Country: ইস্রায়েল
ভাষা: ইংরেজি
সূত্র: Ministry of Health
SODIUM PHENYLBUTYRATE
TRUEMED LTD, ISRAEL
A16AX03
GRANULES
SODIUM PHENYLBUTYRATE 483 MG/G
ORAL
Required
EUROCEPT INTERNATIONAL B.V., THE NETHERLANDS
SODIUM PHENYLBUTYRATE
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.
2020-02-29
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 সম্পূর্ণ নথি পড়ুন
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 সম্পূর্ণ নথি পড়ুন