Trade Names:Ceredase- Solution for injection 80 units/mL
Catalyzes the hydrolysis of glucocerebroside to glucose and ceramide as part of the normal degradation pathway for membrane lipids.
Steady-state enzymatic activity and AUC of the activity increased linearly with the infused dose.
Vd ranged from 49.4 to 282.1 mL/kg.
Plasma Cl, based on plasma enzymatic activity, ranged from 6.34 to 25.39 mL/min/kg.
Following IV infusion over a 4-h period, steady-state enzymatic activity is achieved by 60 min.
Following infusion termination, plasma enzymatic activity declined rapidly with an elimination t ½ ranging from 3.6 to 10.4 min.
Long-term replacement therapy for patients with confirmed diagnosis of type 1 Gaucher disease who exhibit signs and symptoms severe enough to result in moderate to severe anemia, thrombocytopenia with bleeding tendency, bone disease, and/or hepatomegaly or splenomegaly.
Orphan drug status for replacement therapy in types 2 and 3 Gaucher disease.
IV Infuse dose over 1 to 2 h. Individualize dosage. Initial dosage may be as little as 2.5 units/kg 3 times a week up to 60 units/kg as frequently as once a week or as infrequently as every 4 wk. After response is well established, a reduction in dosage may be attempted for maintenance therapy. Progressive reductions can be made at intervals of 3 to 6 mo while carefully monitoring response parameters.
Store at 36° to 46°F. When diluted to 100 to 200 mL, product is stable for up to 18 h when stored at 36° to 46°F.
None well documented.
False-positive pregnancy test.
Hypotension, vasomotor irritability or hot flash.
Fatigue, headache, weakness.
Impaired sense of smell.
Diarrhea, nausea, oral ulcers, vomiting.
Burning and swelling or sterile abscess at venipuncture site, discomfort, pruritus.
Abdominal discomfort, chills, hypersensitivity (abdominal cramping, chest discomfort, flushing, nausea, pruritus, respiratory symptoms, urticaria/angioedema with occasional upper airway involvement), slight fever, transient peripheral edema.
Category C .
May be excreted in breast milk.
Safety and efficacy not established in children younger than 2 yr of age.
Use with caution in patients exhibiting symptoms of hypersensitivity; pretreatment with antihistamines has allowed continued use in some patients.
Use with caution in patients with androgen-sensitive malignancies.
Approximately 13% of patients may develop immunoglobulin G antibody during first yr of therapy, of which 25% may experience symptoms of hypersensitivity.
Because alglucerase is prepared from pooled human placental tissue, there is a risk of transmission of infectious agents (eg, viruses), including Creutzfeldt-Jakob disease.
No obvious toxicity detected after doses up to 234 units/kg.
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