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目的：评价生酮饮食（ketogenic diet, KD）添加治疗对难治性癫痫的有效率、长期保留率和不良反应。方法共有36例难治性癫痫患者进入研究，所有患者住院期间以脂肪与非脂肪比例为4：1饮食开始，出院后继续维持4：1饮食，或根据实际情况适当减低比例，随访观察3个月、6个月、12个月的保留率，并统计疗效和不良反应的发生率。结果36例患儿纳入该研究（男27例，女9例；平均年龄3.25岁）。3个月时有效率为38.9%（14/36）；有10例退出，保留率为72.2%；6个月时有效率为27.8%（10/36），有5例退出，保留率为58.3%；1年时有效率为16.7%（6/36），有9例退出，保留率为33.3%。启动期的不良反应主要有睡眠增加、感觉无力、胃肠道不舒服、高血脂、低血糖以及肝功能异常；维持期的不良反应主要有胃肠道不舒服、容易感染、高脂血症、低血糖及肝功能异常等。结论生酮饮食在我国添加治疗难治性癫痫疗效较高，与国际水平接近，但保留率不高，副作用不是导致治疗失败的主要原因，而提高耐受性和可操作是努力的方向。“,”Objective To evaluate the effective rate, long term retention rate and adverse reactions of ketogenic diet to intractable epilepsy.Methods 36 intractable patients were included in this research, they were started with the diet in which fat and non-fat ratio was 4:1 while hospitalized, and then kept this diet after discharged, or properly decreased the ratio according to actual situation, 3, 6, 12 months retention rates were followed, and rates of curative effect and adverse reactions were counted.Results 36 patients were included in this research (male 27 cases, female 9 cases, average age 3.25 years). Effective rate at 3 months was 38.9% (14/36); 10 cases dropped out, retention rate was 72.2%; effective rate at 6 months was 27.8% (10/36); 5 cases dropped out, retention rate was 58.3%; effective rate at 12 months was 16.7% (6/36); 9 cases dropped out, retention rate was 33.3%. The main adverse reactions in initiation were increased sleep, faint, gastrointestinal intolerance, hyperlipidemia, hypoglycemia and abnormal liver function; adverse reactions in maintenance were gastrointestinal intolerance, vulnerable to infection, hyperlipidemia, hypoglycemia and abnormal liver function.Conclusion Ketogenic diet therapy is an effective treatment to intractable epilepsy, close to international level, but its retention rate is not high, the adverse reactions are not the main cause of treatment failure, increasing the tolerance and operability is what people should work on.