Long-term follow-up of nutritional status in children with GLUT1 Deficiency Syndrome treated with classic ketogenic diet: a 5-year prospective study

Frontiers in Nutrition

Nutrition

Valentina De Giorgis¹  Anna Tagliabue²  Cinzia Ferraris²  Chiara Lessa³  Alessandro Leone³  Andrea Foppiani³  Alberto Battezzati⁴  Marta Pellizzari⁵  Simona Bertoli⁵  Ramona De Amicis⁵  Sara Olivotto⁶  Roberto Previtali⁷  Pierangelo Veggiotti⁷ 

[1] Department of Child Neurology and Psychiatry, IRCCS “C. Mondino” National Neurological Institute, Pavia, Italy;Human Nutrition and Eating Disorder Centre, University of Pavia, Pavia, Italy;Ketogenic Metabolic Therapy Laboratory, Department of Public Health, Experimental and Forensic Medicine University of Pavia, Pavia, Italy;ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy;ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy;Clinical Nutrition Unit, Department of Endocrine and Metabolic Medicine, IRCCS Istituto Auxologico Italiano, Milan, Italy;ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy;Obesity Unit and Laboratory of Nutrition and Obesity Research, Department of Endocrine and Metabolic Diseases, IRCCS Istituto Auxologico Italiano, Milan, Italy;Pediatric Neurology Unit, “V. Buzzi” Hospital, Milan, Italy;Pediatric Neurology Unit, “V. Buzzi” Hospital, Milan, Italy;Biomedical and Clinical Sciences Department, University of Milan, Milan, Italy;

关键词: GLUT1-Deficiency Syndrome;    ketogenic diet;    long-term effect;    nutritional status;    body composition;    energy expenditure;   

DOI  :  10.3389/fnut.2023.1148960

 received in 2023-01-20, accepted in 2023-05-09,  发布年份 2023

来源: Frontiers

【 摘 要 】

IntroductionThe classic ketogenic diet (cKD) is an isocaloric, high fat, low-carbohydrate diet that induces the production of ketone bodies. High consumption of dietary fatty acids, particularly long-chain saturated fatty acids, could impair nutritional status and increase cardiovascular risk. The purpose of this study was to evaluate the long-term effects of a 5-year cKD on body composition, resting energy expenditure, and biochemical parameters in children affected by Glucose Transporter 1 Deficiency Syndrome (GLUT1DS).MethodsThis was a prospective, multicenter, 5-year longitudinal study of children with GLUT1DS treated with a cKD. The primary outcome was to assess the change in nutritional status compared with pre-intervention, considering anthropometric measurements, body composition, resting energy expenditure, and biochemical parameters such as glucose and lipid profiles, liver enzymes, uric acid, creatinine, and ketonemia. Assessments were conducted at pre-intervention and every 12 months of cKD interventions.ResultsKetone bodies increased significantly in children and adolescents, and remained stable at 5 years, depending on the diet. No significant differences were reported in anthropometric and body composition standards, as well as in resting energy expenditure and biochemical parameters. Bone mineral density increased significantly over time according to increasing age. Body fat percentage significantly and gradually decreased in line with the increase in body weight and the consequent growth in lean mass. As expected, we observed a negative trend in respiratory quotient, while fasting insulin and insulin resistance were found to decrease significantly after cKD initiation.ConclusionLong-term adherence to cKD showed a good safety profile on anthropometric measurements, body composition, resting energy expenditure, and biochemical parameters, and we found no evidence of potential adverse effects on the nutritional status of children and adolescents.

【 授权许可】

Unknown   
Copyright © 2023 De Amicis, Leone, Pellizzari, Foppiani, Battezzati, Lessa, Tagliabue, Ferraris, De Giorgis, Olivotto, Previtali, Veggiotti and Bertoli.