Sodium phenylacetate/sodium benzoate

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]
WHO AWaReUnlinkedWikibase error: ⧼unlinkedwikibase-error-statements-entity-not-set⧽
Pregnancy
category
  • US: C (Risk not ruled out)
    External links
    AHFS/Drugs.comMonograph
    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 2 3 4 5 6 7 8 9 "Sodium Phenylacetate and Sodium Benzoate Monograph for Professionals". Drugs.com. Archived from the original on 16 November 2019. Retrieved 12 October 2021.
    2. 1 2 3 4 5 "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.
    Identifiers:
    • "Ammonul". Drug Information Portal. U.S. National Library of Medicine. Archived from the original on 2021-08-29. Retrieved 2021-01-03.
    This article is issued from Offline. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.