Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia

Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia

Intravenous sodium benzoate and sodium phenylacetate have been used successfully in the treatment of acute hyperammonaemia in patients with urea cycle disorders. They provide alternative pathways for waste nitrogen disposal and help maintain nitrogen homeostasis. However, we report three patients wi...

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Bibliographic Details
Published in:Journal of inherited metabolic disease Vol. 23; no. 2; pp. 129 - 136
Main Authors: Praphanproj, V., Boyadjiev, S. A., Waber, L. J., Brusilow, S. W., Geraghty, M. T.
Format: Journal Article
Language:English
Published: Dordrecht Kluwer Academic Publishers 01-03-2000
Springer
Blackwell Publishing Ltd
Subjects:
Biological and medical sciences
Child
Child, Preschool
Drug Overdose
Errors of metabolism
Fatal Outcome
Female
Humans
Injections, Intravenous
Male
Medical sciences
Metabolic diseases
Metabolism, Inborn Errors - blood
Metabolism, Inborn Errors - drug therapy
Miscellaneous hereditary metabolic disorders
Ornithine Carbamoyltransferase - genetics
Ornithine Carbamoyltransferase Deficiency Disease
Phenylacetates - administration & dosage
Phenylacetates - adverse effects
Phenylacetates - therapeutic use
Quaternary Ammonium Compounds - blood
Sodium Benzoate - administration & dosage
Sodium Benzoate - adverse effects
Sodium Benzoate - therapeutic use
Urea - metabolism
Human
Drug
Intravenous administration
Acute
Hyperammonemia
Metabolic diseases
Infant
Urea cycle
Case study
Ammonia
Benzoate
Metabolic disorder
Chemotherapy
Treatment
Sodium
Carboxylate
Poisoning
Hospital
Child
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Summary:Intravenous sodium benzoate and sodium phenylacetate have been used successfully in the treatment of acute hyperammonaemia in patients with urea cycle disorders. They provide alternative pathways for waste nitrogen disposal and help maintain nitrogen homeostasis. However, we report three patients with hyperammonaemia who received inappropriate doses of intravenous sodium benzoate and sodium phenylacetate that resulted in severe complications. Ambiguous medical prescriptions and inadequate cross‐checking of drug dosage by physicians, nurses and pharmacists were the main causes of these incidents. All the patients presented with alteration in mental status, Kussmaul respiration and a partially compensated metabolic acidosis with an increased anion gap. Two patients developed cerebral oedema and hypotension and died. The third survived after haemodialysis. Plasma levels of benzoate and phenylacetate were excessively high. The possible mechanisms of toxicity, management and safety measures are discussed.
ISSN:0141-8955
1573-2665
DOI:10.1023/A:1005661631281