IntravenousGaucher disease type 1, Gaucher disease type 3Adult: Initially, 60 units/kg once every 2 weeks via infusion over 1-2 hours. Dosage is individualised based on the severity of disease and adjusted according to response. Child: >2 years Same as adult dose.
|
Add 5 ml or 10.2 mL of sterile water for inj to the vial labelled as containing 200 mg or 400 mg, respectively, to provide a solution containing 40 units/mL. Further dilute with NaCl 0.9% to a final volume of 100-200 mL.
|
|
Patient with respiratory symptoms in the absence of fever. Children. Pregnancy and lactation.
|
Significant: Hypersensitivity reactions (e.g. anaphylaxis), antibody IgG formation, pulmonary hypertension/pneumonia.
Cardiac disorders: Tachycardia, dyspnoea.
Gastrointestinal disorders: Vomiting, nausea, abdominal cramp, diarrhoea.
General disorders and administration site conditions: Fatigue, fever.
Immune system disorders: Pruritus, urticaria.
Injury, poisoning and procedural complications: Inj site reactions.
Musculoskeletal and connective tissue disorders: Arthralgia, backpain, chills.
Nervous system disorders: Dizziness, headache, paraesthesia.
Respiratory, thoracic and mediastinal disorders: Coughing.
Skin and subcutaneous tissue disorders: Rash.
Vascular disorders: Hypotension, flushing, cyanosis.
|
Monitor CBC, platelets, LFT, IgG antibody formation periodically during the 1st year of treatment; chitotriosidase, ACE, acid phosphatase, Fe, iron-binding capacity, ferritin, vitamin B12; MRI or CT scan of liver and spleen, skeletal x-rays, DXA; pulmonary function tests; ECG; growth in paediatric patients.
|
Description: Imiglucerase is a recombinant form of β-glucocerebrosidase, an enzyme that catalyses the hydrolysis of glucocerebroside to glucose and ceramide. It used to replace glucocerebrosidase enzyme that is deficient in Gaucher’s disease. Pharmacokinetics: Distribution: Volume of distribution: 0.09-0.15 L/kg. Excretion: Elimination half-life: 3.6-10.4 minutes.
|
|
|
A16AB02 - imiglucerase ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
|
Anon. Imiglucerase. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 02/01/2018. Buckingham R (ed). Alglucerase. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/01/2018. Cerezyme Injection (Genzyme Corporation). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed/. Accessed 02/01/2018. Joint Formulary Committee. Imiglucerase. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/01/2018. McEvoy GK, Snow EK, Miller J et al (eds). Imiglucerase. AHFS Drug Information (AHFS DI) [online]. American Society of Health-System Pharmacists (ASHP). https://www.medicinescomplete.com. Accessed 02/01/2018.
|