Imiglucerase Explained

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

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[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,[4] meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg.[5] 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.[6] It is one of more expensive medications, with an annual cost of $200,000 per person in the United States.[7] Imiglucerase has been granted orphan drug status in the United States, Australia, and Japan.[8]

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]

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.

Interactions

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

See also

Notes and References

  1. Weinreb NJ . Imiglucerase and its use for the treatment of Gaucher's disease . Expert Opinion on Pharmacotherapy . 9 . 11 . 1987–2000 . August 2008 . 18627336 . 10.1517/14656566.9.11.1987 . 72183308 .
  2. 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 . 90 . 2 . 157–163 . February 2007 . 17079176 . 10.1016/j.ymgme.2006.09.003 .
  3. Book: Austria-Codex. Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung. Österreichischer Apothekerverlag. Vienna. 2018. de.
  4. 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 . 75 . 8 . 3970–3973 . August 1978 . 29293 . 392911 . 10.1073/pnas.75.8.3970 . free . 1978PNAS...75.3970P .
  5. Web site: Cerezyme Powder for concentrate for solution for infusion, Annex I: Summary of Product Characteristics . Sanofi-Aventis Latvia SIA . Ec.europa.eu. 26 April 2022.
  6. Web site: Cerezyme (imiglucerase for injection) . Genzyme product data sheet. dead. https://web.archive.org/web/20030605012113/http://www.cerezyme.com/global/pi.pdf. 2003-06-05.
  7. Engelberg AB, Kesselheim AS, Avorn J . Balancing innovation, access, and profits--market exclusivity for biologics . The New England Journal of Medicine . 361 . 20 . 1917–1919 . November 2009 . 19828525 . 10.1056/NEJMp0908496 .
  8. Web site: Imiglucerase . Orphanet . 26 April 2022.
  9. Virus shuts Genzyme plant, holds up drugs for 8,000. The Boston Globe. Ailworth E, Weisman R . June 17, 2009.
  10. Web site: Drug interactions between imiglucerase and miglustat . Drugs.com . 11 April 2019 .

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