alglucerase (Ceredase)

Introduction

Glucosylceramidase (beta-glucocerebrosidase).

Indications

long-term replacement therapy for type 1 Gaucher disease

Contraindications

• use with caution in males < 10 years of age (precocious puberty), androgen-sensitive malignancy & in patients with known sensitivity to chorionic gonadotropin

Dosage

• individualize dose
• 2.5 units/kg 3 times/week to 60 units/kg weekly to every 4th week
• 60 units/kg every 2 weeks is the most common dose
• dilute total dose to a final volume not exceeding 200 mL with normal saline
• infuse over 1-2 hours

Injection: 80 units/mL. 5 mL.

Stability:

• when diluted to 100-200 mL, stable up to 18 hours at 2-8 degrees C.
• use promptly after reconstitution
• store unopened vials at 2-8 degrees C

Adverse effects

• common (> 10%)
  • discomfort, swelling & burning at injection site
• uncommon (< 1%)
  • nausea/vomiting, diarrhea, abdominal discomfort, oral ulceration, fever/chills, menstrual irregularities, pruritus, flushing, urticaria, angioedema, chest discomfort, respiratory symptoms, sterile abscess at the site of administration
• other
  • vasomotor irritablity or hot flashes, backache, weakness, transient peripheral edema
  • patients may develop antibodies most likely within the 1st 6 months of therapy
• patients with antibodies to alglucerase are at higher risk of hypersensitivity reactions

Test interactions

may cause false pregnancy test

Notes

• purified from a pool of human placental tissue
• algucerase may contain chorionic gonadotropin

More general terms

• glucosylceramidase
• metabolic agent (metabolic modifier)

Additional terms

• Gaucher's disease