Pompe disease

【Product name】Myozyme, REMINYL, RAZADYNE

[English common name]Alglucosidase alfa

[Chinese common name]A-glucosidase

[English name]

[Chinese other name]

[Indications] LUMIZYME (alglucosidase alfa) is a lysosomal glycogen-specific enzyme for Pompe disease (acid alpha-glucosidase (GAA) deficiency) with advanced (non-infant) disease at age 8 years and older ) but there is no evidence of cardiac hypertrophy. The safety and efficacy of LUMIZYME has not been evaluated in controlled clinical trials in infant-infected patients or in advanced (non-infant) patients under 8 years of age.

[Usage and Dosage] The recommended dose of LUMIZYME is 20 mg / kg body weight every 2 weeks for intravenous infusion.
LUMIZYME is supplied as sterile, pyrogen free, white to white, lyophilized cake or powder for sterile water for injection, USP, to produce a concentration of 5 mg / mL; then further diluted with 0.9% sodium chloride injection USP is used for intravenous infusion. Disposable vials are only available in 50 mg doses.

[Storage] Store LUMIZYME at a refrigerating temperature of 2° to 8°C (36° to 46°F). Do not use LUMIZYME after the expiration date on the vial. The reconstituted and diluted solution should be administered immediately. If not immediately available, the reconstituted and diluted solution can be stable for up to 24 hours at 2° to 8°C (36° to 46°F). It is not recommended to store the reconstituted solution at room temperature. The reconstituted and diluted LUMIZYME solution should be protected from light. Do not freeze or shake.

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