Imiglucerase for Injection(Cerezyme)
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Cerezyme (imiglucerase) is a recombinant, hydrolytic lysosomal
glucocerebrosidase enzyme designed for intravenous administration. It serves as
an enzyme replacement therapy, compensating for the deficient or defective
β-glucocerebrosidase activity characteristic of Type 1 Gaucher disease. The drug
is produced using recombinant DNA technology in Chinese hamster ovary cells,
resulting in a glycoprotein structurally analogous to the human enzyme, with
modifications to enhance cellular uptake.
Upon administration, Cerezyme catalyzes the hydrolysis of accumulated
glucocerebroside into glucose and ceramide, thereby reducing pathological
substrate storage in affected tissues such as the liver, spleen, and bone
marrow. This targeted mechanism addresses the underlying metabolic defect,
leading to clinical improvements in hematologic parameters, organomegaly, and
skeletal involvement. The preparation is supplied as a lyophilized powder for
reconstitution and must be administered under the supervision of healthcare
professionals experienced in managing hypersensitivity reactions, including
anaphylaxis.
Generic name
Imiglucerase for Injection(Cerezyme)
English name
Imiglucerase for Injection
Alternative Names
Cerezyme
Indications
Cerezyme is indicated for the treatment of adults and pediatric patients 2 years of age and older with Type 1 Gaucher disease who present with one or more of the following: anemia, thrombocytopenia, bone disease, hepatomegaly, or splenomegaly.
Therapeutic Target
The therapeutic target is the lysosomal enzyme β-glucocerebrosidase, which is deficient or defective in Type 1 Gaucher disease.
Active Ingredients
Imiglucerase
specifications
Single-dose vial containing 400 units of imiglucerase as a white to off-white lyophilized powder for reconstitution.
Description
Imiglucerase is a hydrolytic lysosomal glucocerebrosidase-specific enzyme
produced by recombinant DNA technology in Chinese hamster ovary cells. It is a
monomeric glycoprotein of 497 amino acids, with four N-linked glycosylation
sites, and is structurally analogous to the human enzyme, differing by a single
amino acid at position 495. The oligosaccharide chains are modified to terminate
in mannose sugars, facilitating cellular uptake.
Dosage and Administration
Dosage is individualized based on disease severity, ranging from 2.5 units/kg
three times a week to 60 units/kg once every two weeks.
The drug is administered by intravenous infusion over 1–2 hours for patients
≥18 kg, and over 2 hours for those <18 kg.
Reconstitute each 400-unit vial with 10.2 mL Sterile Water for Injection,
yielding 40 units/mL. Dilute promptly with 0.9% Sodium Chloride Injection to a
final volume of 100–200 mL (100 mL for patients <18 kg).
Administration should be supervised by healthcare professionals experienced
in managing hypersensitivity reactions.
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