CARBAGLU

Country: Israel

Language: English

Source: Ministry of Health

Active ingredient:

CARGLUMIC ACID

Available from:

MEDISON PHARMA LTD

ATC code:

A16AA05

Pharmaceutical form:

TABLETS DISPERSIBLE

Composition:

CARGLUMIC ACID 200 MG

Administration route:

PER OS

Prescription type:

Required

Manufactured by:

RECORDATI RARE DISEASES, FRANCE

Therapeutic group:

CARGLUMIC ACID

Therapeutic area:

CARGLUMIC ACID

Therapeutic indications:

Carbaglu 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.

Authorization date:

2017-03-23

Patient Information leaflet

1
PATIENT LEAFLET IN ACCORDANCE WITH THE PHARMACISTS' REGULATIONS
(PREPARATIONS)
1986
This medicine is dispensed with a doctor’s prescription only
CARBAGLU DISPERSIBLE TABLETS
Each tablet contains:
CARGLUMIC ACID 200 MG
Inactive ingredients see section 6 (“Additional information”) in
this leaflet.
READ THE ENTIRE LEAFLET CAREFULLY BEFORE YOU START USING THIS
MEDICINE. This leaflet
contains concise information about this medicine. If you have any
further questions,
consult your doctor or pharmacist.
This medicime has been prescribed to treat your illness. Do not pass
it on to others.
It may harm them, even if it seems to you that their illness is
similar to yours.
1. WHAT IS THIS MEDICINE USED FOR?
CARBAGLU can help eliminate excess ammonia from the blood. When
ammonia
accumulates in your blood it is toxic mainly to the brain. In severe
cases, its
accumulation may cause decreased level of consciousness and coma.
CARBAGLU is used to treat hyperammonemia (a condition in which high
levels of
ammonia are found in the plasma), in patients with hyperammonemia
caused by:

lack of a liver enzyme called N-acetylglutamate synthase. Patients
with this rare
condition cannot eliminate nitrogen, a waste material which
accumulates in their
body as a result of eating protein. This medical condition persists
throughout a
patient's life, therefore the need for this treatment is lifelong.

certain acidemia conditions (propionic acidemia, isovaleric acidemia,
methylmalonic acidemia). Patients suffering from one of these
conditions must be
treated during the hyperammonemia stage of their illness.
THERAPEUTIC GROUP:
CPS (carbamoyl phosphate synthase) activator.
2. BEFORE USING THIS MEDICINE
DO NOT USE THIS MEDICINE IF:

You are sensitive (allergic) to the active ingrediant carglumic acid
or to any of the
other ingredients in this medicine.

You are breastfeeding
SPECIAL WARNINGS ABOUT USING THIS MEDICINE

Your doctor will evaluate your individual response to carglumic acid
before
deciding on long-term treatment.
�

Summary of Product characteristics

1
PHYSICIAN'S PRESCRIBING INFORMATION
1
NAME OF THE MEDICINAL PRODUCT
Carbaglu 200 mg dispersible tablets
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
2.1
GENERAL DESCRIPTION
Each tablet contains 200 mg of carglumic acid.
2.2
QUALITATIVE AND QUANTITATIVE COMPOSITION
For a full list of excipients, see section 6.1
3.
PHARMACEUTICAL FORM
Dispersible tablet
The tablets are white and elongated with three score marks and
engraved on one side.
The tablet can be divided into equal halves.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
Carbaglu 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
Carbaglu 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 Carbaglu.
-
In a patient with moderate hyperammonaemia, administer a test dose of
100 to 200 mg/kg/day
2
for 3 days with a constant protein intake and perform repeated
determinations of ammonia
plasma c