HYPERAMMONEMIA IN A PATIENT WITH SHORT-BOWEL SYNDROME AND CHRONIC - RENAL-FAILURE

A patient with short bowel syndrome (SBS) and renal failure developed a disturbance of consciousness associated with hyperammonemia. Abnorma lly low concentrations of ornithine, citrulline, and arginine were obs erved on the plasma aminogram. These results suggested that the activi ties of amino acid synthetase localized in the small intestinal flora were lost. The small intestine is required for arginine synthesis; thu s, infusion limited to the essential amino acids to SBS patients will cause a deficiency of the urea cycle intermediates, ornithine, citrull ine, and arginine and may lead to hyperammonemia. In addition, the ren al insufficiency may have caused decreased excretion of ammonia. In th is patient, supplemental arginine improved the symptoms.