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We report an anesthetic experience with ornithine transcarbamylase deficiency. The patient was 26 years of age who underwent open biopsy of the liver for a definitive diagnosis. OTC deficiency is an X-linked disorder of urea cycle. One of the anesthetic problems is how to control the blood ammonia level resulting in neurological damage which occurs following anesthesia and surgery. Recently it has been advocated that benzoate is effective to prevent hyperammonemia in patients with OTC deficiency. As patients with this disease are frequently complicated with hepatic dysfunction, anesthetics which may cause hepatic damage such as halothane should be avoided.
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