Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases.
Study Goal
The researchers aimed to review the ketogenic diet's role in neurological diseases, its variants, and the development of specialized food products to improve patient quality of life.
Results Summary
The ketogenic diet is effective for glucose transporter 1 deficiency syndrome, pyruvate dehydrogenase deficiency, and refractory epilepsy, but restrictive variants have low palatability and tolerability, with side effects like gastrointestinal disorders and nutrient deficiencies. Food companies are developing specialized products to mitigate these issues.
Population
Patients with neurological diseases, including glucose transporter 1 deficiency syndrome, pyruvate dehydrogenase deficiency, and refractory epilepsy.
Effective Dosage
Not specified
Duration
Not specified
Interactions
None mentioned
| Intervention | Direction | Endpoint | Population | Dosage | Impact | Claim # |
|---|---|---|---|---|---|---|
ketogenic diet (KD) | neutral | glucose transporter 1 deficiency syndrome | - | - | is the first line intervention for | #1 |
ketogenic diet (KD) | neutral | pyruvate dehydrogenase deficiency | - | - | is the first line intervention for | #2 |
ketogenic diet (KD) | neutral | refractory epilepsy | - | - | is recommended for | #3 |
ketogenic diet (KD) | neutral | brain metabolism | - | - | aimed at switching | #4 |
ketogenic diet (KD) | decrease | carbohydrates | - | - | require the almost total exclusion, or a limited consumption of | #5 |
ketogenic diet (KD) | decrease | cereal-based foods | - | - | total avoidance, or a limited consumption of | #6 |
ketogenic diet (KD) | decrease | fruit and vegetable intake | - | - | reduction in | #7 |
ketogenic diet (KD) | neutral | low variability | - | - | characterized by | #8 |
ketogenic diet (KD) | neutral | low palatability | - | - | characterized by | #9 |
ketogenic diet (KD) | neutral | low tolerability | - | - | characterized by | #10 |
ketogenic diet (KD) | neutral | gastrointestinal disorders | - | - | characterized by side-effects, like | #11 |
ketogenic diet (KD) | neutral | nephrolithiasis | - | - | characterized by side-effects, like | #12 |
ketogenic diet (KD) | neutral | growth retardation | - | - | characterized by side-effects, like | #13 |
ketogenic diet (KD) | neutral | hyperlipidemia | - | - | characterized by side-effects, like | #14 |
ketogenic diet (KD) | neutral | mineral and vitamin deficiency | - | - | characterized by side-effects, like | #15 |
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.