Mucopolysaccharidosis VI

Active Ingredient: Galsulfase

Indication for Galsulfase

Population group: only infants (40 days - 1 year old) , children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 years old or older)

Galsulfase is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Mucopolysaccharidosis VI (MPS VI; N-acetylgalactosamine 4-sulfatase deficiency; Maroteaux-Lamy syndrome)

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

1 mg/kg once every week

Route of admnistration

Intravenous

Defined daily dose

1 - 1 mg per kg of body weight

Dosage regimen

From 1 To 1 mg per kg of body weight once every 7 day(s)

Detailed description

The recommended dose regimen for galsulfase is 1 mg/kg body weight administered once every week as an intravenous infusion over 4 hours.

Dosage considerations

The initial infusion rate is adjusted so that approximately 2.5% of the total solution is infused during the first hour, with infusion of the remaining volume (approximately 97.5%) over the next 3 hours.

Active ingredient

Galsulfase

Purified galsulfase, a recombinant form of human N-acetylgalactosamine 4-sulfatase, is a glycoprotein with a molecular weight of approximately 56 kD. Galsulfase is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Mucopolysaccharidosis VI (MPS VI; N-acetylgalactosamine 4-sulfatase deficiency; Maroteaux-Lamy syndrome).

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