Sodium Phenylbutyrate

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Medicines Management & Pharmacy Services (MMPS) LTHT Paediatric Critical Care Administration Guide Intravenous Sodium Phenylbutyrate Dose: Acute hyperammonaemia Confirm dose required with advising metabolic consultant Loading dose: 250mg/kg given over 90mins (this is not always required - please check) Continuous infusion: by 250mg/kg/day If necessary the dose may be increased to 500mg/kg/day (check with metabolic consultant if this is required) This is the metabolic consultants practice and doses are different than stated in the BNFc Route of Administer via a central or peripheral line. administration: Standard concentration of 50mg/mL For children weighing less than or equal to 20kg: 2.5gram in 50mL of glucose 5% or 10% For children weighing over 20kg to 40kg: 5gram in 100mL of glucose 5% or 10% For children weighing over 40kg: 12.5grams in 250mL of glucose 5% or 10% Note: 250mg/kg/day = 0.208mL/kg/hour Products available: Sodium Phenylbutyrate Injection 1g/5mL (200mg/mL) or 2g/10mL (200mg/mL) To prepare an Preferred diluent: Glucose 10% infusion: 2.5gram in 50mL; Draw up 12.5mL of sodium phenylbutyrate 200mg/mL(2.5gram) into a 50mL syringe and make up to 50mL with diluent (50mg/mL) 5gram in 100mL; Withdraw 25mL from the 100mL bag of diluent and discard. Draw up 25mL of sodium phenylbutyrate 200mg/mL(5gram) and add to bag. Mix well. Final concentration 50mg/mL. 12.5gram in 250mL; Withdraw 62.5mL from the 250mL bag of diluent and discard. Draw up 62.5mL of sodium phenylbutyrate 200mg/mL (12.5gram) and add to bag. Mix well. Final concentration 50mg/mL. How to prescribe: Prescribe the continuous infusion on an LTH Critical Care Intravenous Infusion Prescription Sheet. Compatibilities: Compatible at Y-site with sodium benzoate, carnitine and arginine Notes: If there is no compatibility information for specific drugs, do not assume compatibility, use a separate line. Aim is to maintain plasma concentration of ammonia below 60micromol/L and glutamine below 800micromol/L There is also a drug calculator on the bimdg website. For details visit www.bimdg.org.uk. This calculator will use a different method of making up the infusion than above. If using this site please check calculations independently and stability of formulation. References: Trissel, SPC, Medusa, BNFc online (accessed 16/08/21), Evelina Paediatric Formulary (accessed 16/08/21), BMIDG.org.uk (accessed 16/08/21) Provenance Date Produced: May 2013 Reviewed: August 2021 Valid until: August 2023 Author: Teresa Brooks, paediatric cardiology pharmacist Quality Assurance Check by: Jenny Jackson, paediatric pharmacist Produced by Clinical Pharmacy Services For further information contact [email protected] ext 65377 This information has been prepared by Leeds Medicines Management and Pharmacy Services for use by health care professionals at Leeds Teaching Hospitals NHS Trust .

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Sodium Phenylbutyrate (PB), Is a New Active Substance
SCIENTIFIC DISCUSSION This module reflects the initial scientific discussion for the approval of Ammonpas. This scientific discussion has been updated until 1 November 2001. For information on changes after this date please refer to module 8B. 1. Introduction Ammonaps, sodium phenylbutyrate (PB), is a new active substance. It 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. The dossier submitted in support of the application comprises data generated by the applicant: all chemical/pharmaceutical data, the two mutagenicity studies for Part III, and for Part IV, the bioequivalence study and a review of the US IND/NDA programme. Additional information was available from published literature. Urea cycle disorders (UCD) are inherited deficiencies of one of the enzymes involved in the urea cycle, by which ammonium is converted to urea. Ammonium is highly toxic to nerve cells and hyperammonaemia may result in metabolic derangement, leading to anorexia, lethargy, confusion, coma, brain damage, and death. The most severe forms of UCDs occur early in life (complete enzyme deficiencies). The classic neonatal presentation of all the UCD (with the exception of arginase deficiency) is quite uniform and includes, after a short symptom-free interval of one to five days, poor feeding, vomiting, lethargy, muscular hypotonia, hyperventilation, irritability and convulsions.
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Carbaglu and Ravicti
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TRANSPARENCY COMMITTEE Opinion 22 January 2014
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Pheburane, INN-Sodium Phenylbutyrate
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