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Cerezyme (imiglucerase) is a recombinant enzyme therapy used to treat Gaucher disease, a rare lysosomal storage disorder caused by β-glucocerebrosidase deficiency. By replacing the missing enzyme, Cerezyme facilitates the breakdown of accumulated glucocerebroside, alleviating symptoms such as hepatosplenomegaly, anemia, and bone abnormalities.
This medication is administered through intravenous infusion and is tailored to individual patient needs. It has demonstrated efficacy in improving hematological and visceral symptoms, providing a crucial therapeutic option for managing the disease long-term. Due to its specialized nature, Cerezyme is typically used under the supervision of healthcare professionals in hospital or clinical settings.
Imiglucerase for Injection is indicated for long-term enzyme replacement therapy in patients diagnosed with non-neuropathic (Type I) and chronic neuropathic (Type II) Gaucher disease who have significant clinical manifestations of non-neurological lesions.
The clinical manifestations of non-neurological lesions in Gaucher disease include one or more of the following conditions:
1. Anemia excluding that caused by other reasons, such as iron deficiency anemia.
2. Thrombocytopenia.
3. Bone disease excluding that caused by other reasons, such as vitamin D deficiency.
4. Hepatomegaly or splenomegaly.
Dosing
Range: 2.5 units/kg thrice weekly to 60 units/kg every 2 weeks.
Titration: Adjust based on disease severity and therapeutic goals.
Administration Protocol
Reconstitution:
Add 10.2 mL Sterile Water to each 400-unit vial; roll/tilt to dissolve (avoid shaking).
Final concentration: 40 units/mL.
Dilution:
Dilute with 0.9% NaCl to 100–200 mL (100 mL for patients <18 kg).
Infusion:
≥18 kg: 1–2 hours.
<18 kg: 2 hours.
Filtration: Use in-line 0.2 μm filter if needed.
Premedication
For hypersensitivity-prone patients: Antihistamines/corticosteroids.
