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alglucerase (Ceredase)
Glucosylceramidase (beta-glucocerebrosidase).
Indication: 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:
1) individualize dose
2) 2.5 units/kg 3 times/week to 60 units/kg weekly to every 4th week
3) 60 units/kg every 2 weeks is the most common dose
4) dilute total dose to a final volume not exceeding 200 mL with normal saline
5) infuse over 1-2 hours
Injection: 80 units/mL. 5 mL.
Stability:
1) when diluted to 100-200 mL, stable up to 18 hours at 2-8 degrees C.
2) use promptly after reconstitution
3) store unopened vials at 2-8 degrees C
Adverse effects:
1) common (> 10%)
- discomfort, swelling & burning at injection site
2) 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
3) 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
Related
Gaucher's disease
Specific
Alglucerase Parenteral
General
glucosylceramidase; lysosomal acid glucosylceramidase; acidic beta glucosidase; beta-glucocerebrosidase (GBA1, GBA, GC, GLUC)
metabolic agent (metabolic modifier)
Properties
SIZE: entity length = 536 aa
MW = 60 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-39}
MOTIF: signal sequence {21-39}
cysteine residue {C43}
MODIFICATION: cysteine residue {C55}
cysteine residue {C55}
MODIFICATION: cysteine residue {C43}
cysteine residue {C57}
MODIFICATION: cysteine residue {C62}
N-glycosylation site {N58}
cysteine residue {C62}
MODIFICATION: cysteine residue {C57}
N-glycosylation site {N98}
N-glycosylation site {N185}
glutamate residue {E274}
N-glycosylation site {N309}
glutamate residue {E379}
N-glycosylation site {N501}
References
Kaiser Permanente Northern California Regional Drug
Formulary, 1998