CEREZYME

Երկիր: Ավստրալիա

Լեզու: անգլերեն

Աղբյուրը: Department of Health (Therapeutic Goods Administration)

Ակտիվ բաղադրիչ:

Imiglucerase

Հասանելի է:

Genzyme Australasia Pty Ltd

Դաս:

Medicine Registered

Ապրանքի հատկությունները

VERSION: CZM PI ANZ A1205-01 
SUPERCEDES: CZM ANZ A1003-01 
COMMERCIAL 
Page 1 of 10 
PRODUCT INFORMATION 
CEREZYME
®

NAME OF THE MEDICINE 
NON-PROPRIETARY NAME 
imiglucerase - rch powder for solution for infusion. 
DESCRIPTION 
CEREZYME is provided as a white to off - white sterile lyophilised
powder in a clear glass vial and 
contains a nominal value of 200 Units* or 400 Units of imiglucerase
and the excipients mannitol, 
sodium citrate dihydrate, citric acid monohydrate and polysorbate 80.
 The reconstituted solution must 
be diluted further. 

*An Enzyme Unit (U) is defined as the amount of enzyme that
catalyses the hydrolysis of one 
micromole of the synthetic substrate para-nitrophenyl  
-D-glucopyranoside (pnp-Glc) per minute at

37
C. 
It is recommended that the diluted solution be filtered through an in
- line low protein - binding 0.2μ 
filter during administration. 
PHARMACOLOGY 
PHARMACODYNAMICS  
Imiglucerase is a recombinant, macrophage - targeted, variant of
human 
 - glucocerebrosidase, 
purified from Chinese Hamster Ovary cells.  It catalyses the
hydrolysis of the glycolipid, 
glucocerebroside, to glucose and ceramide following the normal
degradation pathway for membrane 
lipids.   

Glucocerebroside is primarily derived from haematopoietic cell
turnover.  Gaucher disease is 
characterised by a functional deficiency in 
 - 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 and
occasionally, as well, in lung, 
kidney and intestine.  Secondary haematological sequelae include
severe anaemia and 
thrombocytopenia in addition to the characteristic progressive
hepatosplenomegaly.  The skeletal 
complications are a common, and frequently the most debilitating and
disabling, feature of Gaucher 
VERSION: CZM PI ANZ A1205-01 
SUPERCEDES: CZM