Included in the formulation
АТХ:A.16.A.B.08 Galsulfase
Pharmacodynamics:Recombinant human N-acetylgalactosamine-4-sulfatase, an agent for enzyme replacement therapy.
As an exogenous enzyme due to the binding of the oligosaccharide chain, it penetrates into the lysosomes and enhances the catabolism of glycosaminoglycan.
Pharmacokinetics:After intravenous administration at a dose of 1 μg / kg once a week, the maximum concentration in the blood plasma is 0.8 μg / ml.
The elimination half-life is 9 minutes.
Indications:It is used in the complex therapy of mucopolysaccharidosis type VI in the Maroto-Lamy syndrome.
IV.E70-E90.E76 Disorders of glycosaminoglycan metabolism
Contraindications:Individual intolerance.
Carefully:Pregnancy and lactation.
Pregnancy and lactation:Recommendations for FDA - Category C. It is used in pregnancy and lactation in cases where the expected effect exceeds the risk for the fetus and infant.
Dosing and Administration:Intravenous by 1 mg / kg once a week.
The highest daily dose: 1 mg / kg.
The highest single dose: 1 mg / kg.
Side effects:Central and peripheral nervous system: headache.
Respiratory system: cough.
Digestive system: diarrhea, abdominal pain.
Musculoskeletal system: arthralgia.
Sense organs: otitis media.
Allergic reactions.
Overdose:During the infusion of bronchospasm, apnea, respiratory distress, hives on the face.
Treatment is symptomatic, discontinuation of infusion.
Interaction:Clinically significant interactions are not described.
Special instructions:Before the infusion of galsulfase, it is necessary to perform a premedication with antihistamines, the action of which increases the risk of sleep apnea in patients with type VI mucopolysaccharidosis.