ArticleOrphanet journal of rare diseases2016
Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study.
Article in Orphanet journal of rare diseases, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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15 citing papers in PubMed, 1 synthesis or guideline pooled it, 40 citations in OpenAlex.
- Neuropsychological attributes of urea cycle disorders: A systematic review of the literature.Journal of inherited metabolic disease · 2019Pooled it
- Clinical characteristics and long-term outcomes of 101 patients with urea cycle disorders in China.Orphanet journal of rare diseases · 2025Article
- Perioperative care in pediatric OTC deficiency and goldenhar syndrome: a case report.Frontiers in pediatrics · 2025Article
- Severe non-hepatic hyperammonaemic encephalopathy in an immunocompromised adolescent with enterocolitis.BMJ case reports · 2024Article
- Article
- Effect of storage on the levels of sodium benzoate in soft drinks sold in some Nigerian market with exposure and health risk assessment.Environmental analysis, health and toxicology · 2022Article
- Metabolite, protein, and tissue dysfunction associated with COVID-19 disease severity.Scientific reports · 2022Article
- The effect of sodium benzoate, L-carnitine, and phenylacetate on valproate-induced hyperammonemia in Male Wistar rats.International journal of physiology, pathophysiology and pharmacology · 2022Article
- Sodium Benzoate Delays the Development ofFrontiers in microbiology · 2022Article
- Towards Understanding COVID-19: Molecular Insights, Co-infections, Associated Disorders, and Aging.Journal of Alzheimer's disease reports · 2021Article
- Considering Proximal Urea Cycle Disorders in Expanded Newborn Screening.International journal of neonatal screening · 2020Review
- Impact of Diagnosis and Therapy on Cognitive Function in Urea Cycle Disorders.Annals of neurology · 2019Observational
- Food Additive Sodium Benzoate (NaB) Activates NFκB and Induces Apoptosis in HCT116 Cells.Molecules (Basel, Switzerland) · 2018Article
- Pharmacologic rescue of hyperammonemia-induced toxicity in zebrafish by inhibition of ornithine aminotransferase.PloS one · 2018Article
- Incidence, disease onset and short-term outcome in urea cycle disorders -cross-border surveillance in Germany, Austria and Switzerland.Orphanet journal of rare diseases · 2017Article
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13 authors at 6 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundThe efficacy and safety of intra-venous (i.v.) sodium benzoate for treating acute episodes of hyperammonemia in urea cycle enzyme disorders (UCD) is well known. However, published data do not provide a clear picture of the benefits and risks of this drug. We report a retrospective multicentre study on the use of i.v. sodium benzoate in patients treated for UCD between 2000 and 2010 in the 6 French reference centres for metabolic diseases.
resultsSixty-one patients with UCDs - 22 ornithine transcarbamylase (20 confirmed, 2 suspected), 18 arginino-succinate synthetase, 15 carbamoyl phosphate synthetase, 3 arginosuccinate lyase, 1 arginase deficiency, 1 N-acetylglutamate synthetase, 1 HHH syndrome - required i.v. sodium benzoate over the course of 95 acute episodes (NH3 > 100 μmol/L or high-risk situations, i.e., gastroenteritis, surgery). Forty out of 61 patients experienced only one episode of decompensation (neonatal coma, 68.6 %). The most frequent cause of late decompensation was infection (55.5 %). A loading dose of i.v. sodium benzoate (median 250 mg/kg over 2 h) was administered for 41/95 acute episodes. The median maintenance dose was 246.1 mg/kg/day, administered via peripheral venous infusion in all cases except one via a central line. The total median duration of i.v. sodium benzoate treatment per episode was 2 days (0-13 days). The median durations of hospitalization in intensive care and metabolic units were 4 days (0-17 days) and 10 days (0-70 days), respectively. Eight patients died during the neonatal coma (n = 6) or surgery (n = 2). The median plasma ammonium level before treatment was 245.5 μmol/L (20.0-2274.0 μmol/L); it decreased to 40.0 μmol/L in patients who were alive (13.0-181.0 μmol/L) at the end of treatment with i.v. sodium benzoate. A decrease in ammonium level to ≤ 100 μmol/L was obtained in 92.8 % of episodes (64/69 of the episodes recorded for the 53 surviving patients). Five patients required another treatment for hyperammonemia (sodium phenylacetate + sodium benzoate, haemofiltration). Eighteen side effects were reported related to the i.v. infusion (local diffusion, oedema).
conclusionThis 10-year retrospective study shows that i.v. sodium benzoate associated with an emergency regimen is an effective and safe treatment for acute episodes of UCD.
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