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What is Sodium Phenylbutyrate
Sodium phenylbutyrate is an adjunctive therapy in the chronic management of urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS).
It must be combined with dietary protein restriction and, in some cases, essential amino acid supplementation.
Sodium phenylbutyrate is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life), as well as in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy.
Brand Name
Buphenyl
Indications & Dosage
- argininosuccinic acid syntherase (AAS) deficiency
- carbamoyl phosphate synthetase (CPS) deficiency
- ornithine transcarbamoylase (OTC) deficiency
For use as an adjunctive therapy to dietary protein restriction and amino acid supplementation in the chronic management of patients with neonatal-onset, or late-onset with history of hyperammonemic encephalopathy, urea cycle disorders ornithine transcarbamoylase (OTC) deficiency, carbamoyl phosphate synthetase (CPS) deficiency, or argininosuccinic acid syntherase (AAS) deficiency
Side Effects
- abdominal pain
- amenorrhea
- anemia
- aplastic anemia
- arrhythmia exacerbation
- bleeding
- constipation
- depression
- drug-induced body odor
- dysgeusia
- ecchymosis
- edema
- elevated hepatic enzymes
- gastritis
- headache
- hyperbilirubinemia
- hyperchloremia
- hypernatremia
- hyperphosphatemia
- hyperuricemia
- hypoalbuminemia
- hypokalemia
- hypophosphatemia
- leukocytosis
- leukopenia
- menstrual irregularity
- metabolic acidosis
- metabolic alkalosis
- nausea
- pancreatitis
- peptic ulcer
- rash
- renal tubular acidosis (RTA)
- syncope
- thrombocytopenia
- thrombocytosis
- vomiting
Contraindications
- breast-feeding
- children
- corticosteroid therapy
- edema
- heart failure
- hepatic disease
- infants
- neonates
- pregnancy
- renal disease
- renal failure
- renal impairment
- sodium restriction
- Azelastine; Fluticasone
- Beclomethasone
- Betamethasone
- Budesonide
- Budesonide; Formoterol
- Ciclesonide
- Colchicine; Probenecid
- Corticosteroids
- Corticotropin, ACTH
- Cortisone
- Deflazacort
- Dexamethasone
- Dichlorphenamide
- Fludrocortisone
- Flunisolide
- Fluticasone
- Fluticasone; Salmeterol
- Fluticasone; Umeclidinium; Vilanterol
- Fluticasone; Vilanterol
- Formoterol; Mometasone
- Haloperidol
- Hydrocortisone
- Methylprednisolone
- Mometasone
- Prednisolone
- Prednisone
- Probenecid
- Sodium Benzoate; Sodium Phenylacetate
- Triamcinolone
- Valproic Acid, Divalproex Sodium