Formulary
Avalglucosidase alfa: Indications, Dosing, Side Effects and Interactions
Avalglucosidase alfa, a modification of alglucosidase alfa, is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement...
MedNext Academy | 3 min read
Avalglucosidase alfa: Indications, Dosing, Side Effects and Interactions
Avalglucosidase alfa, a modification of alglucosidase alfa, is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement...
Drug action
Avalglucosidase alfa, a modification of alglucosidase alfa, is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy in Pompe disease, a lysosomal storage disorder caused by deficiency of acid alpha-glucosidase.
Indications and dose
**Pompe disease (under expert supervision)**
- **Adult** (By Intravenous Infusion): 20 mg/kg every 2 weeks.
**Pompe disease (under expert supervision) for avalglucosidase alfa**
- **Child 7 months-17 years** (By intravenous infusion): 20 mg/kg every 2 weeks; increased if necessary to 40 mg/kg every 2 weeks, dose may only be increased in patients with infantile-onset disease who do not respond adequately to standard dosing, and in the absence of safety concerns.
Cautions
Cardiac dysfunction; infusion-related reactions; respiratory dysfunction; susceptibility to fluid volume overload Cautions, further information Infusion-related reactions Infusion-related reactions can occur and avalglucosidase alfa should only be administered when appropriately trained staff are available; these reactions may be minimised by premedication with an antihistamine, antipyretic, and/or corticosteroid. Patients should be monitored for signs of infusion-related reactions during and after administration, and managed appropriately-consult product literature. M
Side effects
Common or very common Asthenia; chest discomfort; chills; cough; diarrhoea; dizziness; dyspnoea; eye disorders; headache; hypersensitivity; hypertension; influenza like illness; muscle complaints; nausea; oral disorders; pain; skin reactions; tremor; vomiting Uncommon Angioedema; arrhythmias; conjunctivitis; drowsiness; dysphagia; eye pruritus; fever; flushing; gastrointestinal discomfort; hyperhidrosis; hyperthermia; hypotension; localised oedema; paraesthesia; peripheral swelling; respiratory disorders; throat irritation Frequency not known Infusion related reaction
Pregnancy
Use only if potential benefit outweighs risk-no information available. M
Breast feeding
Use only if potential benefit outweighs risk-no information available. M
Medicinal forms
Clinical governance
**Author:** MedNext Clinical Team. **Clinical reviewer:** Dr Shameer Deen, MBBS, MS, MRCS. **Sources:** BNF, Indian Pharmacopoeia, CIMS India. **Correction:** Report errors at support@mednext.academy.
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