Imiglucerase

Imiglucerase is a medication used for the treatment of Gaucher disease, a rare genetic disorder characterized by the deficiency of an enzyme called glucocerebrosidase. This enzyme is responsible for breaking down a fatty substance called glucocerebroside. In Gaucher disease, the accumulation of glucocerebroside in certain organs can lead to various complications. -Miglucerase is a recombinant form of human glucocerebrosidase, produced through genetic engineering techniques. It is administered intravenously and works by replacing the deficient enzyme in individuals with Gaucher disease, helping to break down and eliminate the accumulated glucocerebroside. -Imiglucerase is a recombinant form of human glucocerebrosidase, produced through genetic engineering techniques. It is administered intravenously and works by replacing the deficient enzyme in individuals with Gaucher disease, helping to break down and eliminate the accumulated glucocerebroside. -It is analog of beta-glucocerebrosidase, lysosomal enzyme replacement that plays a role in the hydrolysis of glucocerebroside to glucose and ceramide.

Indications

-Imiglucerase is indicated for the long-term treatment of Type 1 Gaucher disease in pediatric and adult patients. - Indicated for type 1 Gaucher's disease; associated with one or more of the following: anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly

Dosage

-The dosage of imiglucerase is individualized based on factors such as the patient's weight and response to treatment. It is typically administered by intravenous infusion, and the frequency and dose will be determined by the healthcare provider. -IV Infusion; 200 and 400 unit; Children >2 years: 2.5 units/kg IV (1-2 hours infusion) 3 times per week OR 30-60 unit/kg IV 2Weeks initially May initiate at a higher dose/frequency based on disease severity Up to 240 unit/kg every 2 Weeks has been safely used Adults: 2.5 units/kg IV infused over 1-2 hr 3 times per week OR 30-60 unit/kg IV 2 Weeks initially May initiate at a higher dose/frequency based on disease severity Up to 240 unit/kg 2 Weeks has been safely used

Contra-Indications

Imiglucerase is contraindicated in individuals with a known hypersensitivity to the drug or any of its components.

Special Precautions

-Patients receiving imiglucerase should be monitored for signs of hypersensitivity reactions during and after administration. Caution is advised in patients with compromised cardiac or pulmonary function. -Paediatric: It has been administered to patients younger than 2 years of age, however the safety and effectiveness in patients younger than 2 have not been established. Pregnancy: Category C; It should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus. Lactation: Not known if distributed into breast milk, use caution Elderly: Data not sufficient

Side Effects

-Common side effects may include infusion reactions (fever, chills, headache, rash), fatigue, dizziness, and abdominal pain. Serious side effects are rare but may include anaphylaxis, severe hypersensitivity reactions, and respiratory distress. -Hypersensitivity with infusion, Nausea, abdominal pain, vomigin, diarrhea, Rash, Fatigue, Headache, Fever, Dizziness, Chills, Backache, Tachycardia, Pulmonary hypertension, Pneumonia.

Drug Interactions

There are no significant drug interactions reported with imiglucerase. However, it is important for patients to inform their healthcare providers about all medications, including over-the-counter drugs and herbal supplements, they are taking.