Elaprase Infusion
Idursulfase
2mg/ml
Shire Human Genetic Therapies AB
| Pack size | 3ml Vial |
|---|---|
| Dispensing mode | POM |
| Source | SWEDEN |
| Agent | CITY MEDICAL STORE |
| Retail Price | 14938.50 AED |
Indications
Elaprase Infusion is used for:
For the treatment of Hunter syndrome in adults and children ages 5 and older.
Adult Dose
Child Dose
Renal Dose
Administration
Contra Indications
Precautions
Pregnancy-Lactation
Interactions
Adverse Effects
Side effects of Idursulfase :
Mechanism of Action
Hunter's Syndrome is an X-linked recessive disease caused by insufficient levels of the lysosomal enzyme iduronate-2-sulfatase. This enzyme cleaves the terminal 2-O-sulfate moieties from the glycosaminoglycans (GAG) dermatan sulfate and heparan sulfate. Due to the missing or defective iduronate-2-sulfatase enzyme in patients with Hunter's Syndrome, GAG progressively accumulate in the lysosomes of a variety of cells, leading to cellular engorgement, organomegaly, tissue destruction and organ system dysfunction. Treatment of Hunter's Syndrome patients with idursulfase provides exogenous enzyme for uptake into cellular lysosomes. Targeting of idursulfase to the lysosome occurs by endocytosis from the cell surface. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzymes to the M6P receptors on the cell surface, leading to cellular internalization of the enzyme, targeting to intracellular lysosomes and subsequent catabolism of accumulated GAG.
Note
Elaprase 2mg/ml Infusion manufactured by Shire Human Genetic Therapies AB. Its generic name is Idursulfase. Elaprase is availble in United Arab Emirates.
Farmaco UAE drug index information on Elaprase Infusion is not intended for diagnosis, medical advice or treatment; neither intended to be a substitute for the exercise of professional judgment.