Imiglucerase

Overview

Imiglucerase is a medication used in the treatment of Gaucher's disease.[1][2] WP

It is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase. Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Corporation. It is given intravenously after reconstitution as a treatment for Type 1 and Type 3 Gaucher's disease. It is available in formulations containing 200 or 400 units per vial. The specific activity of highly purified human enzyme is 890,000 units/mg, meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg. A typical dose is 2.5U/kg every two weeks, up to a maximum of 60 U/kg once every two weeks, and safety has been established from ages 2 and up. It is one of more expensive medications, with an annual cost of $200,000 per person in the United States. Imiglucerase has been granted orphan drug status in the United States, Australia, and Japan.[3–8] WP

Cerezyme was one of the drugs manufactured at Genzyme's Allston, Massachusetts plant, for which production was disrupted in 2009 after contamination with Vesivirus 2017.[9] WP

Risks and side effects

Side effects

The most common side effect is hypersensitivity, which occurs in about 3% of patients. It is associated with symptoms such as cough, shortness of breath, rashes, itching, and angiooedema. Less common side effects include dizziness, headache, nausea, diarrhea, and reactions at the injection site; they are found in less than 1% of patients.[3] WP

Interactions

Interactions

No clinical interaction studies have been conducted. Miglustat appears to increase the clearance of imiglucerase by 70%, resulting in decreased enzyme activity.[3][10] WP

Chemistry

Harm reduction information

No harm reduction data is on file for this compound yet. That means no dose, timing or combination data from PsychonautWiki or TripSit. Please don't guess a dose from similar compounds. If you have taken it and feel unwell, contact emergency services or a poison control center.

User reports

Pop culture

No references added yet.

Sources

1. Journal / agency Weinreb NJ. Imiglucerase and its use for the treatment of Gaucher's disease. Expert Opinion on Pharmacotherapy. August 2008. doi:10.1517/14656566.9.11.1987 · PMID 18627336
2. Journal / agency Starzyk K, Richards S, Yee J, Smith SE, Kingma W. The long-term international safety experience of imiglucerase therapy for Gaucher disease. Molecular Genetics and Metabolism. February 2007. doi:10.1016/j.ymgme.2006.09.003 · PMID 17079176
3. Other Austria-Codex. 2018
4. Journal / agency Pentchev PG, Brady RO, Blair HE, Britton DE, Sorrell SH. Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue. Proceedings of the National Academy of Sciences of the United States of America. August 1978. doi:10.1073/pnas.75.8.3970 · PMID 29293 · PMC392911
5. Other Cerezyme Powder for concentrate for solution for infusion, Annex I: Summary of Product Characteristics
6. Other Cerezyme (imiglucerase for injection)
7. Journal / agency Engelberg AB, Kesselheim AS, Avorn J. Balancing innovation, access, and profits--market exclusivity for biologics. The New England Journal of Medicine. November 2009. doi:10.1056/NEJMp0908496 · PMID 19828525
8. Other Imiglucerase
9. Journal / agency Ailworth E, Weisman R. Virus shuts Genzyme plant, holds up drugs for 8,000. The Boston Globe. June 17, 2009
10. Other Drug interactions between imiglucerase and miglustat

Text and values on this page are quoted from Wikipedia (CC BY-SA 4.0). Each value links to the exact revision it came from.