Effects of long-term ketogenic diet on glucose metabolism, lipid profile and adipocytokines in epileptic children with Glut1 deficiency syndrome

Purpose: The aim of this study was to evaluate the effects of long-term ketogenic diet (KD) on lipid profile, glucose metabolism and novel adipocytokines in prepubertal children with Glut1 deficiency syndrome (Glut1DS).

Methods: A cross-sectional cohort study was conducted at at the Medical University Innsbruck, Department of Pediatrics IV, Austria, and the University Children's Hospital Essen, Germany. Serum lipids and glucose homeostasis as well as serum leptin and adiponectin concentrations, the soluble leptin receptor (sOB-R), the sOB-R/leptin ratio and the body-mass index were determined by standard methods.

Results: Twenty prepubertal children were included in the study. Ten children (median [range] age 7.2 [4.0 to 12.3] years) were on treatment with the KD and 10 age- and BMI-matched children (8.9 [6.5 to 11.8] years) were on treatment with sulthiame, comprising the control group. Greater ApoA-I (p=0.043) and ApoB (p=0.003) concentrations as well as lower LDL-C/ApoB (p<0.001) and lower HDL-C/ApoA-I (p=0.001) ratios were seen in patients with Glut1DS treated with the KD. Furthermore, children with Glut1DS showed significantly lower fasting serum insulin concentrations (p=0.016) and HOMA-IR (p=0.012) compared to controls. With respect to adipocytokines, leptin concentrations were lower (p=0.023) and the sOB-R/leptin ratio was greater (p=0.022) in patients on KD compared to controls

Conclusion: Our results indicate a differential influence of long-term KD on lipid profile and adipocytokines in prepubertal children with Glut1DS. As expected, glucose metabolism was downregulated, adipocytokine constellation points towards a normal to low normal fat mass. However, serum lipids and apolipoproteins clearly were altered with preponderance of a pro-atherogenic lipoprotein profile, possibly indicating higher risk for the development of future cardiovascular disease.