Alglucerase glucocerebrosidase

U.S. BRAND NAMES — Ceredase®
PHARMACOLOGIC CATEGORY Enzyme
DOSING: ADULTS — Gaucher's disease: I.V.: Initial: 30-60 units/kg every 2 weeks; dosing is individualized based on disease severity; average dose: 60 units/kg every 2 weeks. Range: 2.5 units/kg 3 times/week to 60 units/kg once weekly to every 4 weeks. Once patient response is well established, dose may be reduced every 3-6 months to determine maintenance therapy.
DOSING: PEDIATRIC — Refer to adult dosing.
(For additional information see "Alglucerase (glucocerebrosidase): Pediatric drug information")
DOSING: ELDERLY — Refer to adult dosing.
DOSAGE FORMS — Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Injection, solution [preservative free]: Ceredase®: 80 units/mL (5 mL) [contains human albumin 1%]
DOSAGE FORMS: CONCISE Injection, solution [preservative free]: Ceredase®: 80 units/mL (5 mL)
GENERIC EQUIVALENT AVAILABLE — No
ADMINISTRATION — I.V.: Infuse I.V. over 1-2 hours. Use of an in-line filter is recommended. Do not shake solution as it denatures the enzyme.
COMPATIBILITY — Stable in NS; do not mix with any other additives.
USE — Replacement therapy for Gaucher's disease (type 1)
ADVERSE REACTIONS SIGNIFICANT — Frequency not defined.
Cardiovascular: Peripheral edema
Central nervous system: Chills, fatigue, fever, headache, lightheadedness
Endocrine & metabolic: Hot flashes, menstrual abnormalities
Gastrointestinal: Abdominal discomfort, diarrhea, nausea, oral ulcerations, vomiting
Local: Injection site: Abscess, burning, discomfort, pruritus, swelling
Neuromuscular & skeletal: Backache, weakness
Miscellaneous: Dysosmia; hypersensitivity reactions (abdominal cramping, angioedema, chest discomfort, flushing, hypotension, nausea, pruritus, respiratory symptoms, urticaria); IgG antibody formation (~13%)
CONTRAINDICATIONS — Hypersensitivity to any component of the formulation
WARNINGS / PRECAUTIONS Concerns related to adverse effects: Hypersensitivity reactions: Patients who develop IgG antibodies may be at a higher risk for developing hypersensitivity. Use with caution in patients with prior allergies to hCG.
Disease-related concerns: Androgen-sensitive malignancies: Use with caution in patients with androgen-sensitive malignancies.
Special populations: Pediatrics: Safety and efficacy have not been established in children <2 years of age. May cause early virilization in males <10 years of age.
Other warnings/precautions: Placental tissue: Prepared from pooled human placental tissue that may contain the causative agents of some viral diseases.
PREGNANCY RISK FACTOR — C (show table)
PREGNANCY IMPLICATIONS — Animal studies have not been conducted.
LACTATION — Excretion in breast milk unknown/use caution
MONITORING PARAMETERS — CBC, platelets, liver function tests, IgG antibody formation, acid phosphatase (AP); MRI or CT of liver and spleen, skeletal x-rays, physical exam every 6-12 months
TOXICOLOGY / OVERDOSE COMPREHENSIVE — No obvious toxicity was detected after single doses of up to 234 units/kg.
MECHANISM OF ACTION — Alglucerase is a modified form of glucocerebrosidase; it is prepared from human placental tissue. Glucocerebrosidase is an enzyme deficient in Gaucher's disease. It is needed to catalyze the hydrolysis of glucocerebroside to glucose and ceramide.
PHARMACODYNAMICS / KINETICS — Half-life elimination: ~3-11 minutes