Aglucerase
Synonym :
Glucocerebrosidase Enzyme
Mechanism :
Alglucerase catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide as part of the normal degradation pathway for membrane lipids. Glucocerebroside is primarily derived from hematologic cell turnover. Gaucher disease is characterized by a functional deficiency in b-glucocerebrosidase enzymatic activity and the resultant accumulation of lipid glucocerebroside in tissue macrophages which become engorged and are termed Gaucher cells.
Indication :
Contraindications :
There are no known contraindications.
Dosing :
IV infusion over 1-2 hours: 2.5 units/kg of body weight 3 times a week. Can increase to 60 units/kg once in 1-4 weeks. 60 units/kg every 2 weeks is the recommended regime.
Adverse Effect :
There are no known contraindications.
Pruritis, burning and swelling or sterile abscess at the site of venepuncture. Fever, chills, abdominal discomfort, nausea, vomiting, hypersensitivity like urticaria/angioedema, chest discomfort, hypotension.
Hepatic Dose :
No dosage adjustments are recommended.