Sodium phenylacetate/sodium benzoate
|Sodium phenylacetate||Ammonia detoxicant|
|Sodium benzoate||Ammonia detoxicant|
|Trade names||Ammonul, Ucephan|
|Main uses||High blood ammonia resulting in encephalopathy in urea cycle disorders|
|Side effects||Vomiting, high blood sugar, low potassium, seizures, confusion|
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. It is used for conditions were there is problems with the enzymes involved in the urea cycle. It is given via a central venous catheter.
Common side effects include vomiting, high blood sugar, low potassium, seizures, and confusion. Other side effects may include metabolic acidosis, fluid overload, and skin necrosis. It is a combination of sodium phenylacetate and sodium benzoate. It works by binding to nitrogen.
The combination was approved for medical use in the United States in 1987. In the United States 50 ml of 10% solution of each component costs about 48,000 USD as of 2021. In Europe it was given an orphan designation in 2019.
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.
This is about 2.5 mL/kg for those under 20 kg and 55 mL/m2 for those over 20 kg.
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- "Ammonul Prices, Coupons & Patient Assistance Programs". Drugs.com. Archived from the original on 22 January 2021. Retrieved 12 October 2021.
- "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.
- 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.