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Elaprase is a brand name for Idursulfase. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: Elaprase is indicated for the long-term treatment of patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). Heterozygous females were not studied in the clinical trials.
Verbatim from this product's EMA label. Tap a section to expand.
This treatment should be supervised by a physician or other healthcare professional experienced in the management of patients with MPS II disease or other inherited metabolic disorders. 4). 6. Infusion at home may be considered for patients who have received several months of treatment in the clinic and who are tolerating their infusions well.
Home infusions should be performed under the surveillance of a physician or other healthcare professional. Special populations Elderly patients There is no clinical experience in patients over 65 years of age. 3 Patients with renal or hepatic impairment There is no clinical experience in patients with renal or hepatic insufficiency.
2). 5 mg/kg body weight weekly. 6.
5 mg/kg idursulfase weekly in the TKT024 phase II/III 52-week placebo-controlled study were almost all mild to moderate in severity. The most common were infusion-related reactions, 202 of which were reported in 22 out of 32 patients following administration of a total of 1580 infusions.
In the placebo treatment group 128 infusion-related reactions were reported in 21 out of 32 patients following administration of a total of 1612 infusions. Since more than one infusion-related reaction may have occurred during any single infusion, the above numbers are likely to overestimate the true incidence of infusion reactions.
Related reactions in the placebo group were similar in nature and severity to those in the treated group. The most common of these infusion-related reactions included cutaneous reactions (rash, pruritus, urticaria, and erythema), pyrexia, flushing, wheezing, dyspnoea, headache, vomiting, abdominal pain, nausea, and chest pain.
The frequency of infusion-related reactions decreased over time with continued treatment. Tabulated list of adverse reactions Adverse reactions are listed in table 1 with information presented by system organ class and frequency. Frequency is given as very common (≥1/10), common (≥1/100 to <1/10) or uncommon (≥1/1,000 to <1/100).
The occurrence of an adverse reaction in a single patient is defined as common in view of the number of patients treated. Within each frequency grouping, adverse reactions are 5 presented in order of decreasing seriousness. Adverse reactions only reported during the post marketing period are also included in the table with a frequency “not known” (cannot be estimated from the available data).
Table 1:
Adverse reactions from clinical trials and post-marketing experience in patients treated with Elaprase. 5 mg/kg weekly or every other week. Four patients experienced a hypoxic episode during one or several infusions, which necessitated oxygen therapy in 3 patients with severe underlying obstructive airway disease (2 with a pre-existing tracheostomy).
8). During clinical trials, the most common infusion-related reactions included cutaneous reactions (rash, pruritus, urticaria), pyrexia, headache, hypertension, and flushing. Infusion-related reactions were treated or ameliorated by slowing the infusion rate, interrupting the infusion, or by administration of medicinal products, such as antihistamines, antipyretics, low-dose corticosteroids (prednisone and methylprednisolone), or beta-agonist nebulisation.
Special care should be taken when administering an infusion in patients with severe underlying airway disease. These patients should be closely monitored and infused in an appropriate clinical setting. Caution must be exercised in the management and treatment of such patients by limitation or careful monitoring of antihistamine and other sedative medicinal product use.
Institution of positive-airway pressure may be necessary in some cases. Delaying the infusion in patients who present with an acute febrile respiratory illness should be considered. Patients using supplemental oxygen should have this treatment readily available during infusion in the event of an infusion-related reaction.
Anaphylactoid/anaphylactic reactions Anaphylactoid/anaphylactic reactions, which have the potential to be life threatening, have been observed in some patients treated with idursulfase up to several years after initiating treatment.
Late emergent symptoms and signs of anaphylactoid/anaphylactic reactions have been observed as long as 24 hours after an initial reaction. If an anaphylactoid/anaphylactic reaction occurs the infusion should be immediately suspended and appropriate treatment and observation initiated.
The current medical standards for emergency treatment are to be observed. Patients experiencing severe or refractory anaphylactoid/anaphylactic reactions may require prolonged clinical monitoring. Patients who have experienced anaphylactoid/anaphylactic reactions should be treated with caution when re- administering idursulfase, appropriately trained personnel and equipment for emergency resuscitation (including epinephrine) should be available during infusions.
1 if hypersensitivity is not controllable.
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The most severe episode occurred in a patient with a febrile respiratory illness and was associated with hypoxia during the infusion, resulting in a short seizure. In the fourth patient, who had less severe underlying disease, spontaneous resolution occurred shortly after the infusion was interrupted.
These events did not recur with subsequent infusions using a slower infusion rate and administration of pre-infusion medicinal products, usually low-dose steroids, antihistamine, and beta-agonist nebulisation. The fifth patient, who had pre-existing 6 cardiopathy, was diagnosed with ventricular premature complexes and pulmonary embolism during the study.
4). 4). Immunogenicity Across 4 clinical studies (TKT008, TKT018, TKT024 and TKT024EXT), 53/107 patients (50%) developed anti-idursulfase IgG antibodies at some point. The overall neutralizing antibody rate was 26/107 patients (24%). 5mg/kg weekly idursulfase had at least 1 blood sample that tested positive for anti- idursulfase antibodies, and 37 % (23/63) tested positive for antibodies on at least 3 consecutive study visits.
Twenty-one percent (13/63) tested positive for neutralizing antibodies at least once and 13 % (8/63) tested positive for neutralizing antibodies on at least 3 consecutive study visits. 5 years of age. 1% (16 of 28) tested positive for antibodies on at least three consecutive study visits.
Fifty-four percent of patients tested positive for neutralizing antibodies at least once and half of the patients tested positive for neutralizing antibodies on at least three consecutive study visits. All patients with the complete deletion/large rearrangement genotype developed antibodies, and the majority of them (7/8) also tested positive for neutralizing antibodies on at least 3 consecutive occasions.
All patients with the frameshift/splice site mutation genotype developed antibodies and 4/6 also tested positive for neutralizing antibodies on at least 3 consecutive study visits. 1). Paediatric population Adverse reactions reported in the paediatric population were, in general, similar to those reported in adults.
Reporting of suspected adverse reactions Reporting suspected […]
3). Patients with the complete deletion/large rearrangement genotype Paediatric patients with the complete deletion/large rearrangement genotype have a high probability of developing antibodies, including neutralizing antibodies, in response to exposure to idursulfase.
Patients with this genotype have a higher probability of developing infusion-related adverse events 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.
8). 1 mg) per vial. 6% of the WHO recommended maximum daily intake of 2 g sodium for an adult. Traceability In order to improve the traceability of biological medicinal products, the name and batch number of the administered product should be clearly recorded.