Imiglucerase: Difference between revisions

Revision as of 01:59, 11 February 2025

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.

Imiglucerase (Cerezyme) is used for treatment of adults and pediatric patients 2 years of age and older with Type 1 Gaucher disease that results in one or more of the following conditions:
anemia
thrombocytopenia
bone disease
hepatomegaly or splenomegaly

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.

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

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.

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:

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

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

back pain, chills, dizziness, fatigue, headache, hypersensitivity reactions, nausea, pyrexia, and vomiting.

less common side effects, may include:

Nervous system disorders: dizziness, headache
Cardiac disorders: tachycardia
Vascular disorders: cyanosis, flushing, hypotension
Respiratory, thoracic and mediastinal disorders: cough, dyspnea, pneumonia, pulmonary hypertension
Gastrointestinal disorders: abdominal pain, diarrhea, nausea, vomiting
Immune system disorders: anaphylaxis, hypersensitivity
Skin and subcutaneous tissue disorders: angioedema, pruritus, rash, urticaria
Musculoskeletal and connective tissue disorders: back pain
General disorders and administration site conditions:chest discomfort, chills, fatigue, infusion-site burning, infusion-site discomfort, infusion-site swelling, pyrexia

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.

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

Overdose related information is also available online at poisonhelp.org/help.
In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

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.