Anaesthetic management in maple syrup urine disease.
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.
Kahraman
S
S
Department of Anaesthesiology and Reanimation, Hacettepe University, Ankara, Turkey.
Ercan
M
M
Akkuş
O
O
Erçelen
O
O
Erdem
K
K
Coşkun
T
T
D000768:Anesthesia, General / Q000379:methods*; D000771:Anesthesia, Intravenous; D000778:Anesthetics, Dissociative; D018686:Anesthetics, Intravenous; D002245:Carbon Dioxide / Q000097:blood; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007649:Ketamine; D008297:Male; D008375:Maple Syrup Urine Disease / Q000097:blood; D010100:Oxygen / Q000097:blood; D010313:Partial Pressure; D015742:Propofol; D012307:Risk Factors
D000778:Anesthetics, Dissociative; D018686:Anesthetics, Intravenous; D002245:Carbon Dioxide; D007649:Ketamine; D010100:Oxygen; D015742:Propofol