ObjectiveTuberous sclerosis complex (TSC) is a multiorgan disorder and mostly associated with intractable epilepsy. Now several individual reports suggest that epilepsy in children with TSC might benefit from a ketogenic diet (KD). We prospectively studied the curative effect of 14 children with the KD in the treatment of TSC with epilepsy. MethodsBetween 2008 and 2015, we enrolled 14 children with TSC and epilepsy who received KD treatment in Shenzhen Children's Hospital and followed up for at least three months.Outcome was measured by the change of seizure frequency before and after the KD in the use of anticonvusant drugs, adverse effects, and change in cognitive function. Results14 children aged 8 months to 7 years were included. 7/14 (50%) children had a > 50% reduction in seizure frequency at 3 months on the diet, 5/14 (36%) children had a seizure free response. 12/14 (86%) children with refractory epilepsy, 6/12 (50%) children had a > 50% reduction in seizure frequency, 2 children had reduced medications, one child did not use any antiepileptic drugs during KD. 6 of 12 children with developmental delays had cognitive function improvement. ConclusionsKD is a generally effective and safe therapy for TSC children with epilepsy, especially for refractory epilepsy. KD could reduce antiepileptic drugs, and also improve children's cognitive function.
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.
Objective Compare and analyze the value of β-hydroxybutyrate from two kinds of blood ketone instruments (Optium Xceed blood ketone body instrument from American Abbott laboratories and TBS-1 blood ketone body instrument from Beijing Yicheng company), and provide instructions for clinical applications. Methods Select 7 children patients with epilepsy which have a response to ketogenic diet. The two kinds of blood ketone instruments were used to test blood ketone value of whole blood synchronously in five different periods, and compare the 70 results. Results The measuring results obtained from these two kinds of blood ketone body instruments have statistical difference significantly. The value acquired from TBS-1 blood ketone body instrument is lower than that from Optium Xceed blood ketone body instrument. Measurements using a paired t test, the value of t=12.14, P<0.01. The results from two kinds of blood ketone body instruments are statistically significant. Conclusion The values from 2 kinds of blood ketone instruments are different, which would influence the clinical judgment. At present, the key problem is that there is lack of special blood ketone measure technology for patients with ketogenic diet. How to improve the measure accuracy of high-level blood ketone value remains the future work.