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Data updated: May 26, 2026

SODIUM PHENYLACETATE AND SODIUM BENZOATE (sodium benzoate)

Rare Disease Approved 2016-02-24

Sodium phenylacetate and sodium benzoate is a nitrogen-binding agent indicated as adjunctive therapy for the treatment of acute hyperammonemia and associated encephalopathy in pediatric and adult patients with urea cycle disorders (UCDs). It is used during acute metabolic crises alongside dietary protein restriction, arginine supplementation, and other ammonia-lowering modalities such as hemodialysis. The medication provides a critical alternative vehicle for nitrogen excretion when the primary urea cycle enzymes are deficient.

Source: FDA Label • MAIA PHARMS INC

How SODIUM PHENYLACETATE AND SODIUM BENZOATE Works

This medication functions by creating alternative metabolic pathways for the excretion of waste nitrogen. Phenylacetate conjugates with glutamine to form phenylacetylglutamine (removing two moles of nitrogen per mole of phenylacetate), while benzoate conjugates with glycine to form hippuric acid (removing one mole of nitrogen per mole of benzoate). These resulting compounds are rapidly excreted by the kidneys via glomerular filtration and tubular secretion, effectively bypassing the defective urea cycle to reduce toxic ammonia levels.

5
Indications
--
Phase 3 Trials
10
Years on Market

Details

Status
Prescription
First Approved
2016-02-24
Routes
INTRAVENOUS
Dosage Forms
SOLUTION

SODIUM PHENYLACETATE AND SODIUM BENZOATE Approval History

2017
2018
2019
2020
2021
2022
2023
2024
2025
2026
Original
New Indication
New Form
Label Update
7 FDA actions from 2016 to 2023
Sep 2023 SUPPL
Label · Labeling
Jul 2023 ORIGINAL
Update
May 2023 SUPPL
Label · Labeling

What SODIUM PHENYLACETATE AND SODIUM BENZOATE Treats

3 indications

SODIUM PHENYLACETATE AND SODIUM BENZOATE is approved for 3 conditions since its original approval in 2016. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Hyperammonemia
  • Encephalopathy
  • Urea Cycle Disorder
Source: FDA Label

SODIUM PHENYLACETATE AND SODIUM BENZOATE Competitive Set

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Drugs Similar to SODIUM PHENYLACETATE AND SODIUM BENZOATE

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RAVICTI
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2 shared
HORIZON THERAP US
Shared indications:
Urea Cycle DisorderHyperammonemia
CARBAGLU
CARGLUMIC ACID
1 shared
RECORDATI RARE
Shared indications:
Hyperammonemia
CARGLUMIC ACID
CARGLUMIC ACID
1 shared
NAVINTA LLC
Shared indications:
Hyperammonemia
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Clinical Trial Registry

2 trials
Trial Sponsor ID Phase Status Title
NCT03510741 PILL-Sodium Benzoate & NAC-001 Ph 2 completed Sodium Benzoate and/or N-Acetylcysteine Added to TAU in Patients With Early Schizophrenia Spectrum Disorder.
NCT02026947 AttenPsyc 3418363 2013-000458-23 Ph 2 withdrawn Sodium Benzoate for Treatment of Attenuated/Transient Psychosis. A Randomized Placebo-controlled Trial.
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Active Pipeline

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Key Completed Trials

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Trial Timeline

Full development history with FDA approval milestones

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Understanding FDA Approval Types
Count Type What it means
- ORIG Original approval - drug first enters market
- SUPPL - Efficacy New indication (new disease/condition approved)
- SUPPL - Labeling Label text changes (warnings, dosing updates)
- SUPPL - Manufacturing Production changes (new facility)
- SUPPL - Chemistry Formulation changes (new dosage strength)

Green lines in the timeline show ORIG and Efficacy approvals - the clinically meaningful milestones.

SODIUM PHENYLACETATE AND SODIUM BENZOATE FDA Label Details

Indications & Usage

FDA Label (PDF)

SODIUM PHENYLACETATE AND SODIUM BENZOATE is indicated for the treatment of Hyperammonemia; Encephalopathy; Urea Cycle Disorder.

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Data Sources

Data sourced from official FDA and NIH databases. Click links to verify on original sources.