BACKGROUND:This Scandinavian collaborative retrospective study of children treated with ketogenic diet (KD) highlights indications and effectiveness over two years follow-up. METHODS:Five centres specialised in KD collected data retrospectively on 315 patients started on KD from 1999 to 2009. Twenty-five patients who stopped the diet within four weeks because of compliance-problems and minor side-effects were excluded. Seizure-type(s), seizure-frequency, anti-epileptic drugs and other treatments, mental retardation, autism-spectrum disorder and motor-dysfunction were identified and treatment-response was evaluated. RESULTS:An intention-to-treat analysis was used. Responders (>50% seizure-frequency reduction) at 6, 12 and 24 months were 50%, 46% and 28% respectively, seizure-free were 16%, 13% and 10%. Still on the diet were 80%, 64% and 41% after 6, 12 and 24 months. No child had an increased seizure-frequency. The best seizure outcome was seen in the group with not-daily seizures at baseline (n = 22), where 45%, 41% and 32% became seizure-free at 6, 12 and 24 months A significant improvement in seizure-frequency was seen in atonic seizures at three months and secondary generalised seizures at three and six months. Side-effects were noted in 29 subjects; most could be treated and only two stopped due to hyperlipidaemia and two due to kidney-stones. In 167 patients treated with potassium-citrate, one developed kidney-stones, compared with six of 123 without potassium-citrate treatment (relative risk = 8.1). CONCLUSIONS:As the first study of implementing KD in children in the Scandinavian countries, our survey of 290 children showed that KD is effective and well tolerated, even in such severe patients with therapy-resistant epilepsy, more than daily seizures and intellectual disability in the majority of patients. Long-term efficacy of KD was comparable or even better than reported in newer AEDs. Addition of potassium citrate reduced risk of kidney-stones. Our data indicate that the response might be predicted by seizure-frequency before initiation of the diet but not by age, seizure-type or aetiology.

译文

背景:这项对生酮饮食(KD)治疗的儿童的斯堪的纳维亚合作回顾性研究强调了两年随访的适应症和有效性。
方法:五个专门针对KD的中心回顾性收集了从1999年至2009年开始接受KD治疗的315例患者的数据。25例因依从性问题和轻微副作用而在4周内停止饮食的患者被排除在外。确定癫痫发作类型,癫痫发作频率,抗癫痫药和其他治疗方法,智力低下,自闭症谱系障碍和运动功能障碍,并评估治疗反应。
结果:进行了意向治疗分析。在6、12和24个月时,响应者(癫痫发作频率降低> 50%)分别为50%,46%和28%,无癫痫发作的分别为16%,13%和10%。在6、12和24个月后,饮食仍占80%,64%和41%。没有儿童的癫痫发作频率增加。基线水平不是每天发作的组(n = 22)观察到最佳癫痫发作结局,其中65%,41%和32%在6、12和24个月时无癫痫发作。癫痫发作频率有显着改善在三个月的无力性癫痫发作和三个和六个月的继发性全身性癫痫发作中可见。在29名受试者中发现了副作用。大多数可以治疗,只有两个因高脂血症而停药,另外两个因肾结石而停药。在167例接受柠檬酸钾治疗的患者中,有1例发展为肾结石,而123例中有6例未进行柠檬酸钾治疗(相对风险= 8.1)。
结论:作为在斯堪的纳维亚国家对儿童实施KD的第一项研究,我们对290名儿童的调查显示,KD是有效的并且具有良好的耐受性,即使在这种具有治疗耐药性癫痫的严重患者中,KD也比每日癫痫发作和智力障碍更为严重。大多数患者。 KD的长期疗效与更新的AED中报道的相当或什至更好。柠檬酸钾的添加减少了肾结石的风险。我们的数据表明,反应可能是由饮食开始前的癫痫发作频率预测的,而不是年龄,癫痫发作类型或病因学的预测。

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