生酮饮食疗法在难治性癫痫中的应用  

Application of ketogenic diet therapy in refractory epilepsy

作　　者：廖建湘[1] 

机构地区：[1]重庆医科大学附属深圳儿童医院癫痫中心,广东深圳518038

出　　处：《中国实用儿科杂志》2016年第1期41-45,共5页Chinese Journal of Practical Pediatrics

摘　　要：我国从2004年开始应用生酮饮食（KD）治疗癫痫，其适应证包括葡萄糖载体蛋白1（GLUT-1）缺陷症、丙酮酸脱氢酶缺陷症（PDHD），难治性癫痫如婴儿痉挛、Dra—vet综合征、肌阵挛-失张力癫痫（Doose综合征）、结节性硬化症合并癫痫、Landau—Kleffner综合征等，以及难治性癫痫持续状态。KD治疗的禁忌证包括脂肪代谢途径先天缺陷、卟啉病，以及难以配合的患者。KD治疗前应行咨询和评估，治疗时需注意食物选择、制备以及适用年龄、地区等问题。从普通饮食过渡到KD需要1～2周，2：1—4：1的配比饮食可产生有临床治疗作用的酮症状态。可采用药物与KD联合治疗。KD治疗时建议补充其他营养素。推荐至少尝试3个月。有效的患者维持治疗2年左右。从KD恢复到普通饮食通常需2～3个月。KD治疗需要密切随访和评估，临床实践认为KD治疗安全。The ketogenic diet （KD） has been used in the treatment of epilepsy in China mainland since 2004. Indications include glucose transporter protein 1 （GLUT-1 ） deficiency, pyruvate dehydrogenase deficiency （PDHD） , myoclonus astatic epilepsy （Doose syndrome）, tuberous sclerosis complicated with epilepsy, Rett syndrome, Dravet syndrome, infant spasms, Landau Kleffner syndrome, Lafora disease, and superrefractory status epilepticus. The contraindications include fat metabolic pathway inborn errors, porphyrins diseases, and patients who are not able to cooperate with. The KD therapy complications are less, and by conservative treatment most are reversible. Transition from ordinary diet to KD often lasts 1--2 weeks, 2： 1--4：1 diet can produce ketosis of clinical therapeutic effect. It is recommended that KD might be tried at least for three months. Good responders should maintain the therapy for 2 years or so. It often takes 2 to 3 months for KD to turn back to normal diet. KD therapy should be combined with close follow-up and essessment, and it is considered safe in clinical practice.

关 键 词：生酮饮食 癫痫 难治性 合并症 

分 类 号：R72[医药卫生—儿科]