VPRIV

Nchi: Australia

Lugha: Kiingereza

Chanzo: Department of Health (Therapeutic Goods Administration)

Viambatanisho vya kazi:

Velaglucerase alfa

Inapatikana kutoka:

Shire Australia Pty Ltd

Darasa:

Medicine Registered

Taarifa za kipeperushi

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VPRIV
®

(VELAGLUCERASE ALFA GHU) 
 POWDER FOR SOLUTION FOR INFUSION 

CONSUMER MEDICINE INFORMATION 

WHAT IS IN THIS LEAFLET 

This leaflet answers some common 
questions about VPRIV.  It does not 
contain all of the available information.  
Reading this leaflet does not take the 
place of talking to your doctor or 
pharmacist.   

Please read this leaflet before you 
start taking VPRIV. 

All medicines have risks and benefits.  
Your doctor has weighed the possible 
risks of taking VPRIV against the 
expected benefits. 

If you have any concerns about taking 
VPRIV, ask your doctor or pharmacist. 

Keep this leaflet.  You may want to 
read it again. 

WHAT IS VPRIV USED FOR 

VPRIV is used to treat Type 1 
Gaucher disease.  Gaucher disease is 
a genetic disorder caused by a 
missing or defective enzyme called 
glucocerebrosidase.  When this 
enzyme is missing or does not work 
properly, a substance called 
glucocerebroside builds up inside cells 
in the body.  VPRIV is used to replace 
the missing or defective enzyme, 
glucocerebrosidase, in patients with 
Gaucher disease.  This type of 
treatment is called enzyme 
replacement therapy (ERT). 

Your doctor may have prescribed 
VPRIV for another use.  Ask your 
doctor if you have any questions about 
why VPRIV has been prescribed for 
you. 

VPRIV is not addictive. 

VPRIV is not expected to affect your 
ability to drive a car or operate 
machinery. 

VPRIV is only available on a doctor’s 
prescription. 

BEFORE YOU TAKE VPRIV 

_WHEN YOU MUST NOT USE IT _

Do not use VPRIV if: 

•  You are allergic 
(hypersensitive) to 
velaglucerase alfa or any of the 
other ingredients of VPRIV. 
•  The package is torn or shows 
signs of tampering. 
•  The expiry date (EXP) printed 
on the pack has passed. The 
expiration date refers to the�

Tabia za bidhaa

_Product Information                              

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 PRODUCT INFORMATION 

VPRIV
®
 (VELAGLUCERASE ALFA GHU) 

NAME OF THE MEDICINE 

VPRIV powder for solution for infusion. 

Active Ingredient:  velaglucerase alfa ghu 

CAS number: 37228-64-1 

DESCRIPTION 

Velaglucerase alfa ghu is a glycoprotein produced by gene-activation
technology in a 
human cell line.  The monomer is approximately 63 kDa, has 497 amino
acids and the 
same amino acid sequence as the naturally occurring human enzyme, 
glucocerebrosidase.  There are 5 potential N-linked glycosylation
sites, four of which are 
occupied.  Velaglucerase alfa ghu is manufactured to contain
predominantly high-
mannose-type glycans to facilitate internalisation of the enzyme
by the phagocytic target 
cells via the mannose receptor. 

VPRIV is supplied in 400 U/vial (10 mg) of velaglucerase alfa ghu.
 VPRIV is a sterile, 
preservative free lyophilised powder in single-use vials which
requires reconstitution and 
dilution, and is intended for intravenous infusion only.  VPRIV
contains the following 
excipients: sucrose, sodium citrate, citric acid monohydrate and
polysorbate 20. 

PHARMACOLOGY 

GENERAL 
Gaucher disease is an autosomal recessive disorder caused by
mutations in the GBA 
gene which results in a deficiency of the lysosomal enzyme,
beta-glucocerbrosidase.  
This enzymatic deficiency causes an accumulation of glucocerebroside
primarily in 
macrophages, giving rise to foam cells or “Gaucher cells”.  In
this lysosomal storage 
disorder, clinical features are reflective of the distribution of
Gaucher cells in the liver, 
spleen, bone marrow, skeleton, and lungs.  The accumulation of
glucocerbroside in the 
liver and spleen leads to organomegaly.  Bone involvement res