Hyperammonemic encephalopathy due to essential amino acid hyperalimentation.

Hyperammonemic encephalopathy has occasionally been reported in uremic patients receiving hyperalimentation with essential amino acid (EAA) as a source of nitrogen as one of the remaining treatment options when the enteric routes were prohibited. We encountered this complication in a patient with normal renal function. A rat animal model was designed to elucidate the mechanism of hyperammonemia resulting from hyperalimentation with EAA as a source of nitrogen. Sixty-four male Long-Evan rats were divided into eight groups receiving feeds ad libitum or different formula of hyperalimentation. Hyperammonemia was found in every rat given hyperalimentation with EAA as the only nitrogen source. Using the Tukey honestly significant difference test, the results were significantly higher (p < 0.001) than that of the control group which were given feeds ad libitum and those groups given hyperalimentation for the same number of days but with mixed amino acid (MAA) as the nitrogen source. Adding arginine to EAA for a further four days after initial administration of EAA hyperalimentation for three days only slightly lowered the mean serum ammonia level. When compared to that of the three-day EAA hyperalimentation group, the difference was not statistically significant. Adding arginine, citrulline, and ornithine to EAA for a further four days significantly lowered the mean serum ammonia level. When we changed EAA hyperalimentation to MAA hyperalimentation for a further four days, the mean serum ammonia level decreased dramatically to nearly normal. Hyperalimentation using EAA as the exclusive source of nitrogen resulted in hyperammonemia. A deficiency of arginine or other amino acids of the urea cycle failed to account completely for the hyperammonemia observed.(ABSTRACT TRUNCATED AT 250 WORDS)