生酮饮食添加治疗难治性癫（癇）36例

Clinical observation of ketogenic diet adding for refractory epilepsy in 36 cases of children

目的 观察生酮饮食（KD）添加治疗儿童难治性癫（癇）的临床疗效、保留率、不良反应及其对认知功能的影响,并比较住院治疗组与门诊治疗组不同实施方案的临床疗效及保留率.方法 选择2012年5月至2013年10月在郑州大学第三附属医院河南省小儿脑瘫康复治疗中心接受添加治疗的36例难治性癫（癇）患儿,包括住院治疗组及门诊治疗组,住院治疗组KD方案采用经典模式,即启动时禁食,脂肪和蛋白质、碳水化合物比例为4∶1;门诊治疗组KD方案无需禁食,脂肪和蛋白质、碳水化合物比例由1∶1逐渐升高至4∶1,疗效的评估以KD添加治疗前癫（癇）发作频率为基线,通过家长记录癫（癇）日记,了解癫（癇）发作的频率、类型、每次发作持续时间、发作程度变化,并于添加KD治疗前及KD治疗3、6、12个月行Gesell发育量表评定.结果 住院治疗组KD治疗3、6、12个月总有效率分别为60.0％、45.0％、40.0％,保留率分别为80.0％、55.0％、40.0％,完全控制率30.0％;门诊治疗组3、6、12个月总有效率分别为37.5％、25.0％、18.8％,保留率分别为62.5％、37.5％、18.8％,完全控制率12.5％;住院治疗组与门诊治疗组临床疗效比较差异有统计学意义（P＜0.05）,保留率比较差异无统计学意义（P＞0.05）;住院治疗组短期不良反应主要表现为胃肠道反应,长期不良反应为肾结石1例,门诊治疗组不良反应多轻微;36例难治性癫（癇）患儿认知功能改善共9例,住院治疗组6例,门诊治疗组3例,五大能区发育商多组间比较大运动（F=3.287,P=0.025）及适应性能区（F=8.335,P=0.000）差异有统计学意义.结论 KD添加治疗儿童难治性癫（癇）住院治疗与门诊治疗均安全有效;住院治疗组的疗效较门诊治疗组好,2组保留率相当;提高患者依从性仍是KD治疗成败的重要因素;KD治疗对难治性癫（癇）患儿的认知功能有所改善,主要表现在大运动及适应性能区.

Objective To observe the clinical efficacy,retention rates,adverse reactions of the ketogenic diet （KD） and their effects on cognitive function in children with refractory epilepsy,and to compare the clinical efficacy and retention rates of in-patient group and out-patient group.Methods This study enrolled 36 patients with refractory epilepsy who received KD therapy in Rehabilitation Center for Children with Cerebral Palsy in Henan Province of the Third Affiliated Hospital of Zhengzhou University from May 2012 to Oct.2013.They were divided into in-patient group and out-patient group.The in-patient group received classic KD therapy which started with fasting,the ratio of lipid to non-lipid was 4 ∶ 1 ;while the out-patient group was not asked for fasting,and lipid to non-lipid ratio gradually increased to 4 ∶ 1.Seizure frequency before treatment was set as the baseline,the clinical efficiency was evaluated,changes in seizure frequency,type,degree and the duration of each episode were recorded during KD therapy,and Gesell Developmental Scale assessment was respectively performed prior to KD therapy,3,6,12 months after KD therapy.Results The total effective rates in the in-patient group were 60.0%,45.0%,40.0%,respectively after 3,6,12 months of therapy,the retention rates were 80.0%,55.0%,and 40.0%,respectively,and the complete control rate was 30% ;while the total effective rates in out-patient group were 37.5%,25.0%,and 18.8%,respectively after 3,6,12 months of treatment,the retention rates were 62.5 %,37.5 %,18.8%,respectively,and the complete control rate was 12.5 % ;the in-patient group showed a significant difference compared with the out-patient group in the clinical efficacy （P 〈 0.05）,two groups had no significant difference in retention rate （P 〉 0.05）,short-time adverse effects in the in-patient group were mainly gastrointestinal reactions and long-term adverse effects were mainly kidney stones,adverse effects in outpatient group were striking;9 patients had cognitive improvement,gross motor （F =3.287,P =0.025） and adaptability （F =8.335,P =0.000） were compared among different groups and the difference was significant.Conclusions KD therapy for children with refractory epilepsy are safe and effective in both out-patients and in-patients;the in-patient group had better efficacy;two groups had no significant difference in retention rate,and the improvement of patient compliance is still an important factor for the success of KD therapy.KD therapy can improve cognitive function of children with refractory epilepsy,mainly in gross motor and adaptability.