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Anaesthesia. 1996 Jun;51(6):575-8.

Anaesthetic management in maple syrup urine disease.

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Department of Anaesthesiology and Reanimation, Hacettepe University, Ankara, Turkey.


Two children with maple syrup urine disease undergoing emergency and elective surgery are reported. The administration of ketamine to a 12-month-old boy without neurological problems and propofol infusion to a 5-year-old girl with a history of convulsions, was uneventful. We believe that a full knowledge of the pathophysiology of maple syrup urine disease and potential peri-operative problems associated with the disease are essential, so that patients are not subjected to unnecessary risks.

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