Fabry Disease

Active Ingredient: Agalsidase beta

Indication for Agalsidase beta

Population group: only adults (18 - 65 years old)

Agalsidase beta is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease (α-galactosidase A deficiency). 2

For this indication, competent medicine agencies globally authorize below treatments:

1 mg/kg body weight once every 2 weeks

Route of admnistration

Intravenous

Defined daily dose

1 mg per kg of body weight

Dosage regimen

1 mg per kg of body weight once every 14 day(s)

Detailed description

The recommended dose of agalsidase beta is 1 mg/kg body weight administered once every 2 weeks as an intravenous infusion.

Dosage considerations

The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.

Active ingredient

Agalsidase beta

Agalsidase beta is a recombinant form of human α-galactosidase A and is produced by recombinant DNA technology using a mammalian Chinese Hamster Ovary (CHO) cell culture. The amino acid sequence of the recombinant form, as well as the nucleotide sequence which encoded it, are identical to the natural form of α-galactosidase.

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