Sodium phenylacetate/sodium benzoate

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Sodium phenylacetate/sodium benzoate
Combination of
Sodium phenylacetateAmmonia detoxicant
Sodium benzoateAmmonia detoxicant
Names
Trade namesAmmonul, Ucephan
Clinical data
Main usesHigh blood ammonia resulting in encephalopathy in urea cycle disorders[1]
Side effectsVomiting, high blood sugar, low potassium, seizures, confusion[1]
Pregnancy
category
  • US: C (Risk not ruled out)
External links
AHFS/Drugs.comMonograph
US NLMSodium phenylacetate/sodium benzoate
Legal
Legal status

Sodium phenylacetate/sodium benzoate, sold under the brand name Ammonul among others, is a combination medication used to treat high blood ammonia that results in encephalopathy.[1] It is used for conditions were there is problems with the enzymes involved in the urea cycle.[2] It is given via a central venous catheter.[1]

Common side effects include vomiting, high blood sugar, low potassium, seizures, and confusion.[1] Other side effects may include metabolic acidosis, fluid overload, and skin necrosis.[2] It is a combination of sodium phenylacetate and sodium benzoate.[1] It works by binding to nitrogen.[2]

The combination was approved for medical use in the United States in 1987.[1] In the United States 50 ml of 10% solution of each component costs about 48,000 USD as of 2021.[3] In Europe it was given an orphan designation in 2019.[4]

Medical uses

It is used for high blood ammonia that results in encephalopathy when the underlying cause relates to problems in the urea cycle.[2]

These urea cycle disorders include deficiency in carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), and argininosuccinate lyase (ASL).[1]

It may be used together with other measures such as arginine supplementation, caloric supplementation, restricting protein, and hemodialysis.[2]

Dosage

It is given as an initial dose of 5.5 g/m2 of each component over about 2 hours and than a similar dose over 24 hrs.[1]

This is about 2.5 mL/kg for those under 20 kg and 55 mL/m2 for those over 20 kg.[5]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 "Sodium Phenylacetate and Sodium Benzoate Monograph for Professionals". Drugs.com. Archived from the original on 16 November 2019. Retrieved 12 October 2021.
  2. 2.0 2.1 2.2 2.3 2.4 "DailyMed - AMMONUL- sodium phenylacetate and sodium benzoate injection, solution, concentrate". dailymed.nlm.nih.gov. Archived from the original on 21 March 2021. Retrieved 12 October 2021.
  3. "Ammonul Prices, Coupons & Patient Assistance Programs". Drugs.com. Archived from the original on 22 January 2021. Retrieved 12 October 2021.
  4. "EU/3/19/2157: Orphan designation for the treatment of ornithine transcarbamylase deficiency". Archived from the original on 24 June 2019. Retrieved 12 October 2021.
  5. Gahart, Betty L.; Nazareno, Adrienne R. (23 August 2014). 2015 Intravenous Medications - E-Book: A Handbook for Nurses and Health Professionals. Elsevier Health Sciences. p. 1114. ISBN 978-0-323-08477-2. Archived from the original on 12 October 2021. Retrieved 12 October 2021.

External links

Identifiers:
  • "Ammonul". Drug Information Portal. U.S. National Library of Medicine. Archived from the original on 2021-08-29. Retrieved 2021-01-03.