[Patient-controlled sedation using propofol for a patient with von Gierke disease].

Patient-controlled sedation (PCS) using propofol under spinal anesthesia in transurethral lithotripsy was carried out in a 44 year old patient with von Gierke disease accompanied with liver dysfunction, chronic renal failure, hypoglycemia and metabolic acidosis. After administering spinal anesthesia PCS was started (0.2 mg.kg-1 intravenous bolus dose of propofol; infusion at 2 mg.kg-1.h-1; a three-minute lockout time interval following an initial doses of 0.4 mg.kg-1). PCS with propofol, throughout the operation, brought about adequate sedation level for this patient with 2 or 3 on Wilson's sedation score, and the sedative effect by propofol diminished quickly within 15 minutes after the end of PCS. In addition, respiratory depression due to this sedation which would be worse in acidotic condition was not seen using PCS during the operation. This patient was much satisfied with this sedation in an interview during the postoperative period. PCS using propofol is a useful method without respiratory depression for a patient with von Gierke disease.