V. Praphanproj et al., Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia, J INH MET D, 23(2), 2000, pp. 129-136
Intravenous sodium benzoate and sodium phenylacetate have been used success
fully in the treatment of acute hyperammonaemia in patients with urea cycle
disorders. They provide alternative pathways for waste nitrogen disposal a
nd help maintain nitrogen homeostasis. However, we report three patients wi
th hyperammonaemia who received inappropriate doses of intravenous sodium b
enzoate and sodium phenylacetate that resulted in severe complications. Amb
iguous medical prescriptions and inadequate cross-checking of drug dosage b
y physicians, nurses and pharmacists were the main causes of these incident
s. All the patients presented with alteration in mental status, Kussmaul re
spiration 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 phen
ylacetate were excessively high. The possible mechanisms of toxicity, manag
ement and safety measures are discussed.