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Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases

1
International Center for the Assessment of Nutritional Status (ICANS), Department of Food Environmental and Nutritional Sciences (DeFENS), University of Milan, Via Sandro Botticelli 21, 20133 Milan, Italy
2
Human Nutrition and Eating Disorder Research Center, Department of Public Health, Experimental and Forensic Medicine, University of Pavia, Via Agostino Bassi 21, 27100 Pavia, Italy
3
Pediatric Neurology Unit, “V. Buzzi” Hospital, Via Lodovico Castelvetro 32, 20154 Milan, Italy
*
Author to whom correspondence should be addressed.
Nutrients 2019, 11(5), 1104; https://doi.org/10.3390/nu11051104
Received: 23 March 2019 / Revised: 12 May 2019 / Accepted: 15 May 2019 / Published: 17 May 2019
(This article belongs to the Special Issue Ketogenic Dietary Therapies: Recent Findings and Future Perspectives)
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Abstract

The ketogenic diet (KD) is the first line intervention for glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency, and is recommended for refractory epilepsy. It is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate diet aimed at switching the brain metabolism from glucose dependence to the utilization of ketone bodies. Several variants of KD are currently available. Depending on the variant, KDs require the almost total exclusion, or a limited consumption of carbohydrates. Thus, there is total avoidance, or a limited consumption of cereal-based foods, and a reduction in fruit and vegetable intake. KDs, especially the more restrictive variants, are characterized by low variability, palatability, and tolerability, as well as by side-effects, like gastrointestinal disorders, nephrolithiasis, growth retardation, hyperlipidemia, and mineral and vitamin deficiency. In recent years, in an effort to improve the quality of life of patients on KDs, food companies have started to develop, and commercialize, several food products specific for such patients. This review summarizes the foods themselves, including sweeteners, and food products currently available for the ketogenic dietary treatment of neurological diseases. It describes the nutritional characteristics and gives indications for the use of the different products, taking into account their metabolic and health effects. View Full-Text
Keywords: ketogenic diet; food; epilepsy; glucose transporter 1 deficiency syndrome; pyruvate dehydrogenase deficiency; children; adult ketogenic diet; food; epilepsy; glucose transporter 1 deficiency syndrome; pyruvate dehydrogenase deficiency; children; adult
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Leone, A.; De Amicis, R.; Lessa, C.; Tagliabue, A.; Trentani, C.; Ferraris, C.; Battezzati, A.; Veggiotti, P.; Foppiani, A.; Ravella, S.; Bertoli, S. Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases. Nutrients 2019, 11, 1104.

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