[Type II citrullinemia triggered by acetaminophen].

We report a 19-year-old man with type II citrullinemia triggered by the administration of acetaminophen when he caught a cold. He was admitted to our hospital of impairment of consciousness and abnormal behaviors. On admission he was comatose and laboratory data revealed respiratory alkalosis which was probably induced by hyperammonemia. Liver dysfunction was mild. Plain CT scans showed brain edema, and EEG revealed triphasic waves. Analysis of plasma and urine amino acids showed a significant increase in citrulline and the activity of argininosuccinate synthetase was markedly reduced in a liver biopsy specimen. Histopathology of the liver revealed inflammatory changes which was probably induced by acetaminophen. Branched chain amino acids transfusion was effective for consciousness disturbance, and sodium benzoate was effective for hyperammonemia. We reviewed 28 patients reported in the literature from 1981 to 1992 to evaluate the probable triggering episodes in type II citrullinemia. Four categories of probable trigger were found, which included 1) stress, 2) liver dysfunction, 3) alcohol, and 4) drugs. Avoidance of these triggers, early diagnosis, and treatment seem most important for the good prognosis and the quality of life.