FATAL HYPERAMMONEMIA IN A PATIENT WITH SYSTEMIC LUPUS-ERYTHEMATOSUS

We treated a 31-year-old woman with systemic lupus erythematosus, rena l failure with nephrotic syndrome, and a long-standing seizure disorde r, who developed severe hyperammonemia with a fatal outcome. Blood che mistry examination did not indicate liver disease, and amino acid conc entrations 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, includi ng the administration of aspirin and cimetidine, hypoalbuminemia, and renal failure might elevate the concentration of the serum free fracti on of VPA.