ObjectiveTo investigate the current situation of ketogenic diet treatment centers in China, including the target, quantity, indication diseases, staffing and regional distribution of ketogenic diet therapy centers.MethodsMembers from the China Association Against Epilepsy (CAAE) received an investigation on the development situation of ketogenic diet therapy in their own epilepsy centers through an online survey. ResultsAt present, there are 91 centers for ketogenic diet therapy in China, and among them, there are 74 centers (81.32%) only treat pediatric patients. 59 centers of them (64.83%) were established between 2010 and 2020; patients of 63 centers (69.23%) have been treated with ketogenic therapy for status epilepticus (SE), among which only 9 centers (14.29%) have 5 or more epileptic patients on averge per month. 35 centers (38.46%) are offering ketogenic diet treatment for indications other than epilepsy. The main diseases are autism spectrum disorder, encephalitis, febrile infection related epilepsy syndrome (FIRES), rare diseases and glycolipid metabolic diseases. All ketogenic diet treatment centers are equipped with high-quality professionals, mainly neurologists and pediatric physicians; There are significant differences in regional distribution, with centers in the eastern region accounting for more than half (53.85%). ConclusionsThe development of ketogenic diet therapy in China has achieved initial results, but there are still some problems such as small number of patients treated and uneven geographical distribution.
Objective To investigate the compliance of ketogenic diet in children with refractory epilepsy and its correlation with the curative effect, and to provide support and basis for the implementation and improvement of the long-term management of ketogenic diet in this patient population. MethodsA total of 106 children and their families who were followed up on ketogenic diet in the Department of Pediatrics of Fudan University from March 2019 to January 2022 in the Department of Ketogenic Multidisciplinary Treatment (MDT) were selected by convenience sampling method. General data questionnaire and ketogenic compliance questionnaire were used for investigation and follow-up. ResultsThe mean compliance of ketogenic diet in refractory epilepsy children was (13.27±3.68). The compliance scores of ketogenic children with different therapeutic effects and cognitive functions were significantly different. The compliance score was significantly correlated with the therapeutic effect and cognitive level, that is, the higher the therapeutic effect of ketogenic diet in children with higher compliance score, the better the cognitive improvement. ConclusionThe compliance of ketogenic diet in children with refractory epilepsy needs to be further improved. Improving the compliance of ketogenic diet is of great significance for the efficacy of ketogenic diet. Medical staff should actively develop the corresponding intervention program and follow-up management mode to further improve the treatment compliance of children's families, improve the treatment effect and improve the quality of life of children's families.
ObjectiveTo compare the efficacy and compliance of children children with refractory epilepsy receiving ketogenic diet (KD) in outpatient department with children receiving KD treatment in inpatient department. MethodsA retrospective study of 44 children with intractable epilepsy receiving the modified classical ketogenic diets in outpatient department from June 2014 to December 2015, who were followed-up during the third, sixth and twelfth month. Records of epileptic seizures and adverse reactions were used to evaluate the efficacy and retention rate of inpatient department KD treatment in children with refractory epilepsy, and compared with 104 children receiving KD treatment in inpatient department at the same period. ResultsThirty-four of the forty-four children comleted observation after 12-month follow-up, 15 cases had been seizure freedom, 22 cases had more than 50% reduction in seizure frequency, 12 patients had less than 50% reduction in seizure frequency.The total effective rate of the KD therapy in outpatient department was 64.7%, and the retention rate was 71%. 18 of of the 104 children with KD treatment in inpatient department at the same period comleted observation after 12-month follow-up, 3 cases had been seizure freedom, 5 cases had more than 50% reduction in seizure frequency, 13 cases had less than 50% reduction in seizure frequency.The total effective rate of the KD therapy in inpatient department was 27.8%, and the retention rate was 17.3%. ConclusionThe KD therapy in outpatient department is effective to children with intractable epilepsy, and there is a highly efficacy and compliance of children receiving KD in outpatient department comparing with children receiving KD in inpatient department. Therefore, it's optional to children with refractory epilepsy who can't received KD by inpatient department because of insufficient number of beds.
ObjectiveKetogenic diet (KD) has shown promising efficacy in the treatment of super-refractory status epilepticus (SRSE); however, its adverse effects have not been systematically evaluated. This study aimed to analyze the safety profile of adjunctive KD therapy for SRSE and explore potential risk factors. MethodsProspective data from 13 SRSE patients (3 adolescents, 10 adults; mean age 34.6±18.4 years) at Xuanwu Hospital, Capital Medical University (July 2020–December 2024) who received KD adjunctive therapy after failing conventional treatments were collected. Adverse reactions were observed, and a systematic literature review (up to March 2025) was conducted for meta-analysis. ResultsIn the single-center cohort of 13 patients, common adverse events included gastrointestinal intolerance (53.8%), hematologic and metabolic abnormalities such as thrombocytosis (84.6%), hyperammonemia (76.9%), dyslipidemia (69.2%), and hypocalcemia (69.2%), as well as nutritional deficits including hypoalbuminemia (61.5%), anemia (53.8%), and transient weight loss (61.5%). Most adverse events were transient and reversible with timely adjustments to the KD regimen. The meta-analysis (25 studies, 251 cases; mean age 16.1±19.0 years) revealed a spectrum of major adverse events, including gastrointestinal intolerance (26.7%), hypoglycemia (19.1%), acidosis (17.5%), and hyperlipidemia (12.0%). ConclusionsThe ketogenic diet as adjunctive therapy for super-refractory status epilepticus demonstrates a manageable safety profile. Reported adverse events are primarily confined to gastrointestinal intolerance, metabolic derangements, and nutritional deficits, with notable occurrences of thrombocytosis and hyperammonemia requiring timely clinical management. This study provides critical evidence-based support for KD implementation in SRSE treatment protocols.
ObjectiveAnalyze and compare the differences in the efficacy and adverse reactions of various ketogenic diet (KD) in the treatment of refractory epilepsy in children.MethodsSystematic search of electronic databases, including PubMed, Embase, Ovid MEDLINE, Web of Science and the Central Register of Cochrane Controlled Trials, published in English January 2000 Relevant research from January to August 2020. Results: Finally, 11 articles were included and 781 cases were included. Meta-analysis (NMA) method was used to compare 6 classic ketogenic diets (Classic ketogenic diet, CKD), Gradual ketogenic diet initiation (GRAD-KD), and the first modified Atkins diet of 20 g carbohydrates/d (Initial 20 g of carbohydrate/day of modified Atkins diet, IMAD), modified Atkins diet (MAD), low glycemic index diet (LGID) and medium-chain fatty acid diet (Medium-chain triglyceride diet, MCT) Therapeutic effect and adverse reactions of 3, 6, and 12 months.ResultsFrom the results of the direct comparative analysis, CKD and MAD showed superior clinical efficacy in 50% seizure reduction at 3 months to CAU, and the difference was statistically significant [OR=10.58, 95%CI (3.47, 32.40), P<0.05; OR=11.31, 95%CI (5.04, 25.38), P<0.05]; the clinical efficacy of 90% seizure reduction at 3 months for MAD was superior to that of CAU with statistical significance [OR=4.95, 95%CI (1.90, 12.88), P<0.05]. The results of further network meta-analysis suggested that for the comparison of 50% seizure reduction at 3 months, IMAD, GRAD-KD, CKD, MAD, and MCT were superior to CAU, and the difference was statistically significant [OR=0.03; 95%CI (0.00, 0.30), P<0.05; OR=0.07; 95%CI (0.01, 0.76), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05; OR=0.13; 95%CI (0.03, 0.67), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05]. For the comparison of 90% seizure reduction at 3 months, CKD, GRAD-CK, IMAD, MAD, and MCT were superior to CAU, and the differences were statistically significant [OR=0.05; 95%CI (0.00, 0.31), P<0.05; OR=0.22; 95%CI (0.00, 0.39), P<0.05; OR=0.03; 95%CI (0.00, 0.62), P<0.05; OR=0.12; 95%CI (0.01, 0.60), P<0.05; OR=0.09; 95%CI (0.00, 0.91), P<0.05]. It is suggested in the cumulative probability plot that: the optimal clinical regimen for 50% seizure reduction at 3 months was IMAD (Rank1=0.91), the optimal clinical regimen for 50% seizure reduction at 6 months was CKD (Rank1=0.40), the optimal clinical regimen for 50% seizure reduction at 12 months was MCT (Rank1=0.64); the optimal clinical regimen for 90% seizure reduction at 3 months was IMAD (Rank1=0.94), the optimal clinical regimen for 90% seizure reduction at 6 months was LGIT (Rank1=0.44), and the optimal clinical regimen for 90% seizure reduction at 12 months was MCT (Rank1=0.41); the optimal clinical regimen for seizure reduction at 3 months was GRAD-CK (Rank1=0.46), the optimal clinical regimen for seizure reduction at 6 months was LGIT (Rank1=0.58), and the optimal clinical regimen for seizure reduction at 12 months was CKD (Rank1=0.56). It is suggested in the benefit-risk assessment that among the three KDs (CKD, MAD, MCT) with better 50% and 90% seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.47, CF=0.86); among the two KDs (CKD, MAD) with better seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.45); among the two KDs (CKD, MCT) with better 50% and 90% seizure reduction at 12 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.65).ConclusionsIn this study, IMAD showed the optimal clinical efficacy at 3 months and MCT at 12 months. With stable efficacy and low incidence of adverse reactions in 12 months, CKD was the optimal treatment regimen for children with refractory epilepsy after the comprehensive evaluation.
Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe epileptic encephalopathy characterized by critical illness, complex nursing requirements, the need for multidisciplinary collaboration, and high-intensity care during its acute phase. Based on a review of relevant literature and specific nursing practices, this article summarizes the latest advancements in the acute-phase care of children with FIRES. It focuses on aspects such as the management of status epilepticus, fever care, airway management, nutritional support and ketogenic diet, family support, and multidisciplinary collaboration. The aim is to provide a reference for clinical nursing practices and related research.
ObjectiveTo explore the efficacy of ketogenic diet on developmental and epileptic encephalopathy caused by PIGA gene mutation. Method A retrospective analysis was conducted on patients with developmental and epileptic encephalopathy admitted to Guangdong Sanjiu Brain Hospital from March 2016 to June 2020. Patients with positive PIGA gene mutations were selected, and their clinical characteristics and treatment effects were analyzed. ResultA total of 6 epilepsy patients with positive PIGA gene mutations were collected, all of whom were male. 5 cases were heterozygous mutations originating from the mother, and 1 case was a new mutation. All 6 patients were accompanied by varying degrees of psychomotor developmental delay, various types of epileptic seizures, multifocal discharge on EEG, and varying degrees of brain hypoplasia indicated by cranial MRI. All 6 patients met the criteria for drug-resistant epilepsy and were recommended to undergo ketogenic diet treatment, but three patients were discontinued in the early stages. Among them, Case 3 experienced hyperlipidemia on the fifth day of ketogenic diet and was discontinued, while Case 5 experienced transient hypoglycemia on the second day and the family refused to use it. Case 6: After one week of ketogenic diet, the family members voluntarily stopped using it. Only three patients adhered to a long-term ketogenic diet for more than 2 years. The efficacy of ketogenic diet treatment in cases 1 and 4 was very significant, reaching a seizure free state. Case 2 showed a 50% reduction in seizure frequency after ketogenic diet treatment. Case 4 developed hyperlipidemia after two years of ketogenic diet, and after discontinuing the ketogenic diet for about two months, the blood lipids returned to normal. Comparing patients in the ketogenic group with those in the non ketogenic group, it was found that the ketogenic group had a clear therapeutic effect after treatment. Among them, two patients had no seizures for more than a year and showed significant progress in development compared to before. Two years after ketogenic diet treatment, the EEG showed a significant decrease or disappearance of epileptic discharge compared to before. ConclusionPatients with developmental latency caused by PIGA gene mutations have an early only age, diverse types of sizes, varying degrees of psychomotor developmental delay, and some are compatible by von as possible.
Objective To systematically review the efficacy and safety of a very low-calorie ketogenic diet (VLCKD) in patients who were overweight or obese. Methods From inception to August 2021, the electronic databases PubMed, EMbase, Web of Science, The Cochrane Library, CNKI, WanFang Data, VIP, and CBM were searched for randomized controlled trials (RCTs) of VLCKD in patients with overweight or obesity. Two reviewers independently screened the literature, extracted data, and evaluated the risk of bias of the included studies. Then, meta-analysis was performed using Stata 16.0 software. Results A total of 5 RCTs involving 245 patients were included. Among patients with baseline body mass index (BMI) ≥30 kg/m2, the meta-analysis showed that compared with the control group, VLCKD could significantly reduce the BMI (MD=−0.24, 95%CI −0.39 to −0.08, P<0.05), weight (MD=−7.00, 95%CI −10.48 to −3.53, P<0.05) and waist circumference (MD=−7.40, 95%CI −12.68 to −2.12, P<0.05) . The subgroup analysis results showed that compared with the control diet, VLCKD could significantly reduce the glucose (MD=−9.60, 95%CI −17.52 to −1.69, P<0.05), glycated hemoglobin (MD=−0.24, 95%CI −0.39 to −0.08, P<0.05), insulin resistance index (MD=−0.90, 95%CI −1.08 to −0.73, P<0.05) and triglycerides (MD=−41.42, 95%CI −53.78, −29.06, P<0.05) in patients with type 2 diabetes and with obesity or overweight. In patients with obesity or overweight, VLCKD could increase high-density lipoprotein cholesterol (MD=8.60, 95%CI 0.17 to 17.03, P<0.05) when the intervention lasted longer than 12 months. In patients with obesity or overweight, VLCKD had no effect on insulin, total cholesterol, low-density lipoprotein, urea, creatinine, or uric acid. Patients with VLCKD had a higher rate of adverse events than those in the control groups; however, there was no significant difference in the rate when the intervention lasted longer than 4 months. Conclusion The current evidence shows that VLCKD can reduce BMI, weight, and waist circumference and reduce fasting glucose, HbA1c, insulin resistance index, and triglycerides among patients with type 2 diabetes and with obesity or overweight. However, VLCKD has no effect on insulin, total cholesterol, low-density lipoprotein, urea, creatinine, or uric acid. Due to the limited quantity and quality of the included studies, more high-quality studies are needed to verify the above conclusion.