Drug reference
Alglucosidase alfa is an enzyme produced by recombinant DNA technology licensed for long-term replacement therapy in Pompe disease, a lysosomal storage disorder caused by deficiency of acid alpha- glucosidase.
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Pompe disease (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 20 mg/kg every 2 weeks
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Cardiac dysfunction. infusion-related reactions—consult product literature. respiratory dysfunction
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▶Common or very common Anxiety. arrhythmias. chest discomfort. chills. cough. cyanosis. diarrhoea. dizziness. fatigue. feeling hot. fever. flushing. hyperhidrosis. hypersensitivity. hypertension. irritability. local swelling . muscle complaints. nausea. oedema. pallor. paraesthesia. respiratory disorders. skin reactions. throat complaints. tremor. vomiting ▶Frequency not known Abdominal pain. angioedema. apnoea. arthralgia. cardiac arrest. dyspnoea. excessive tearing. eye inflammation. headache. hypotension. nephrotic syndrome. peripheral coldness. proteinuria. vasoconstriction SIDE-EFFECTS, FURTHER INFORMATION Infusion-related reactions are very common, calling for use of antihistamine, antipyretic, or corticosteroid; consult product literature for details.
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Toxicity in animal studies, but treatment should not be withheld.
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Manufacturer advises avoid—no information available.
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▶Monitor closely if cardiac dysfunction. ▶Monitor closely if respiratory dysfunction. ▶Monitor immunoglobulin G (IgG) antibody concentration.
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For intravenous infusion (Myozyme ®), give intermittently in Sodium chloride 0.9%; reconstitute 50 mg with 10.3 mL water for injections to produce 5 mg/mL solution; gently rotate vial without shaking; dilute requisite dose with infusion fluid to give a final concentration of 0.5–4 mg/mL; give through a low protein-binding in-line filter (0.2 micron) at an initial rate of 1 mg/kg/hour increased by 2 mg/kg/hour every 30 minutes to max. 7 mg/kg/hour. Blood and nutrition
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There can be variation in the licensing of different medicines containing the same drug. Powder for solution for infusion ▶Myozyme (Genzyme Therapeutics Ltd) Alglucosidase alfa 50 mg Myozyme 50mg powder for concentrate for solution for infusion vials | 1 vial P £356.06 (Hospital only) 2.11 Tyrosinaemia type I ENZYME INHIBITORS › 4- HYDROXYPHENYLPYRUVATE DIOXYGENASE INHIBITORS
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20 mg/kg every 2 weeks
First-line fragment. Read the complete Indications and Dose section above.
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