Fatal propofol infusion syndrome in association with ketogenic diet
Fatal propofol infusion syndrome in association with ketogenic diet
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DOI：
10.1055/s-2004-820992
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发表时间：
2004-08-01
期刊：
影响因子：
1.4
通讯作者：
Peters, J
中科院分区：
文献类型：
--
作者：
Baumeister, FAM;Oberhoffer, R;Peters, J
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经常致命的并发症，即丙泊酚输注综合征。其特征是代谢性酸中毒、脂血、横纹肌溶解和心肌衰竭。异丙酚输注综合征是由脂肪酸氧化受损引起的。除了抗惊厥药，生酮饮食，高脂肪，低碳水化合物，足够的蛋白质饮食，是一种有效的治疗难以控制的癫痫发作。我们报告一个10岁的男孩患有灾难性癫痫，谁开发致命的异丙酚输注综合征时，生酮饮食开始。丙泊酚等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。
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.