生酮饮食添加治疗难治性癫癎长期有效率、保留率分析及不良反应监测

Long-term effective rate, retention rate and tolerability of the ketogenic diet in paediatrics drug-resistant epilepsies

目的 评价生酮饮食（KD）在治疗儿童难治性癫癎过程中的长期有效率、保留率和不良反应情况。方法 前瞻性研究2011年11月至2013年12月在西安交通大学第二附属医院儿内科进行KD 治疗的36例难治性癫癎患者，发作减少50% 以上为有效。评价有效率、保留率，并分析退出 KD治疗的原因、可能有预测价值的临床指标以及不良反应发生情况。结果 36例患儿纳入本研究（男29例，女7例；平均年龄2.84岁）。第1、3、6、12个月的有效率分别为50.0% 、52.8% 、47.2% 及41.7% ；保留率分别为94.4% 、91.2% 、69.4% 和52.8% 。17例患者中途退出 KD，7例（41.2%）因患儿家长医从性差，5例（29.4%）因疗效不佳退出，2例（10.6%）患儿因反复感染退出。年龄、病程、病因及发作类型不能作为预测KD是否能成功的因素；KD 前使用过的抗癫癎药物种类越少，KD成功率越高。启动期的不良反应主要有睡眠增多、乏力、消化道不适、高脂血症、低血糖以及肝功能受损；维持期的不良反应主要有消化道不适、易于感染、高脂血症及微量元素缺乏等。结论 KD是一种安全有效的治疗儿童药物难治性癫癎的措施。

Objective To evaluate the long-term effective rate,retention rate and tolerability of the ketogenic diet （KD） in pediatric drug-resistant epilepsies. Methods Data of 36 children who were treated in Department of Pediatrics,the Second Affiliated Hospital of Xi＇an Jiaotong University from Nov. 2011 to Dec. 2013 and had continuous follow-up of at least 12 months after initiation of the KD were analyzed prospectively. Response was defined as 50% seizure reduction. The effective rate, retention rate,＂ outcome-predictive＂ value of various clinical factors were also as- sessed. The causes of the patients withdrew from KD and side effects were recorded and analyzed. Results Thirty-six children（29 boys ,7 girls;mean age of 2.84 years）were included. The effective rate was 50.0%, 52.8% ,47.2% and 41.7% at the 1,3,6,12 months ; the retention rate respectively was 94.4%, 91.2%, 69.4% and 52.8 %. Seventeen cases withdrew from KD. Seven cases （41.2%） ceased KD because of a lack of compliance,5 patients because of poor outcome, and 2 cases because of repeated infection. None of the age, disease duration, etiology and seizure type could be used as the predictor for the favourable treatment for outcome. The antiepileptic drugs before KD might be predicted the efficacy of the KD. The less amount of the antiepileptic drugs, the more opportunity of the KD might have to succeed. The side effects on the start-up period were drowsiness, week and digestive discomfort, hyperlipoidemia, hypoglycemia and hepatic dysfunction. The side effects on the maintenance period were digestive discomfort, susceptibility to infec- tion,hyperlipoidemia, the deficiency of trace elements. Conclusions The KD is a safe and potentially effective method in treatment of refractory epilepsy patients who do not respond to customary medication therapies.