NEXVIAZYME

Valsts: Izraēla

Valoda: angļu

Klimata pārmaiņas: Ministry of Health

Aktīvā sastāvdaļa:

AVALGLUCOSIDASE ALPHA

Pieejams no:

SANOFI ISRAEL LTD

ATĶ kods:

A16AB22

Zāļu forma:

POWDER FOR CONCENTRATE FOR SOLUTION FOR INFUSION

Kompozīcija:

AVALGLUCOSIDASE ALPHA 100 MG/VIAL

Ievadīšanas:

I.V

Receptes veids:

Required

Ražojis:

GENZYME IRELAND LTD

Ārstniecības joma:

AVALGLUCOSIDASE ALPHA

Ārstēšanas norādes:

Nexviazyme is indicated long-term enzyme replacement therapy (ERT) for the treatment of patients with Pompe disease (acid α-glucosidase deficiency)

Autorizācija datums:

2022-10-25

Lietošanas instrukcija

IMMUNOTESTING SERVICES -
HCP GUIDE
NEXVIAZYME
®
(AVALGLUCOSIDASE ALFA)
GUIDANCE FOR HEALTH CARE PROFESSIONALS ON IMMUNOLOGY TESTING
SERVICES PROVIDED WITH NEXVIAZYME
®
ADMINISTRATION
MAT-IL-2300268
_YOU ARE ENCOURAGED TO REPORT ANY SUSPECTED ADVERSE EVENTS_ _TO
MINISTRY OF HEALTH _
_ACCORDING TO THE NATIONAL REGULATION BY USING AN ONLINE FORM _
_HTTPS://SIDEEFFECTS.HEALTH.GOV.IL/ _
Page 2 of 8
1.
Objectives and goals
2.
Key contacts
3.
Testing recommendations
4.
Testing practicalities
4.1.
Description of the Immunotesting services
4.2.
Procedure to access the Immunotesting services
5.
Reporting adverse events
AE
Adverse Event
HCP
Health Care Professional
IAR
Infusion-Associated Reaction
ADA
Antidrug Antibodies
SmPC
Summary of Product Characteristics
CONTENTS
ABBREVIATIONS
Page 3 of 8
AIMS OF THE IMMUNOTESTING SERVICES GUIDE
Nexviazyme
®
(avalglucosidase alfa) treatment should be supervised by a physician
experienced in the management of patients with Pompe disease or other
inherited
metabolic or neuromuscular diseases
The Nexviazyme® Immunotesting services guide is part of the
educational materials
provided to physicians involved in managing patients with Pompe
disease treated with
Nexviazyme®. Treating physicians may make this material available to
other health care
professionals (HCPs) involved in the management of the disease as
required. The main
purposes of the Immunotesting service guide are to:
1.
Guide
HCPs
to
carry out
immunological
testing
which
will
help
to
further
characterize the potential mechanism of infusion-associated reactions
(IARs)
and hypersensitivity reactions, and appropriately manage patients
experiencing
loss of treatment response due to antidrug antibodies (ADA).
2.
Provides information on the Sanofi Rare Disease Specialty Testing
program, for
immunological testing practicalities.
1.
OBJECTIVES AND GOALS
Page 4 of 8
•
TO REPORT ADVERSE EVENT(S) (AE) OCCURRING IN ASSOCIATION WITH THE USE
OF
NEXVIAZYME®:
Please contact the Ministry of Health by using an online form
https://sideeffe

Produkta apraksts

1
NEXVIAZYME
1.
NAME OF THE MEDICINAL PRODUCT
Nexviazyme
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each vial contains 100 mg of avalglucosidase alfa.
After reconstitution, each vial contains a total extractable volume of
10.0 ml at a concentration of
10 mg of avalglucosidase alfa* per ml.
*Avalglucosidase alfa is a human acid α-glucosidase produced in
Chinese hamster ovary cells (CHO)
by recombinant DNA technology, which is subsequently conjugated with
approximately 7
hexamannose structures (each containing two terminal
mannose-6-phosphate (M6P) moieties) to
oxidised sialic acid residues on the molecule, thereby increasing
bis-M6P levels.
For the full list of excipients, see section 6.1.
3.
PHARMACEUTICAL FORM
Powder for concentrate for solution for infusion
White to pale yellow lyophilised powder
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
Nexviazyme (avalglucosidase alfa) is indicated for long-term enzyme
replacement therapy for the
treatment of patients with Pompe disease (acid α-glucosidase
deficiency).
4.2
POSOLOGY AND METHOD OF ADMINISTRATION
Nexviazyme treatment should be supervised by a physician experienced
in the management of
patients with Pompe disease or other inherited metabolic or
neuromuscular diseases.
Posology
Patients may be pre-treated with antihistamines, antipyretics, and/or
corticosteroids to prevent or
reduce allergic reactions.
The recommended dose of avalglucosidase alfa is 20 mg/kg of body
weight administered once every
2 weeks.
_Dose modification for IOPD patients _
For IOPD patients who experience lack of improvement or insufficient
response in cardiac,
respiratory, and/or motor function while receiving 20 mg/kg, a dose
increase to 40 mg/kg every other
week should be considered in the absence of safety concerns (e.g.,
severe hypersensitivity,
anaphylactic reactions, or risk of fluid overload).
2
In patients who do not tolerate avalglucosidase alfa at 40 mg/kg every
other week (e.g., severe
hypersensitivity, anaphylactic reactions, or risk of fluid overload),
consider decre