Ucedane

מדינה: האיחוד האירופי

שפה: אנגלית

מקור: EMA (European Medicines Agency)

מרכיב פעיל:

carglumic acid

זמין מ:

Eurocept International BV

קוד ATC:

A16AA05

INN (שם בינלאומי):

carglumic acid

קבוצה תרפויטית:

Other alimentary tract and metabolism products,

איזור תרפויטי:

Hyperammonemia; Amino Acid Metabolism, Inborn Errors

סממני תרפויטית:

Ucedane is indicated in treatment of:hyperammonaemia due to N-acetylglutamate synthase primary deficiency;Hyperammonaemia due to isovaleric acidaemia;Hyperammonaemia due to methymalonic acidaemia;Hyperammonaemia due to propionic acidaemia.

leaflet_short:

Revision: 11

מצב אישור:

Authorised

תאריך אישור:

2017-06-23

עלון מידע

18
B. PACKAGE LEAFLET
19
PACKAGE LEAFLET: INFORMATION FOR THE USER
UCEDANE 200 MG DISPERSIBLE TABLETS
carglumic acid
READ ALL OF THIS LEAFLET CAREFULLY BEFORE YOU START TAKING THIS
MEDICINE BECAUSE IT CONTAINS
IMPORTANT INFORMATION FOR YOU.
-
Keep this leaflet. You may need to read it again.
-
If you have any further questions, ask your doctor or pharmacist.
-
This medicine has been prescribed for you only. Do not pass it on to
others. It may harm
them, even if their signs of illness are the same as yours.
-
If you get any side effects, talk to your doctor, or pharmacist. This
includes any possible
side effects not listed in this leaflet. See section 4.
WHAT IS IN THIS LEAFLET
1.
What Ucedane is and what it is used for
2.
What you need to know before you take Ucedane
3.
How to take Ucedane
4.
Possible side effects
5.
How to store Ucedane
6.
Contents of the pack and other information
1.
WHAT UCEDANE IS AND WHAT IT IS USED FOR
Ucedane can help eliminating excessive ammonia plasma levels (elevated
ammonia level in
the blood). Ammonia is especially toxic for the brain and leads, in
severe cases, to reduced
levels of consciousness and to coma.
Hyperammonaemia may be due to
•
the lack of a specific liver enzyme N-acetylglutamate synthase.
Patients with this rare
disorder are not able to eliminate nitrogen waste, which builds up
after eating protein.
This disorder persists during the entire life of the affected patient
and therefore the
need for this treatment is lifelong.
•
isovaleric acidaemia, methylmalonic acidaemia or propionic acidaemia.
Patients suffering
from one of these disorders need treatment during the hyperammonaemia
crisis.
2.
WHAT YOU NEED TO KNOW BEFORE YOU TAKE UCEDANE
DO NOT TAKE UCEDANE
if you are allergic to carglumic acid or any of the other ingredients
of Ucedane (listed in section 6).
Do not take Ucedane during breast-feeding.
WARNINGS AND PRECAUTIONS
Talk to your doctor or pharmacist before taking Ucedane.
Ucedane treatment should be initiated under the supervision of a
physician experienced

מאפייני מוצר

1
ANNEX I
SUMMARY OF PRODUCT CHARACTERISTICS
2
1.
NAME OF THE MEDICINAL PRODUCT
Ucedane 200 mg dispersible tablets
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each tablet contains 200 mg of carglumic acid.
For a full list of excipients, see section 6.1.
3.
PHARMACEUTICAL FORM
Dispersible tablet.
The tablets are rod-shaped, white and biconvex with three score lines
on both sides and engraving
“L/L/L/L” on one side. Approximate tablet dimensions are 17 mm in
length and 6 mm in width.
The tablet can be divided into four equal doses.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
Ucedane is indicated in treatment of
•
hyperammonaemia due to N-acetylglutamate synthase primary deficiency.
•
hyperammonaemia due to isovaleric acidaemia.
•
hyperammonaemia due to methymalonic acidaemia.
•
hyperammonaemia due to propionic acidaemia.
4.2
POSOLOGY AND METHOD OF ADMINISTRATION
Ucedane treatment should be initiated under the supervision of a
physician experienced
in the
treatment of metabolic disorders.
Posology
•
For N-acetylglutamate synthase deficiency:
Based on clinical experience, the treatment may be started as early as
the first day of life. The initial
daily dose should be 100 mg/kg, up to 250 mg/kg if necessary.
It should then be adjusted individually in order to maintain normal
ammonia plasma levels
(see
section 4.4).
In the long term, it may not be necessary to increase the dose
according to body weight as long
as adequate metabolic control is achieved; daily doses range from 10
mg/kg to 100 mg/kg.
_Carglumic acid responsiveness test _
It is recommended to test individual responsiveness to carglumic acid
before initiating any long
term treatment. As examples
-
In a comatose child, start with a dose of 100 to 250 mg/kg/day and
measure ammonia
plasma concentration at least before each administration. It should
normalise within a
few
hours after starting Ucedane.
-
In a patient with moderate hyperammonaemia, administer a test dose of
100 to
200 mg/kg/day for 3 days with a constant protein intake and p