Fatal hyperammonemia in a patient with systemic lupus erythematosus

Haruo Ichikawa, Tetsuki Amano, Kenji Kawabata, Masahiko Kushiro, Jun Wada, Yoshio Nagake, Hirofumi Making

Research output: Contribution to journalArticlepeer-review

8 Citations (Scopus)


We treated a 31-year-old woman with systemic lupus erythematous, renal failure with nephrotic syndrome, and along-standing seizure disorder, who developed severe hyperammonemia with a fatal outcome. Blood chemistry examination did not indicate liver disease, and amino acid concentrations did not suggest a defect in the urea cycle. Discontinuation of anticonvulsant treatment with valproic acid (VPA) failed to bring about improvement. We speculated that hyperammonemia in this case was induced by VPA, and the existence of other underlying factors, including the administration of aspirin and cimetidine, hypoalbuminemia, and renal failure might elevate the concentration of the serum free fraction of VPA.

Original languageEnglish
Pages (from-to)700-703
Number of pages4
JournalInternal Medicine
Issue number8
Publication statusPublished - 1998


  • Aspirin
  • Cimetidine
  • Hemodialysis
  • Hyperammonemic encephalopathy
  • Valproic acid (VPA)

ASJC Scopus subject areas

  • Internal Medicine


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