Ammonaps
Country: Evrópusambandið
Tungumál: enska
Heimild: EMA (European Medicines Agency)
Upplýsingar fylgiseðill
(PIL)
16-12-2022
Vara einkenni
(SPC)
16-12-2022
Opinber matsskýrsla
(PAR)
09-11-2009
Virkt innihaldsefni:
Sodium phenylbutyrate
Fáanlegur frá:
Immedica Pharma AB
ATC númer:
A16AX03
INN (Alþjóðlegt nafn):
sodium phenylbutyrate
Meðferðarhópur:
Other alimentary tract and metabolism products,
Lækningarsvæði:
Ornithine Carbamoyltransferase Deficiency Disease; Citrullinemia; Carbamoyl-Phosphate Synthase I Deficiency Disease
Ábendingar:
Ammonaps is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase orargininosuccinate synthetase.It is indicated in all patients with neonatal-onset presentation (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.
Vörulýsing:
Revision: 21
Leyfisstaða:
Authorised
Leyfisdagur:
1999-12-07
Upplýsingar fylgiseðill
24
B. PACKAGE LEAFLET
25
PACKAGE LEAFLET: INFORMATION FOR THE USER
AMMONAPS 500 MG TABLETS
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 AMMONAPS is and what it is used for
2.
What you need to know before you take AMMONAPS
3.
How to take AMMONAPS
4.
Possible side effects
5.
How to store AMMONAPS
6.
Contents of the pack and other information
1.
WHAT AMMONAPS IS AND WHAT IT IS USED FOR
AMMONAPS is prescribed to patients with urea cycle disorders. Patients
with these rare disorders
have a deficiency of certain liver enzymes and are therefore unable to
eliminate nitrogen waste.
Nitrogen is a building block of proteins, because of this, there is a
build up of nitrogen in the body
after eating protein. Nitrogen waste, in the form of ammonia, is
especially toxic for the brain and
leads, in severe cases, to reduced levels of consciousness and to
coma.
AMMONAPS helps the body to eliminate nitrogen waste, reducing the
amount of ammonia in your
body.
2.
WHAT YOU NEED TO KNOW BEFORE YOU TAKE AMMONAPS
DO NOT TAKE AMMONAPS
-
if you are pregnant.
-
if you are breast-feeding.
-
if you are allergic to sodium phenylbutyrate or any of the other
ingredients of this medicine
(listed in section 6).
WARNINGS AND PRECAUTIONS
Talk to your doctor before taking AMMONAPS
-
if you have difficulty swallowing. AMMONAPS tablets can get stuck in
the oesophagus and
cause ulcers. If you have difficulty swallowing it is recommended to
use AMMONAPS
granules instead.
-
if you suffer from heart failure, a decr
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Vara einkenni
1
ANNEX I
SUMMARY OF PRODUCT CHARACTERISTICS
2
1.
NAME OF THE MEDICINAL PRODUCT
AMMONAPS 500 mg tablets.
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each tablet contains 500 mg sodium phenylbutyrate.
Excipient(s) with known effect
Each tablet contains 2.7 mmol (62 mg) of sodium.
For the full list of excipients, see section 6.1.
3.
PHARMACEUTICAL FORM
Tablet.
The tablets are off-white, oval and embossed with “UCY 500”.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
AMMONAPS 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_
presentation (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
AMMONAPS treatment should be supervised by a physician experienced in
the treatment of urea
cycle disorders.
The use of AMMONAPS tablets is indicated for adults and children who
are able to swallow tablets.
AMMONAPS is also available as granules for infants, children who are
unable to swallow tablets and
for patients with dysphagia.
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 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 (40 tablets)
have not been established.
_Therapeutic monitoring:_
Plasma levels of ammonia, arginine, essential amino acids (especially
branched chain amino acids), carnitine and serum proteins sho
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