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目的评估生酮饮食(KD)在难治性癫患儿治疗中的疗效和安全性。方法对KD治疗的难治性癫患儿22例进行前瞻性研究,观察期为12周。男16例,女6例。22例患儿中,婴儿痉挛症13例,Lennox-Gastaut综合征4例,Dravet综合征2例,未分类者3例。按照Johns Hopkins方案的改良方法配制KD。每天监测尿酮体确保酮症状态;记录发作频率和形式以确定起效时间,通过Engel分级评估疗效,KD引入前、引入第4周、引入第12周行血液生化检查并判断KD对难治性癫患儿血糖和血脂的影响,监测并处理患儿不良反应。结果所有病例至少予KD治疗2周,19例至少完成4周,10例至少完成12周。16例患儿出现癫缓解,均于3周内(1～15 d)起效,第1周尤为明显。8例发作曾完全控制,均于5周内(1～32 d)发生。达Ⅰ、Ⅱ级疗效者分别占36.4%(8/22例)、22.7%(5/22例)。性别、年龄、治疗前病程、病因和不同分型对疗效无明显影响。尽管血糖于KD引入第4周时较基础值明显升高,但仍维持在正常范围;三酰甘油和胆固醇于KD引入第12周时较基础值明显升高,超过或接近正常值高限。不良反应主要为可耐受的消化道症状和代谢紊乱。结论KD具有起效迅速、有效率高、不良反应轻微等特点,是一种可行的抗癫方式。
Objective To evaluate the efficacy and safety of ketogenic diet (KD) in the treatment of children with refractory epilepsy. Methods Twenty-two children with intractable epilepsy treated with KD were prospectively studied for 12 weeks. 16 males and 6 females. Of the 22 children, 13 were infantile spasms, 4 were Lennox-Gastaut syndrome, 2 were Dravet syndrome, and 3 were unclassified. KD was formulated according to the modified Johns Hopkins protocol. Daily monitoring of urinary ketone body to ensure the state of ketosis; seizure frequency and form to determine the onset time, by Engel grading to assess the efficacy of KD before the introduction of the first 4 weeks, the introduction of the first 12 weeks of blood biochemical tests and determine KD refractory epilepsy children with blood sugar and blood lipids, monitoring and treatment of children with adverse reactions. Results All cases were treated with KD for at least 2 weeks, 19 cases completed at least 4 weeks and 10 cases completed at least 12 weeks. Epilepsy was relieved in 16 cases, all within 3 weeks (from 1 to 15 days), especially in the first week. Eight episodes of complete control had occurred within 5 weeks (1 to 32 days). Grade Ⅰ and Ⅱ patients accounted for 36.4% (8/22) and 22.7% (5/22) respectively. Sex, age, pre-treatment course, etiology and different typing had no significant effect on the curative effect. Although blood glucose significantly increased from baseline at the 4th week of KD, it remained within the normal range. Triglyceride and cholesterol significantly increased from the baseline at the 12th week of KD introduction, exceeding or approaching the upper limit of normal. Adverse reactions are mainly tolerable gastrointestinal symptoms and metabolic disorders. Conclusion KD has the characteristics of quick onset, high efficiency and mild adverse reactions, which is a feasible anti-epileptic method.