulfase) patients and VPRIV patients in the US, the company added.