Formulary
Cipaglucosidase alfa: Indications, Dosing, Side Effects and Interactions
Cipaglucosidase alfa is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy in Pompe disease, a lysosomal...
MedNext Academy | 3 min read
Cipaglucosidase alfa: Indications, Dosing, Side Effects and Interactions
Cipaglucosidase alfa is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy in Pompe disease, a lysosomal...
Drug action
Cipaglucosidase 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 [in combination with miglustat] (under expert supervision)**
- **Adult** (By Intravenous Infusion): 20 mg/kg every 2 weeks, infusion to be started 1 hour (but no more than 3 hours) after taking miglustat.
Cautions
Cardiac dysfunction; infusion-related reactions; respiratory dysfunction Cautions, further information Infusion-related reactions Infusion-related reactions can occur and cipaglucosidase 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 Arrhythmias; asthenia; chest discomfort; chills; cough; diarrhoea; dizziness; drowsiness; dyspnoea; fever; flatulence; flushing; gastrointestinal discomfort; headaches; hyperhidrosis; hypersensitivity; hypertension; muscle complaints; muscle weakness; nausea; pain; skin reactions; taste altered; tremor; vomiting Uncommon Arthralgia; asthma; balance impaired; hypotension; malaise; musculoskeletal stiffness; oesophageal disorder; oral disorders; oropharyngeal discomfort; pallor; paraesthesia; peripheral swelling; throat oedema; wheezing
Interactions
**Other interactions (1):**
- No interaction studies have been performed related to the use of cipaglucosidase alfa or with cipaglucosidase alfa in combination with miglustat.
Pregnancy
Avoid (toxicity in animal studies). M
Breast feeding
Avoid (present in milk in animal studies). 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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