[Anesthetic management for an emergency operation in a patient with von Gierke disease].

A 45-year-old patient with von Gierke disease was scheduled for emergency operation due to acute appenditis. He had a significant hepatomegaly and was on oral medication for hyperlipemia and hyperlithuria. General anesthesia was induced with thiopental and suxamethonium, and maintained with nitrous oxide, oxygen, sevoflurane and vecuronium. The perioperative course was uneventful, although the patient revealed mild metabolic acidosis.