Ammonaps 940mgg granules

Country: Միացյալ Թագավորություն

language: անգլերեն

source: MHRA (Medicines & Healthcare Products Regulatory Agency)

active_ingredient:

Sodium phenylbutyrate

MAH:

Swedish Orphan Biovitrum Ltd

ATC_code:

A16AX03

INN:

Sodium phenylbutyrate

dosage:

940mg/1gram

pharmaceutical_form:

Granules

administration_route:

Gastroenteral; Oral

class:

No Controlled Drug Status

prescription_type:

Valid as a prescribable product

leaflet_short:

BNF: 09080100; GTIN: 7350031440102

PIL

1
PACKAGE LEAFLET: INFORMATION FOR THE USER
AMMONAPS 940 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 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 suffer from heart failure, a decrease in your kidney function
or other diseases where the
retention of the sodium salt contained in this medicine may make your
condition worse.
-
if you have decreased kidney or liver function, since AMMONAPS is

SPC

OBJECT 1
AMMONAPS 940 MG/G GRANULES
Summary of Product Characteristics Updated 19-Dec-2016 | Swedish
Orphan Biovitrum Ltd
1. Name of the medicinal product
AMMONAPS 940 mg/g granules
2. Qualitative and quantitative composition
Each gram of granules contains 940 mg of sodium phenylbutyrate.
One small spoon of AMMONAPS granules contains 149 mg of sodium.
One medium sized spoon of AMMONAPS granules contains 408 mg of sodium.
One large spoon of AMMONAPS granules contains 1200 mg of sodium.
For the full list of excipients, see section 6.1.
3. Pharmaceutical form
Granules.
The granules are off-white.
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.
AMMONAPS granules should be administered orally (to infants and
children unable to swallow tablets
and to patients with dysphagia) or via gastrostomy or nasogastric
tube.
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 have not been
established.
_Therapeutic monitoring:_ Plas