Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease)
De Vivo disease is characterized by early epileptic encephalopathy, delayed psychomotor development, spasticity, the formation of microcephaly, ataxia, dysarthria, alternating hemiplegia, and a decrease in glucose and lactate levels in the cerebrospinal fluid. Epilepsy is pharmacoresistant and the t...
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| Format: | Article |
| Language: | Russian |
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ABV-press
2021-11-01
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| Series: | Русский журнал детской неврологии |
| Subjects: | |
| Online Access: | https://rjdn.abvpress.ru/jour/article/view/377 |
| _version_ | 1826557979357347840 |
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| author | N. G. Lyukshina |
| author_facet | N. G. Lyukshina |
| author_sort | N. G. Lyukshina |
| collection | DOAJ |
| description | De Vivo disease is characterized by early epileptic encephalopathy, delayed psychomotor development, spasticity, the formation of microcephaly, ataxia, dysarthria, alternating hemiplegia, and a decrease in glucose and lactate levels in the cerebrospinal fluid. Epilepsy is pharmacoresistant and the therapy for this syndrome is the ketogenic diet (until the time when will development of genetic targeted therapy). In GLUT1 deficiency syndrome, mutations are found in the SLC2A1 gene that lead to a decrease in glucose transport across the cell membrane. The “classic” ketogenic diet is a special high-fat, low-carbohydrate diet that helps to control seizures in some people with epilepsy. It is prescribed by a physician and carefully monitored by a dietitian. It is usually used in children with seizures that do not respond to medications. It is stricter than the modified Atkins diet, requiring careful measurements of calories, fluids, and proteins. Foods are weighed and measured. Normal dietary fats, which are used predominantly in the classical ketogenic diet, consist of a mixture of mainly long chain triglyceride (LCT) fats with a small amount of short and medium chain triglyceride (MCT) fats. The MCT ketogenic diet uses a fat supplement that consists only of MCT fats (MCT oil). |
| first_indexed | 2024-04-10T01:31:45Z |
| format | Article |
| id | doaj.art-9e48dfad8a774336ad75ec5bcf7db9c6 |
| institution | Directory Open Access Journal |
| issn | 2073-8803 2412-9178 |
| language | Russian |
| last_indexed | 2025-03-14T08:37:12Z |
| publishDate | 2021-11-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Русский журнал детской неврологии |
| spelling | doaj.art-9e48dfad8a774336ad75ec5bcf7db9c62025-03-02T12:58:02ZrusABV-pressРусский журнал детской неврологии2073-88032412-91782021-11-01163636810.17650/2073-8803-2021-16-3-63-68255Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease)N. G. Lyukshina0MIDEAL LLCDe Vivo disease is characterized by early epileptic encephalopathy, delayed psychomotor development, spasticity, the formation of microcephaly, ataxia, dysarthria, alternating hemiplegia, and a decrease in glucose and lactate levels in the cerebrospinal fluid. Epilepsy is pharmacoresistant and the therapy for this syndrome is the ketogenic diet (until the time when will development of genetic targeted therapy). In GLUT1 deficiency syndrome, mutations are found in the SLC2A1 gene that lead to a decrease in glucose transport across the cell membrane. The “classic” ketogenic diet is a special high-fat, low-carbohydrate diet that helps to control seizures in some people with epilepsy. It is prescribed by a physician and carefully monitored by a dietitian. It is usually used in children with seizures that do not respond to medications. It is stricter than the modified Atkins diet, requiring careful measurements of calories, fluids, and proteins. Foods are weighed and measured. Normal dietary fats, which are used predominantly in the classical ketogenic diet, consist of a mixture of mainly long chain triglyceride (LCT) fats with a small amount of short and medium chain triglyceride (MCT) fats. The MCT ketogenic diet uses a fat supplement that consists only of MCT fats (MCT oil).https://rjdn.abvpress.ru/jour/article/view/377developmental encephalopathyepilepsygeneticsglut1 deficiencydevelopmental delayketogenic dietmct |
| spellingShingle | N. G. Lyukshina Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) Русский журнал детской неврологии developmental encephalopathy epilepsy genetics glut1 deficiency developmental delay ketogenic diet mct |
| title | Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) |
| title_full | Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) |
| title_fullStr | Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) |
| title_full_unstemmed | Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) |
| title_short | Experience with the use of a ketogenic diet with type 1 glucose transporter deficiency (De Vivo disease) |
| title_sort | experience with the use of a ketogenic diet with type 1 glucose transporter deficiency de vivo disease |
| topic | developmental encephalopathy epilepsy genetics glut1 deficiency developmental delay ketogenic diet mct |
| url | https://rjdn.abvpress.ru/jour/article/view/377 |
| work_keys_str_mv | AT nglyukshina experiencewiththeuseofaketogenicdietwithtype1glucosetransporterdeficiencydevivodisease |