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Pronunciation:

(al GLOO ser ase)

U.S. Brand Names:

Ceredase®

Synonyms:

Glucocerebrosidase

Generic Available:

Use:

Replacement therapy for Gaucher's disease (type 1)

Pregnancy Risk Factor:

Pregnancy Implications:

Animal studies have not been conducted.

Lactation:

Excretion in breast milk unknown/use caution

Contraindications:

Hypersensitivity to any component of the formulation

Warnings/Precautions:

Prepared from pooled human placental tissue that may contain the causative agents of some viral diseases. Patients who develop IgG antibodies may be at a higher risk for developing hypersensitivity. Use caution with androgen sensitive malignancies or prior allergies to hCG. May cause early virilization in males <10 years of age.

Adverse Reactions:

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%)

Overdosage/Toxicology:

No obvious toxicity has been detected after single doses up to 234 units/kg.

Stability:

Refrigerate (4°C), do not freeze. Contains no preservatives. Do not store opened vials for future use. Dilute with NS to a final volume 200 mL. Do not shake. Once diluted, 100 mL and 200 mL solutions for infusion are stable for 18 hours when stored at 2°C to 8°C.

Compatibility:

Compatible: Stable in NS

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

Dosage:

I.V.: Children and Adults: 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 1-4 times/week. Once patient response is well established, dose may be reduced every 3-6 months to determine maintenance therapy.

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

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

Test Interactions:

False positive pregnancy tests

Patient Education:

Inform prescriber of all prescriptions, OTC medications, or herbal products you are taking, and any allergies you have. Do not take any new medication during therapy unless approved by prescriber. This medication will not cure Gaucher's disease, but rather, may help control it. Treatment is required for life. May cause abdominal discomfort, nausea, or vomiting (small, frequent meals, good mouth care, chewing gum, or sucking lozenges may help); these symptoms should go away with continued use. Inform prescriber if pain, swelling, or redness occurs at injection site or if GI symptoms persist. Pregnancy/breast-feeding precautions: Inform prescriber if you are or intend to become pregnant. Consult prescriber if breast-feeding.

Dental Health: Effects on Dental Treatment:

No significant effects or complications reported

Dental Health: Vasoconstrictor/Local Anesthetic Precautions:

No information available to require special precautions

Mental Health: Effects on Mental Status:

None reported

Mental Health: Effects on Psychiatric Treatment:

None reported

Dosage Forms:

Injection, solution [preservative free]: 10 units/mL (5 mL); 80 units/mL (5 mL) [contains human albumin 1%]

International Brand Names:

Ceredase® (IL)

References

Barton NW, Brady RO, Dambrosia JM, et al, "Replacement Therapy for Inherited Enzyme Deficiency - Macrophage-Targeted Glucocerebrosidase for Gaucher's Disease,"N Engl J Med, 1991, 324(21):1464-70.

Charrow J, Andersson HC, Kaplan P, et al, "Enzyme Replacement Therapy and Monitoring for Children With Type 1 Gaucher Disease: Consensus Recommendations,"J Pediatr, 2004, 144(1):112-20.

Whittington R and Goa KL, "Alglucerase: A Review of Its Therapeutic Use in Gaucher's Disease,"Drugs, 1992, 44(1):72-93.

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