Imiglucerase
Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C. 3.2.1.45. 497 residue protein with N-linked carbohydrates, MW=59.3 kD. Imiglucerase is prepared by modification of the oligosaccharide chains of human Beta-glucocerebrosidase. The modification alters the sugar residues at the non-reducing ends of the oligosaccharide chains of the glycoprotein so that they are predominantly terminated with mannose residues
Mechanism of effect
Imiglucerase catalyzes the hydrolysis of the glycolipid, glucocerebroside, to glucose and ceramide as part of the normal degradation pathway for membrane lipids.
Pharmacodynamic
Gaucher disease is characterized by a functional deficiency in Beta-glucocerebrosidase enzymatic activity and the resultant accumulation of lipid glucocerebroside in tissue macrophages which become engorged and are termed Gaucher cells.
Gaucher cells are typically found in liver, spleen and bone marrow. This can lead to an enlarged spleen and liver (hepatosplenomegaly). Secondary hematologic sequelae include severe anemia and thrombocytopenia. Injections of imiglucerase into Gaucher disease patients leads to elevated serum levels of the enzyme and reduction in the accumulation of glucocerebroside leading to reduced anemia and thrombocytopenia, reduced spleen and liver size, and decreased cachexia
Pharmacokinetics
Volume of distribution:0.09 to 0.15 L/kg
Half life:3.6-10.4 min
Clearance:14.5 +/- 4.0 mL/min/kg
Dosage
Gaucher Disease
Adult
Indicated for type 1 Gaucher's disease; associated with one or more of the following: anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly
2.5 units/kg IV infused over 1-2 hr 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Up to 240 unit/kg q2Weeks has been safely used
Pediatric
>2 years: 2.5 units/kg IV (1-2 hours infusion) 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Up to 240 unit/kg q2Weeks has been safely used
Side effects
Diarrhea , Headache , nausea , dizziness , vomiting , flushing , fever , itching , tiredness , Abdominal pain , RashInteractions
MiglustatPoints of recommendation
Have blood work checked as you have been told by the doctor. Talk with the doctor.
Allergic effects that may or may not be unsafe may happen during and after the infusion. Talk with the doctor.
Other drugs may be given before this medicine to help avoid side effects.
Tell your doctor if you are pregnant or plan on getting pregnant. You will need to talk about the benefits and risks of using imiglucerase while you are pregnant.
Tell your doctor if you are breast-feeding. You will need to talk about any risks to your baby.ش
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