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

Leone, Alessandro, De Amicis, Ramona, Lessa, Chiara, Tagliabue, Anna, Trentani, Claudia, Ferraris, Cinzia, Battezzati, Alberto, Veggiotti, Pierangelo, Foppiani, Andrea, Ravella, Simone, Bertoli, Simona
Nutrients 2019 v.11 no.5
adverse effects, brain, digestive system diseases, epilepsy, food industry, fruits, glucose, glucose transporters, grain foods, growth retardation, health effects assessments, hyperlipidemia, ketogenic diet, ketone bodies, low carbohydrate diet, markets, metabolism, nutritional intervention, nutritive value, palatability, patients, pyruvate dehydrogenase (lipoamide), quality of life, renal calculi, sweeteners, vegetable consumption, vitamin deficiencies
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.