Imiglucerase

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What is Imiglucerase?[edit | edit source]

  • Imiglucerase (Cerezyme) is a hydrolytic lysosomal glucocerebrosidase-specific enzyme used for therapy of type 1 Gaucher disease.
  • Imiglucerase is prepared by recombinant techniques using Chinese hamster ovary cells.
  • It differs from the native enzyme in one amino acid (histidine at position 495 instead of arginine) and by modification of the glycosylation sites so that they terminate in mannose sugars, which are specifically recognized and taken up by macrophages.



What are the uses of this medicine?[edit | edit source]


How does this medicine work?[edit | edit source]

  • Gaucher disease is characterized by a deficiency of β-glucocerebrosidase activity, which results in accumulation of glucocerebroside in various tissues including liver, spleen, and bone marrow.
  • The mannose sugars on imiglucerase mediate binding to and internalization by cells including macrophages.
  • Cerezyme catalyzes the hydrolysis of glucocerebroside to glucose and ceramide.


Who Should Not Use this medicine ?[edit | edit source]

  • This medicine have no usage limitations.


What drug interactions can this medicine cause?[edit | edit source]

  • Tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take.


Is this medicine FDA approved?[edit | edit source]

  • Imiglucerase was approved for use in the United States in 1994 and soon became the most widely used enzyme replacement therapy for Gaucher disease.
  • It is available as a lyophilized powder in vials of 200 and 400 Units.


How should this medicine be used?[edit | edit source]

  • For patients who experience hypersensitivity reactions to Cerezyme premedicate with and/or corticosteroids.
  • Monitor patients for the occurrence of new hypersensitivity reactions.

Recommended dosage:

  • The recommended dosage ranges from 2.5 units/kg three times a week to 60 units/kg once every two weeks.
  • For patients weighing 18 kg and greater, infuse the diluted Cerezyme solution over 1 to 2 hours.
  • For patients weighing less than 18 kg, infuse the diluted Cerezyme solution over 2 hours.
  • Titrate the dosage based on clinical manifestations of disease and therapeutic goals for the patient.

Administration:


What are the dosage forms and brand names of this medicine?[edit | edit source]

This medicine is available in fallowing doasage form:

  • As injection: 400 units of imiglucerase as a lyophilized powder in a single-dose vial.

This medicine is available in fallowing brand namesː

  • Cerezyme


What side effects can this medication cause?[edit | edit source]

The most common side effects of this medicine include: In adults:

  • In pediatric patients 2 years of age and older are similar to adults.

less common side effects, may include:


What special precautions should I follow?[edit | edit source]

  • Hypersensitivity reactions, some of which are serious and include anaphylaxis, have been reported. Consider periodic monitoring of patients during the first year of treatment for IgG antibody formation. If a severe hypersensitivity reaction occurs, discontinue Cerezyme treatment and initiate appropriate medical treatment.
  • Inform patients and caregivers that the Gaucher patient registry has been established in order to better understand the variability and progression of Gaucher disease and to continue to monitor and evaluate long-term treatment effects of Cerezyme.
  • Available published literature suggests a small amount of imiglucerase is present in breast milk immediately following an infusion of imiglucerase. Lactating women with Gaucher disease treated with Cerezyme should be encouraged to enroll in the Gaucher patient registry.


What to do in case of emergency/overdose?[edit | edit source]

  • In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.


Can this medicine be used in pregnancy?[edit | edit source]

  • Imiglucerase use in pregnant women have not identified a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes.
  • There are risks associated with symptomatic Type I Gaucher disease in pregnancy.
  • There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to Cerezyme during pregnancy.
  • Pregnant women exposed to Cerezyme and health care providers are encouraged to contact the Gaucher patient registry at 1-800-745-4447, extension 15500 or visit www.registrynxt.com.


Can this medicine be used in children?[edit | edit source]

  • The safety and effectiveness of Cerezyme have not been established in pediatric patients younger than 2 years of age.


What are the active and inactive ingredients in this medicine?[edit | edit source]

Active ingredient:

  • IMIGLUCERASE

Inactive ingredients:

  • MANNITOL
  • TRISODIUM CITRATE DIHYDRATE
  • DISODIUM HYDROGEN CITRATE
  • POLYSORBATE 80


Who manufactures and distributes this medicine?[edit | edit source]

Manufactured by:

  • Cerezyme is a registered trademark of Genzyme Corporation.


What should I know about storage and disposal of this medication?[edit | edit source]

  • Store refrigerated at 2°C to 8°C (36°F to 46°F).


genetic disorder agents[edit source]

cystic fibrosis agents

enzyme replacement therapy

glucosylceramide synthase inhibitors (substrate restriction therapy)

lysosomal acid lipase deficiency agents

miscellaneous

homocystinuria agents

Huntington disease agents

Monoclonal Antibodies

Tyrosinemia Agents

Urea Cycle Disorder Agents

Hematologic Agents

Imiglucerase Resources

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