Formulary
Vestronidase alfa: Indications, Dosing, Side Effects and Interactions
Vestronidase alfa is an enzyme produced by recombinant DNA technology used for long-term replacement therapy of non-neurological manifestations of...
MedNext Academy | 3 min read
Vestronidase alfa: Indications, Dosing, Side Effects and Interactions
Vestronidase alfa is an enzyme produced by recombinant DNA technology used for long-term replacement therapy of non-neurological manifestations of...
Drug action
Vestronidase alfa is an enzyme produced by recombinant DNA technology used for long-term replacement therapy of non-neurological manifestations of mucopolysaccharidosis VII (Sly syndrome), a lysosomal storage disorder caused by deficiency of beta-glucuronidase.
Indications and dose
**Non-neurological manifestations of mucopolysaccharidosis VII (under expert supervision)**
- **Adult** (By Intravenous Infusion): 4 mg/kg every 2 weeks.
**Non-neurological manifestations of mucopolysaccharidosis VII (under expert supervision) for vestronidase alfa**
- **Neonate** (By intravenous infusion): 4 mg/kg every 2 weeks.
- **Child** (By intravenous infusion): 4 mg/kg every 2 weeks.
Cautions
Acute febrile illness (delay treatment); acute respiratory illness (delay treatment); hypersensitivity reactions Cautions, further information Hypersensitivity reactions Serious hypersensitivity reactions, including anaphylaxis, have been reported; these may be minimised by pre-treatment with a non-sedating antihistamine with or without an antipyretic, given 30-60 minutes before the start of the infusion. Mepsevii should only be administered by appropriately trained staff, and patients should be observed for at least 1 hour after the infusion; manage hypersensitivity reactions by temporarily interrupting the infusion, reducing the rate of infusion and/or treatment with antihistamines, antipyretics, and/or corticosteroids. If a severe reaction occurs, stop infusion immediately and start appropriate medical treatment; caution is advised on re-administration. M
Side effects
Common or very common Diarrhoea; febrile seizure; hypersensitivity; skin reactions
Pregnancy
Avoid unless potential benefit outweighs risk (no information available). M
Breast feeding
Specialist sources indicate use with caution (no information available). Large molecular weight suggests limited excretion into milk and drug molecule likely to be destroyed in the infant's gastro-intestinal tract; monitor breast-fed infants carefully.
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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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