Sodium Benzoate and Sodium Phenylacetate

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What is Sodium Benzoate and Sodium Phenylacetate combination medicine

Sodium benzoate and sodium phenylacetate are used together in an injectable preparation as an adjunctive treatment of acute hyperammonemia.

The oral form of sodium benzoate; sodium phenylacetate is no longer available in the United States.

Sodium benzoate; sodium phenylacetate provide an alternative pathway for waste nitrogen excretion in patients with disorders of the urea cycle. 

Brand Name

  • Ammonul
  • Ucephan

Indications & Dosage

  • hyperammonemia

General dosing information:

  • Uncontrolled hyperammonemia can rapidly cause brain damage or death and should be treated immediately, especially in neonates presenting with coma, regardless of whether the specific causative enzyme deficiency has been identified. If the enzyme deficiency is unknown, administer 600 mg/kg of arginine HCl over 90 minutes and an equivalent dose over 24 hours to all hyperammonemic infants with suspected urea cycle deficiency.
  • Do not give more than one loading dose due to prolonged phenylacetate plasma concentrations and toxicity. In a trial, the mean treatment duration per episode was 4.6 days (range, 1—72 days).48775

For the adjunctive treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle

for patients with carbamyl phosphate synthetase or ornithine transcarbamylase deficiency

Side Effects

  1. acute respiratory distress syndrome (ARDS)
  2. agitation
  3. alopecia
  4. anemia
  5. anuria
  6. asthenia
  7. ataxia
  8. bleeding
  9. bradycardia
  10. cardiac arrest
  11. cardiomyopathy
  12. cerebral degeneration
  13. cerebral edema
  14. chest pain (unspecified)
  15. cholestasis
  16. coagulopathy
  17. coma
  18. confusion
  19. dehydration
  20. diarrhea
  21. disseminated intravascular coagulation (DIC)
  22. dyspnea
  23. edema
  24. encephalopathy
  25. fever
  26. fluid retention
  27. flushing
  28. GI bleeding
  29. hallucinations
  30. hepatic failure
  31. hyperammonemia
  32. hyperglycemia
  33. hyperkalemia
  34. hypernatremia
  35. hypertension
  36. hyperventilation
  37. hypocalcemia
  38. hypoglycemia
  39. hypokalemia
  40. hyporeflexia
  41. hypotension
  42. impaired cognition
  43. increased intracranial pressure
  44. infection
  45. injection site reaction
  46. intracranial bleeding
  47. irritability
  48. jaundice
  49. metabolic acidosis
  50. nausea
  51. pancytopenia
  52. pericardial effusion
  53. pneumothorax
  54. pruritus
  55. psychosis
  56. pulmonary edema
  57. rash
  58. renal failure (unspecified)
  59. seizures
  60. skin necrosis
  61. subdural hematoma
  62. tachypnea
  63. tetany
  64. thrombocytopenia
  65. thrombosis
  66. tremor
  67. urinary retention
  68. urticaria
  69. visual impairment
  70. vomiting

Monitoring Parameters

  • plasma ammonia concentrations
  • serum bicarbonate
  • serum creatinine/BUN
  • serum electrolytes
  • total CO2

Contraindications

  • breast-feeding
  • diabetes mellitus
  • edema
  • extravasation
  • heart failure
  • hepatic disease
  • hypernatremia
  • hypertension
  • hypokalemia
  • intraarterial administration
  • intramuscular administration
  • labor
  • pregnancy
  • renal failure
  • renal impairment
  • seizures
  • sodium restriction

Interactions

  • Acetaminophen; Aspirin, ASA; Caffeine
  • Acetaminophen; Caffeine; Magnesium Salicylate; Phenyltoloxamine
  • Aluminum Hydroxide
  • Aluminum Hydroxide; Magnesium Carbonate
  • Aluminum Hydroxide; Magnesium Hydroxide
  • Aluminum Hydroxide; Magnesium Hydroxide; Simethicone
  • Aluminum Hydroxide; Magnesium Trisilicate
  • Amoxicillin
  • Amoxicillin; Clarithromycin; Lansoprazole
  • Amoxicillin; Clarithromycin; Omeprazole
  • Amoxicillin; Clavulanic Acid
  • Ampicillin
  • Ampicillin; Sulbactam
  • Antacids
  • Aspirin, ASA
  • Aspirin, ASA; Butalbital; Caffeine
  • Aspirin, ASA; Butalbital; Caffeine; Codeine
  • Aspirin, ASA; Caffeine; Dihydrocodeine
  • Aspirin, ASA; Caffeine; Orphenadrine
  • Aspirin, ASA; Carisoprodol
  • Aspirin, ASA; Carisoprodol; Codeine
  • Aspirin, ASA; Citric Acid; Sodium Bicarbonate
  • Aspirin, ASA; Dipyridamole
  • Aspirin, ASA; Omeprazole
  • Aspirin, ASA; Oxycodone
  • Aspirin, ASA; Pravastatin
  • Azelastine; Fluticasone
  • Beclomethasone
  • Betamethasone
  • Bismuth Subsalicylate
  • Bismuth Subsalicylate; Metronidazole; Tetracycline
  • Budesonide
  • Budesonide; Formoterol
  • Calcium Carbonate; Magnesium Hydroxide
  • Carbenicillin
  • Choline Salicylate; Magnesium Salicylate
  • Ciclesonide
  • Colchicine
  • Colchicine; Probenecid
  • Corticosteroids
  • Corticotropin, ACTH
  • Cortisone
  • Deflazacort
  • Dexamethasone
  • Dichlorphenamide
  • Dicloxacillin
  • Fludrocortisone
  • Flunisolide
  • Fluticasone
  • Fluticasone; Salmeterol
  • Fluticasone; Umeclidinium; Vilanterol
  • Fluticasone; Vilanterol
  • Formoterol; Mometasone
  • Hydrocortisone
  • Magnesium Hydroxide
  • Magnesium Salicylate
  • Methadone
  • Methylprednisolone
  • Mometasone
  • Nafcillin
  • Omeprazole; Amoxicillin; Rifabutin
  • Oxacillin
  • Penicillin G
  • Penicillin G Benzathine
  • Penicillin G Benzathine; Penicillin G Procaine
  • Penicillin G Procaine
  • Penicillin V
  • Penicillins
  • Piperacillin
  • Piperacillin; Tazobactam
  • Prednisolone
  • Prednisone
  • Probenecid
  • Salsalate
  • Sodium Phenylbutyrate
  • Ticarcillin
  • Ticarcillin; Clavulanic Acid
  • Triamcinolone
  • Valproic Acid, Divalproex Sodium

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