PHEBURANE

Country: Իսրայել

language: անգլերեն

source: Ministry of Health

active_ingredient:

SODIUM PHENYLBUTYRATE

MAH:

TRUEMED LTD, ISRAEL

ATC_code:

A16AX03

pharmaceutical_form:

GRANULES

composition:

SODIUM PHENYLBUTYRATE 483 MG/G

administration_route:

ORAL

prescription_type:

Required

manufactured_by:

EUROCEPT INTERNATIONAL B.V., THE NETHERLANDS

therapeutic_area:

SODIUM PHENYLBUTYRATE

therapeutic_indication:

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 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.

authorization_date:

2020-02-29

PIL

ةلديصلا ةمظنأ بجومب كلهتسملل ةرشن
1986 ـ )تارضحتسم(
طقف بيبط ةفصو بسح ءاودلا ق
ّ
وسي
ناروبيف
تابيبح
يئاود رادقم ةدحوب اهتيمكو ةلاعفلا
ةداملا
تاريتوب لينيف مويدوص غلم 483 ىلع يوتحي
تابيبح مارغ 1
Sodium phenylbutyrate 483 mg/g granules
زوركس غلم 768 ـو مويدوص غلم 124 ىلع ءاودلا
يوتحي
.تاريتوب لينيف مويدوص نم مارغ 1 لكل
رظنأ :رضحتسملا يف ةيساسحلا تادلومو
ةلاعفلا ريغ داوملا
ةرقفلاو "ءاودلا تابكرم ضعب نع ةماه
تامولعم" 2 ةرقفلا
."ةيفاضإ تامولعم" 6
.ءاودلل كلامعتسإ لبق اهتياهن ىتح نعمتب
ةرشنلا أرقإ
ترفوت اذإ .ءاودلا نع ةزجوم تامولعم ىلع
ةرشنلا هذه يوتحت
.يلديصلا وأ بيبطلا عجار ،ةيفاضإ ةلئس
أ
كيدل
مهرضي دق وهف .نيرخلآل هيطعت لا ،كلجأ نم
ءاودلا اذه فصو
.كتلاحل ةهباشم ةيبطلا مهتلاح نأ كل ادب
ولو ىتح
؟ءاودلا صصخم ضرغ يلأ )1
.ايرويلا ةرود يف تابارطضإ نم نوناعي
نيذلا ىضرملا جلاعل
تابارطضإ عم نمزملا لماعتلل ممتم جلاعك
ناروبيف فصو متي
:تاميزنلإا صقن اهيف لخدتي يتلا
،ايرويلا ةرود
carbamylphosphate synthetase,
ornithine transcarbamylase
.argininosuccinate synthetase وأ
عم(
ً
اثيدح نيدولوملا ىضرملا ةفاكل صصخم
ءاودلا
ىلولأا مايلأا 28 للاخ رهظي يذلا ،لماك
يميزنإ صقن
رهظي ىضرمل صصخم ءاودلا
،كلذك .)مهتايح نم
يف يئزج صقن عم( رثكأ مدقتم رمع يف ضرملا
مهيدل
نيذلا )ةايحلا نم لولأا رهشلا دعب رهظي
يذلا ،ميزنلإا
مد

SPC

1. NAME OF THE MEDICINAL PRODUCT
PHEBURANE
®
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 _
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
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_
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 must be combined with dietary protein restriction and, in
some ca