Kumada Tomohiro, Imai Katsumi, Takahashi Yukitoshi, Nabatame Shin, Oguni Hirokazu
Department of Pediatrics, Shiga Medical Center for Children, Japan.
National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.
Brain Dev. 2018 Mar;40(3):188-195. doi: 10.1016/j.braindev.2017.11.003. Epub 2017 Nov 26.
In Japan, Meiji 817-B (M817-B), a powdered ketogenic milk, has been available since the ketogenic diet was introduced to infants and tube-fed children with medication-resistant epilepsy in the 1980s.
We retrospectively evaluated the efficacy, tolerability, and side effects of the ketogenic diet using M817-B as the main source of daily food intake for patients with epilepsy by sending questionnaires to the members of a subcommittee of the Japan Epilepsy Society that focuses on the proper use of M817-B.
A total of 42 patients were enrolled. Age at the initiation of the diet therapy ranged from 3 to 244 months (median, 32.5 months). Thirty-four patients were fed via tube, and the remaining 8 were fed orally. About 93% of patients were able to continue the diet for 1 month, 74% for 3 months, and 64% for 6 months. The median period of continuation was 16 months. One patient was able to continue as long as 7 years. The ketogenic ratio was maintained at about 3.0. The seizure-free rate and responder (>50% seizure reduction) rate were about 10% and 30-40%, respectively during the 12 months on the diet. Mean serum beta-hydroxybutyrate increased to almost 4 mM at 1 month and was maintained during the diet period. Side effects, which required discontinuation of the diet therapy, occurred in 11 of 42 patients and included hypertonia, weight loss, vomiting, hypoglycemia, metabolic acidosis, and hypokalemia.
M817-B could be used long-term with demonstrated efficacy in seizure reduction, although there are some side effects that may require cessation of the diet therapy.
在日本,自20世纪80年代生酮饮食被引入患有药物难治性癫痫的婴儿和管饲儿童以来,明治817 - B(M817 - B),一种粉末状生酮奶粉,就已上市。
我们通过向日本癫痫协会一个专注于正确使用M817 - B的小组委员会成员发送问卷,回顾性评估了以M817 - B作为癫痫患者日常食物摄入主要来源的生酮饮食的疗效、耐受性和副作用。
共纳入42例患者。饮食治疗开始时的年龄范围为3至244个月(中位数为32.5个月)。34例患者通过管饲喂养,其余8例经口喂养。约93%的患者能够持续饮食1个月,74%持续3个月,64%持续6个月。持续的中位时间为16个月。1例患者能够持续长达7年。生酮比例维持在约3.0。在饮食的12个月期间,无癫痫发作率和反应者(癫痫发作减少>50%)率分别约为10%和30 - 40%。平均血清β - 羟基丁酸在1个月时升至近4 mM,并在饮食期间维持。42例患者中有11例出现需要停止饮食治疗的副作用,包括张力亢进、体重减轻、呕吐、低血糖、代谢性酸中毒和低钾血症。
M817 - B可长期使用,在减少癫痫发作方面有显著疗效,尽管存在一些可能需要停止饮食治疗的副作用。