"The National Institutes of Health has launched DS-Connect, a Web-based health registry that will serve as a national health resource for people with Down syndrome and their families, researchers, and health care providers.
(velaglucerase alfa for injection)
The active ingredient of VPRIV is velaglucerase alfa, which is produced by gene activation technology in a human fibroblast cell line. Velaglucerase alfa is a glycoprotein of 497 amino acids; with a molecular weight of approximately 63 kDa. Velaglucerase alfa has the same amino acid sequence as the naturally occurring human enzyme, glucocerebrosidase. Velaglucerase alfa contains 5 potential N-linked glycosylation sites; four of these sites are occupied by glycan chains. Velaglucerase alfa contains predominantly high mannose-type N-linked glycan chains. The high mannose type N-linked glycan chains are specifically recognized and internalized via the mannose receptor present on the surface on macrophages, the cells that accumulate glucocerebroside in Gaucher disease. Velaglucerase alfa catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide in the lysosome.
VPRIV is a hydrolytic lysosomal glucocerebroside-specific enzyme. VPRIV is dosed by Units/kg, where one Unit of enzyme activity is defined as the quantity of enzyme required to convert one micromole of p-nitrophenyl ß-D-glucopyranoside to p-nitrophenol per minute at 37°C.
VPRIV is supplied as a sterile, preservative free, lyophilized powder in single-use vials, each containing 400 Units, for intravenous use. Following reconstitution with Sterile Water for Injection, USP, the solution contains the components listed in Table 2.
Table 2: Composition of VPRIV Reconstituted Solution
|Extractable 400 Units/vial|
|velaglucerase alfa||400 Units|
|citric acid, monohydrate||5.04 mg|
|polysorbate 20||0.44 mg|
|sodium citrate, dihydrate||51.76 mg|
What are the possible side effects of velaglucerase alfa (VPRIV)?
Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.
Some people receiving a velaglucerase alfa injection have had a reaction to the infusion (when the medicine is injected into the vein). Most infusion reactions have been mild. However, tell your caregiver right away if you feel dizzy, nauseated, light-headed, sweaty, itchy, short of breath, or have a fast heartbeat, chest tightness, or trouble breathing during the injection.
Less serious side effects may...
What are the precautions when taking velaglucerase alfa for injection (VPRIV)?
Before using velaglucerase, tell your doctor or pharmacist if you are allergic to it; or to alglucerase; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.
Before using this medication, tell your doctor or pharmacist your medical history.
During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.
It is unknown if this medication passes into breast milk. Consult your doctor before...
Last reviewed on RxList: 5/21/2015
This monograph has been modified to include the generic and brand name in many instances.
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