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How to use NEXVIAZYME (alglucosidase α)
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Article source: Seagull Pharmacy
Jul 17, 2025

NEXVIAZYME (alglucosidase α) is a treatment for Pompe disease, usually for injection: 100 mg avalglucosidase a-ngpt, a white to light yellow lyophilized powder, in a single-dose vial for reconstitution.

How to use NEXVIAZYME (alglucosidase α)

Understanding the correct use of the drug is the key to ensuring the treatment effect.

Dosage and frequency of medication

NEXVIAZYME (alglucosidase α) is administered by intravenous infusion, and the dose is adjusted according to the patient's weight. For patients weighing 30 kg or more, the recommended dose is 20mg/kg every two weeks; for patients weighing less than 30 kg, 40 mg/kg is recommended every two weeks. This precise dose setting is designed to meet the treatment needs of patients of different weights.

Infusion and reconstitution dilution steps

NEXVIAZYME (alglucosidase α) lyophilized powder needs to be reconstituted and diluted before infusion. When reconstituted, sterile water for injection should be used, and direct impact on the lyophilized powder should be avoided to reduce foaming. When diluting, choose a suitable 5% glucose injection infusion bag and mix gently to avoid stirring and producing bubbles. When infusing, the initial rate is recommended to be 1 mg/kg/hour. If there are no adverse reactions, it can be gradually increased to an appropriate rate.

Correct use of NEXVIAZYME (alglucosidase α) requires strict adherence to dosage and frequency requirements, as well as reconstitution, dilution and infusion steps to ensure the best efficacy of the drug.

Adverse reactions when using NEXVIAZYME (alglucosidase α)

Understanding the adverse reactions that may be caused by the drug will help patients respond in time and ensure safety.

Common adverse reactions

When using NEXVIAZYME (alglucosidase α), patients may experience common adverse reactions such as headache, fatigue, diarrhea, nausea, and joint pain. These reactions are mostly mild to moderate and can be tolerated by most patients.

Serious adverse reactions

Although rare, NEXVIAZYME (alglucosidase α) may also cause serious adverse reactions, such as allergic reactions and infusion-related reactions. These reactions may include chest discomfort, erythema, generalized edema, hypotension and other symptoms, which require immediate medical attention.

When using NEXVIAZYME (alglucosidase α), patients should pay attention to common and serious adverse reactions. Once abnormalities are found, they should seek medical advice in time.

Methods to alleviate adverse reactions of NEXVIAZYME (alglucosidase α)

Mastering methods to alleviate adverse reactions will help improve the quality of life of patients.

Pretreatment and infusion rate adjustment

To prevent or alleviate adverse reactions, patients can use antihistamines, antipyretics and/or corticosteroids for pretreatment before infusion. At the same time, the infusion rate can be adjusted in time according to the patient's reaction to reduce the incidence of adverse reactions.

Symptomatic treatment and observation

For adverse reactions that have occurred, patients should follow the doctor's advice for symptomatic treatment. If the allergic reaction is severe, the drug should be stopped immediately and medical attention should be sought. In addition, patients should receive close medical monitoring during the infusion process to detect and treat adverse reactions in a timely manner.

Methods to alleviate the adverse reactions of NEXVIAZYME (alglucosidase alfa) include pretreatment, infusion rate adjustment, symptomatic treatment and observation. Patients should actively cooperate with doctors in treatment in order to reduce adverse reactions and improve treatment effects.

Note: For internal discussion among medical personnel only. For specific medication, please consult the attending physician. Drug information may change over time. For the latest information, we recommend adding a medical consultant or consulting for free online.
Avalglucosidase alfa(Nexviazyme)
Avalglucosidase alfa(Nexviazyme)
Treatment of late-onset Pompe disease in patients aged 1 year and older.
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