Pheburane
Land: Den Europæiske Union
Sprog: engelsk
Kilde: EMA (European Medicines Agency)
Indlægsseddel
(PIL)
10-01-2024
Produktets egenskaber
(SPC)
10-01-2024
Offentlige vurderingsrapport
(PAR)
08-08-2013
Aktiv bestanddel:
Sodium phenylbutyrate
Tilgængelig fra:
Eurocept International B. V.
ATC-kode:
A16AX03
INN (International Name):
sodium phenylbutyrate
Terapeutisk gruppe:
Various alimentary tract and metabolism products
Terapeutisk område:
Carbamoyl-Phosphate Synthase I Deficiency Disease
Terapeutiske indikationer:
Treatment of chronic management of urea-cycle disorders.
Produkt oversigt:
Revision: 10
Autorisation status:
Authorised
Autorisation dato:
2013-07-30
Indlægsseddel
32
B. PACKAGE LEAFLET
33
PACKAGE LEAFLET: INFORMATION FOR THE PATIENT
PHEBURANE 483 MG/G GRANULES
Sodium phenylbutyrate
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 PHEBURANE is and what it is used for
2. What you need to know before you take PHEBURANE
3. How to take PHEBURANE
4. Possible side effects
5. How to store PHEBURANE
6. Contents of the pack and other information
1.
WHAT PHEBURANE IS AND WHAT IT IS USED FOR
PHEBURANE contains the active substance sodium phenylbutyrate which is
used to treat
patients of all ages with urea cycle disorders. These rare disorders
are due to a deficiency of
certain liver enzymes which are necessary to eliminate waste nitrogen
in the form of ammonia.
Nitrogen is a building block of proteins, which are an essential part
of the food we eat. As the
body breaks down protein after eating, waste nitrogen, in the form of
ammonia, accumulates
because the body cannot eliminate it. Ammonia is especially toxic for
the brain and leads, in
severe cases, to reduced levels of consciousness and to coma.
This medicine helps the body to eliminate waste nitrogen, reducing the
amount of ammonia in
your body. However PHEBURANE must be used along with a diet reduced in
proteins,
designed especially for you by the doctor and the dietician. You must
follow this diet carefully.
2.
WHAT YOU NEED TO KNOW BEFORE YOU TAKE PHEBURANE
DO NOT TAKE PHEBURANE IF YOU:
are allergic to sodium phenylbutyrate or any of the other ingredients
of this medicine
(listed in section 6).
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Produktets egenskaber
1
ANNEX I
SUMMARY OF PRODUCT CHARACTERISTICS
2
1.
NAME OF THE MEDICINAL PRODUCT
PHEBURANE 483 mg/g granules
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each gram of granules contains 483 mg of sodium phenylbutyrate.
Excipient(s) with known effect
Each gram of sodium phenylbutyrate contains 124 mg (5.4 mmol) of
sodium and 768 mg of sucrose.
For the full list of excipients, see section 6.1.
3.
PHARMACEUTICAL FORM
Granules.
White to off-white granules.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
PHEBURANE is indicated as adjunctive therapy in the chronic management
of urea cycle disorders,
involving deficiencies of carbamylphosphate synthetase, ornithine
transcarbamylase or
argininosuccinate synthetase.
It is indicated in all patients with
_neonatal-onset _
disease (complete enzyme deficiencies, presenting
within the first 28 days of life). It is also indicated in patients
with
_late-onset _
disease (partial enzyme
deficiencies, presenting after the first month of life) who have a
history of hyperammonaemic
encephalopathy.
4.2
POSOLOGY AND METHOD OF ADMINISTRATION
PHEBURANE treatment should be supervised by a physician experienced in
the treatment of urea
cycle disorders.
Posology
The daily dose should be individually adjusted according to the
patient’s protein tolerance and the
daily dietary protein intake needed to promote growth and development.
The usual total daily dose of sodium phenylbutyrate in clinical
experience is:
450 - 600 mg/kg/day in neonates, infants and children weighing less
than 20 kg.
9.9 - 13.0 g/m
2
/day in children weighing more than 20 kg, adolescents and adults.
The safety and efficacy of doses in excess of 20 g/day of sodium
phenylbutyrate have not been
established.
_Therapeutic monitoring _
3
Plasma levels of ammonia, arginine, essential amino acids (especially
branched chain amino acids),
carnitine and serum proteins should be maintained within normal
limits. Plasma glutamine should be
maintained at levels less than 1,000 μmol/L.
_ _
_Nutritional management _
PHEBURANE
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