Elaprase (TM) Solution for Intravenous Infusion 2mg ml

Pajjiż: Singapor

Lingwa: Ingliż

Sors: HSA (Health Sciences Authority)

Ingredjent attiv:

Idursulfase

Disponibbli minn:

SANOFI-AVENTIS SINGAPORE PTE. LTD.

Kodiċi ATC:

A16AB09

Dożaġġ:

6.0 mg/vial

Għamla farmaċewtika:

INFUSION, SOLUTION CONCENTRATE

Kompożizzjoni:

Idursulfase 6.0 mg/vial

Rotta amministrattiva:

INTRAVENOUS

Tip ta 'preskrizzjoni:

Prescription Only

Manifatturat minn:

Shire HGT, Inc

L-istatus ta 'awtorizzazzjoni:

ACTIVE

Data ta 'l-awtorizzazzjoni:

2010-12-24

Fuljett ta 'informazzjoni

DESCRIPTION
ELAPRASE is a formulation of idursulfase, a purified form of human iduronate-2-sulfatase, a lysosomal
enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is
an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the gly-
cosaminoglycans (GAG) dermatan sulfate and heparan sulfate in the lysosomes of various cell types.
Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons.
The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide
structures. The enzyme activity of idursulfase is dependent on the post-translational modification of a
specific cysteine to formylglycine. Idursulfase has a specific activity ranging from 41 to 77 U/mg of
protein (one unit is defined as the amount of enzyme required to hydrolyze 1 µmole of heparin disac-
charide substrate per hour under the specified assay conditions).
ELAPRASE is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic clear to
slightly opalescent, colorless solution that must be diluted prior to administration in 0.9% Sodium Chlo-
ride Injection, USP. Each vial contains an extractable volume of 3.0 mL with an idursulfase concen-
tration of 2.0 mg/mL at a pH of approximately 6, providing 6.0 mg idursulfase, 24.0 mg sodium chloride,
6.75 mg sodium phosphate monobasic monohydrate, 2.97 mg sodium phosphate dibasic heptahydrate,
and 0.66 mg polysorbate 20. ELAPRASE does not contain preservatives; vials are for single use only.
CLINICAL PHARMACOLOGY
MECHANISM OF ACTION
Hunter syndrome (Mucopolysaccharidosis II, MPS II) is an X-linked recessive disease caused by
insufficient levels of the lysosomal enzyme iduronate-2-sulfatase. This enzyme cl

Karatteristiċi tal-prodott

1
ELAPRASE
® (IDURSULFASE)
Solution for intravenous infusion
WARNING: RISK OF ANAPHYLAXIS
LIFE-THREATENING ANAPHYLACTIC REACTIONS HAVE OCCURRED IN SOME PATIENTS
DURING AND UP TO 24
HOURS AFTER ELAPRASE INFUSIONS. ANAPHYLAXIS, PRESENTING AS RESPIRATORY
DISTRESS, HYPOXIA,
HYPOTENSION, URTICARIA AND/OR ANGIOEDEMA OF THROAT OR TONGUE HAVE BEEN
REPORTED TO OCCUR
DURING AND AFTER ELAPRASE INFUSIONS, REGARDLESS OF DURATION OF THE
COURSE OF TREATMENT.
CLOSELY OBSERVE PATIENTS DURING AND AFTER ELAPRASE ADMINISTRATION AND
BE PREPARED TO
MANAGE ANAPHYLAXIS. INFORM PATIENTS OF THE SIGNS AND SYMPTOMS OF
ANAPHYLAXIS AND HAVE
THEM SEEK IMMEDIATE MEDICAL CARE SHOULD SYMPTOMS OCCUR. PATIENTS WITH
COMPROMISED
RESPIRATORY FUNCTION OR ACUTE RESPIRATORY DISEASE MAY BE AT RISK OF
SERIOUS ACUTE EXACERBATION
OF THEIR RESPIRATORY COMPROMISE DUE TO HYPERSENSITIVITY REACTIONS, AND
REQUIRE ADDITIONAL
MONITORING [_SEE WARNINGS AND PRECAUTIONS AND ADVERSE REACTIONS_].
INDICATIONS AND USAGE
ELAPRASE
is
indicated
for
patients
with
Hunter
syndrome
(Mucopolysaccharidosis
II,
MPS
II).
ELAPRASE has been shown to improve walking capacity in these patients.
DOSAGE AND ADMINISTRATION
The recommended dosage regimen of ELAPRASE is 0.5 mg/kg of body weight
administered every week
as an intravenous infusion.
Parenteral drug products should be inspected visually for particulate
matter and discoloration prior to
administration, whenever solution and container permit.
PREPARATION INSTRUCTIONS
Prepare and use ELAPRASE according to the following steps using
aseptic technique:
a.
Determine the total volume of ELAPRASE to be administered and the
number of vials needed
based on the patient’s weight and the recommended dose of 0.5 mg/kg.
Patient’s weight (kg)
×
0.5 mg per kg of ELAPRASE
÷
2 mg per mL =
Total mL of ELAPRASE
Total mL of ELAPRASE
÷
3 mL per vial = Total number of vials
Round up to the next whole vial to determine the total number of vials
needed. Remove the
required number of vials from the refrigerator to allow them to reach
room temperature