Baumeister F A M, Oberhoffer R, Liebhaber G M, Kunkel J, Eberhardt J, Holthausen H, Peters J
Children's Hospital of the Technical University Munich, Munich, Germany.
Neuropediatrics. 2004 Aug;35(4):250-2. doi: 10.1055/s-2004-820992.
Propofol is used for the treatment of refractory status epilepticus. When given as a long-term infusion propofol may cause a rare but frequently fatal complication, the propofol infusion syndrome. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. Propofol infusion syndrome is caused by impaired fatty acid oxidation. Beside anticonvulsants the ketogenic diet, a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. We report a 10-year-old boy with catastrophic epilepsy, who developed fatal propofol infusion syndrome when a ketogenic diet was initiated. Substances like propofol which impair fatty acid oxidation may pose an increased risk if combined with ketogenic diet.
丙泊酚用于治疗难治性癫痫持续状态。当长期输注丙泊酚时，可能会引起一种罕见但常致命的并发症——丙泊酚输注综合征。其特征为代谢性酸中毒、脂血症、横纹肌溶解和心肌衰竭。丙泊酚输注综合征是由脂肪酸氧化受损引起的。除抗惊厥药外，生酮饮食，即高脂、低碳水化合物、适量蛋白质饮食，是治疗难治性癫痫的有效方法。我们报告一名10岁患有严重癫痫的男孩，在开始生酮饮食时发生了致命的丙泊酚输注综合征。像丙泊酚这样损害脂肪酸氧化的物质，如果与生酮饮食联合使用，可能会增加风险。
