Qiao Xiaoying, Ye Zimeng, Wen Jialun, Lin Sufang, Cao Dezhi, Chen Li, Zou Dongfang, Zou Huafang, Zhang Man, Chen Zhibin, Kwan Patrick, Scheffer Ingrid E, Qin Jiong, Liao Jianxiang
Department of Neurology, Shenzhen Children's Hospital, Shenzhen, 518038, Guangdong, China.
Department of Medicine (Austin Hospital), University of Melbourne, Melbourne, Victoria, 3084, Australia.
Acta Epileptol. 2025 Feb 7;7(1):10. doi: 10.1186/s42494-024-00199-8.
The ketogenic diet (KD) therapy is a primary treatment for drug-resistant epilepsy, and beta-hydroxybutyrate (BHB) is the main ketone produced during KD. However, the pattern of increase in BHB levels is not well understood, and the reference range for BHB need to be defined. The aim of this study was to evaluate the BHB levels in the first three months, especially one week, after KD initiation, and to explore the physiological reference range for BHB.
In our study, a fasting initiation strategy was used for the majority of patients (252/300, 84%) who underwent fasting for 24-48 h, the rest fasted for at least 12 h. The concentration of blood BHB was measured four times a day during the first week, at one month and three months. Seizure frequency was recorded at one week, one month and three months. Responders were defined as those with a seizure reduction 50% or more compared to baseline. BHB levels were compared between responders and non-responders. The BHB levels of responders were used to calculate the reference range.
A total of 300 patients were recruited, of whom 172 (57%) had accessible BHB data. BHB levels rapidly rose to 2.0 mmol/L at 19 h, peaked at 4.2 mmol/L at 43 h of therapy, and stabilized by three months. The reference range for BHB was 1.1 to 4.9 mmol/L.
BHB levels increased rapidly following fasting, reaching the peak at day 2, stabilizing from the end of the first week through three months. The lower reference limit for BHB to ensure KD efficacy should be set at 1.1 mmol/L.
生酮饮食（KD）疗法是耐药性癫痫的主要治疗方法，β-羟基丁酸（BHB）是KD期间产生的主要酮体。然而，BHB水平升高的模式尚不清楚，且BHB的参考范围有待确定。本研究的目的是评估KD开始后头三个月，尤其是第一周的BHB水平，并探索BHB的生理参考范围。
在我们的研究中，大多数患者（252/300，84%）采用禁食启动策略，禁食24 - 48小时，其余患者至少禁食12小时。在第一周、一个月和三个月期间，每天测量四次血BHB浓度。在一周、一个月和三个月时记录癫痫发作频率。缓解者定义为与基线相比癫痫发作减少50%或更多的患者。比较缓解者和未缓解者的BHB水平。使用缓解者的BHB水平计算参考范围。
共招募了300名患者，其中172名（57%）有可获取的BHB数据。BHB水平在19小时时迅速升至2.0 mmol/L，在治疗43小时时达到峰值4.2 mmol/L，并在三个月时稳定下来。BHB的参考范围为1.1至4.9 mmol/L。
禁食后BHB水平迅速升高，在第2天达到峰值，从第一周结束到三个月稳定。确保KD疗效的BHB下限参考值应设定为1.1 mmol/L。
