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生酮饮食和改良阿特金斯饮食疗法治疗遗传病因所致儿童难治性癫痫。

The Ketogenic and Modified Atkins Diet Therapy for Children With Refractory Epilepsy of Genetic Etiology.

机构信息

Mayo Clinic, Rochester.

Mayo Clinic, Rochester.

出版信息

Pediatr Neurol. 2019 May;94:32-37. doi: 10.1016/j.pediatrneurol.2018.12.012. Epub 2018 Dec 29.

DOI:10.1016/j.pediatrneurol.2018.12.012
PMID:30803845
Abstract

BACKGROUND

The ketogenic diet is an accepted treatment modality in refractory childhood epilepsy. In this study, we analyzed the efficacy and tolerability of the ketogenic and modified Atkins diets in children with refractory epilepsy of genetic etiology and studied the effect of the diet on seizure frequency.

METHODS

The records of children with a genetic etiology for refractory epilepsy treated with ketogenic and modified Atkins diet between September 2005 and July 2016 were reviewed. We documented age of seizure and diet onset, seizure characteristics, and specific genetic etiology. The proportion of children remaining on the diet and responder rates (greater than 50% seizure reduction) were noted at one, three, six, 12, and 24 months after diet initiation. Tolerability and safety profile were also recorded.

RESULTS

Fifty-nine children with a genetic etiology (63% females, median age at diet onset 2.2 years) were initiated on the diet at our center. Fifty-three (90%) were started on a traditional ketogenic diet, whereas six started a modified Atkins diet. The adverse events at the initiation of diet were vomiting (24%), hypoglycemia (15%), and refusal to feed (11%). Three children stopped the diet before discharge because of poor compliance, severe reflux, and ketoacidosis (n = 1 each). The proportion of children remaining on the diet at one, three, six, 12, and 24 months was 95%, 86%, 69%, 64%, and 47%. The responder rates were 63%, 61%, 54%, 53%, and 41% at one, three, six, 12, and 24 months, respectively.

CONCLUSIONS

The ketogenic diet is an effective treatment modality in children with refractory epilepsy of genetic etiology.

摘要

背景

生酮饮食是治疗儿童难治性癫痫的一种公认的治疗方法。在这项研究中,我们分析了生酮饮食和改良阿特金斯饮食在遗传性病因难治性癫痫儿童中的疗效和耐受性,并研究了饮食对癫痫发作频率的影响。

方法

回顾了 2005 年 9 月至 2016 年 7 月期间接受生酮饮食和改良阿特金斯饮食治疗的遗传性病因难治性癫痫儿童的病历。我们记录了癫痫发作和饮食开始的年龄、癫痫发作特征和特定的遗传病因。在开始饮食后 1、3、6、12 和 24 个月时,记录了继续饮食的儿童比例和应答率(癫痫发作减少 50%以上)。还记录了耐受性和安全性概况。

结果

59 名遗传性病因(63%为女性,饮食开始时的中位年龄为 2.2 岁)的儿童在我们中心开始接受该饮食。53 名(90%)开始接受传统生酮饮食,6 名开始接受改良阿特金斯饮食。饮食开始时的不良反应有呕吐(24%)、低血糖(15%)和拒食(11%)。由于依从性差、严重反流和酮症酸中毒(各 1 例),有 3 名儿童在出院前停止了饮食。在 1、3、6、12 和 24 个月时,继续饮食的儿童比例分别为 95%、86%、69%、64%和 47%。在 1、3、6、12 和 24 个月时,应答率分别为 63%、61%、54%、53%和 41%。

结论

生酮饮食是遗传性病因难治性癫痫儿童的一种有效治疗方法。

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