IntravenousGaucher disease type 1, Gaucher disease type 3Adult: As a long-term enzyme replacement therapy in patients who exhibit clinically significant non-neurological manifestations including anaemia, bone disease, hepatomegaly or splenomegaly, and thrombocytopenia: Initially, 60 units/kg once every 2 weeks via infusion. Doses of 15 units/kg once every 2 weeks have been given to improve haematological parameters and organomegaly (but not bone parameters). Initial infusions are administered at a rate of ≤0.5 unit/kg/min, then at subsequent doses, infusion rates may be increased up to 1 unit/kg/min. Alternatively, initial dose range of 2.5 units/kg 3 times weekly, up to 60 units/kg once every 2 weeks may be administered via infusion over 1-2 hours. Dosage is individualised based on clinical manifestations of disease and adjusted according to response and therapeutic goals. Dosage recommendations may vary among individual products and between countries (refer to specific product guidelines). Child: ≥2 years Same as adult dose.
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Reconstitute a 400 units vial with 10.2 mL sterile water for inj resulting in a 40 units/mL concentration. Withdraw the appropriate volume of reconstituted solution and further dilute in 0.9% NaCl IV solution to a final volume of 100-200 mL. Gently invert the infusion bag to mix the solution; avoid vigorous shaking and agitation. Administer the diluted solution through an in-line low protein-binding 0.2-micrometre filter to remove any protein particles.
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Patient who has developed antibodies or symptoms of hypersensitivity to alglucerase. Patients who have undergone splenectomy. Children. Pregnancy and lactation.
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Significant: IgG antibody formation, hypersensitivity reactions, including anaphylaxis; pulmonary hypertension.
Cardiac disorders: Tachycardia, chest discomfort, cyanosis.
Gastrointestinal disorders: Nausea, vomiting, diarrhoea, abdominal cramping.
General disorders and administration site conditions: Infusion site discomfort, swelling, or burning; inj site sterile abscess; fever, fatigue, rigors.
Immune system disorders: Angioedema.
Musculoskeletal and connective tissue disorders: Back pain, arthralgia.
Nervous system disorders: Dizziness, headache, paraesthesia.
Respiratory, thoracic and mediastinal disorders: Cough, dyspnoea, pneumonia.
Skin and subcutaneous tissue disorders: Rash, urticaria, pruritus.
Vascular disorders: Hypotension, flushing.
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Monitor CBC, platelets, LFTs, IgG antibody formation (periodically during the 1st year of therapy); pulmonary function tests, and levels of ACE, chitotriosidase, acid phosphatase, vitamin B, Fe, Fe-binding capacity, and ferritin. Perform CT scan or MRI (liver and spleen volume), skeletal x-rays, dual-energy x-ray absorptiometry (DXA), and ECG or echocardiography. Closely monitor for signs and symptoms of hypersensitivity.
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Description: Imiglucerase, an analogue of β-glucocerebrosidase, is an enzyme produced by recombinant DNA technology using mammalian Chinese hamster ovary cell culture. β-glucocerebrosidase, the enzyme deficient in Gaucher disease, is needed to catalyse the hydrolysis of glucocerebroside to glucose and ceramide and prevent the accumulation of glucocerebroside in body tissues including the liver, spleen, and bone marrow. Imiglucerase replaces the deficient glucocerebrosidase endogenous enzyme, thereby correcting initial pathophysiology and preventing secondary pathology. Pharmacokinetics: Distribution: Volume of distribution: 0.09-0.15 L/kg. Excretion: Elimination half-life: 3.6-10.4 minutes.
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Intact vials: Store between 2-8°C. Solutions diluted in 0.9% NaCl for infusion are stable for up to 24 hours when stored between 2-8°C. Protect from light.
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A16AB02 - imiglucerase ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
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Anon. Imiglucerase. AHFS Clinical Drug Information [online]. Bethesda, MD. American Society of Health-System Pharmacists, Inc. https://www.ahfscdi.com. Accessed 23/05/2022. Anon. Imiglucerase. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 11/04/2022. Buckingham R (ed). Alglucerase. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 11/04/2022. Cerezyme 400 Units (Sanofi-Aventis [Malaysia] Sdn. Bhd.). National Pharmaceutical Regulatory Agency - Ministry of Health Malaysia. https://www.npra.gov.my. Accessed 11/04/2022. Cerezyme 400 Units Powder for Concentrate for Solution for Infusion (Aventis Pharma Ltd, Trading as: Sanofi Genzyme). MHRA. https://products.mhra.gov.uk. Accessed 11/04/2022. Cerezyme 400 Units Powder for Concentrate for Solution for Infusion (Sanofi-Aventis Singapore Pte. Ltd.). MIMS Singapore. http://www.mims.com/singapore. Accessed 23/05/2022. Cerezyme Injection, Powder, Lyophilized, for Solution (Genzyme Corporation). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 11/04/2022. Joint Formulary Committee. Imiglucerase. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 11/04/2022. Sanofi-Aventis New Zealand Limited. Cerezyme Powder for Infusion, 40 U/mL, 400 U/vial data sheet 16 July 2020. Medsafe. http://www.medsafe.govt.nz. Accessed 11/04/2022.
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