靜脈型生酮飲食治療頑固型癲癇重積狀態之回溯性研究

Parenteral Ketogenic Diet for Patients with Refractory Status Epilepticus: A Retrospective Study

黃婉苓(Wan-Ling Huang) ； 林佩燁(Pei-Yeh Lin) ； 林建志(Jainn-Jim Lin) ； 林光麟(Kuang-Lin Lin) ； 王煇雄(Huei-Shyong Wang)

《台灣營養學會雜誌》 45卷4期 (2021/12) Pp. 141-150

https://doi.org/10.6691/NSJ.202112_45(4).0003

摘要

生酮飲食（ketogenic diet）是一種高脂質、低碳水化合物和適量蛋白質的飲食型態，臨床上常用於治療癲癇症。本研究目的在探討頑固型癲癇重積狀態（refractory status epilepticus）無法經由腸道給予生酮飲食時，短期使用靜脈型生酮飲食之療效和安全性。回顧2013年11月至2021年01月期間，於北部某醫學中心兒童重症加護病房，因頑固型癲癇重積狀態接受靜脈型生酮飲食之患者，總計有11位病患（6位男性），年齡中位數為8歲11個月（四分位距，4歲9個月及15歲7個月），治療日數中位數為11.5天（四分位距，6.5及18.5天），最長達37天；治療後達到酮症（ketosis）之中位數為3天（四分位距，2及3天），最長達12天；開始給予腸道生酮飲食之中位數為5.5天（四分位距，2.5及11天），結果高達72%（8/11）患者癲癇發作次數減少50%以上（包括完全沒有發作），產生副作用含高澱粉酶血症45%（5/11）、酸中毒36%（4/11）、重度高三酸甘油酯血症27%（3/11）和低血糖症18%（2/11）等，在調降生酮比例、停止或降低靜脈輸注量後可自行恢復正常。對於因頑固型癲癇重積狀態不適合腸道生酮飲食之患者，給予短期及低比例之靜脈型生酮飲食，可改善癲癇發作，但應小心監測副作用，並於病況改善時，盡早轉換至腸道生酮飲食。

關鍵字

生酮飲食 ； 靜脈型生酮飲食 ； 頑固型癲癇重積狀態

並列摘要

The ketogenic diet (KD) is a high fat, low carbohydrate and moderate amount of protein diet. KD has been used to treat children with refractory epilepsy since the 1920s. The purpose of this study was to investigate the efficacy and safety of parenteral ketogenic diet (PKD) when patients with refractory status epilepticus were unable to receive oral or tube feeding. Eleven patients (6 males) who received PKD in the pediatric intensive care unit at a medical center in northern Taiwan from November 2013 to January 2021 were enrolled in. The median age of PKD initiation was 8 years and 11 months (IQR 4 years and 9 months to 15 years and 7 months). Duration of PKD management was median 11.5 days (IQR 6.5-18.5) with maximum duration up to 37 days; time to achieve ketosis ( -hydroxybutyrate ＞ 2 mmol / L) was median 3 days (IQR 2-3) with maximum duration up to 12 days; to start enteral feeding was median 5.5 days (IQR 2.5-11). 72% (8/11) patients had over 50% seizure reduction (included seizure free). The side effects comprised hyperamylasemia 45% (5/11), severe acidosis 36% (4/11), severe hypertriglyceridemia 27% (3/11) and hypoglycemia 18% (2/11), but it returned to normal after lowering the keto-ratio, stopping or reducing the amount of intravenous infusion. For patients with refractory status epilepticus who are temporarily not suitable for the enteral KD, receiving a short-term and low keto-ratio PKD could reduce seizure attack. Side effects of PKD should be carefully monitored, and switching to enteral KD should be considered as soon as possible.

並列關鍵字

ketogenic diet ； parenteral ketogenic diet ； refractory status epilepticus