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Ketogenic diets

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https://www.readbyqxmd.com/read/27919115/febrile-infection-related-epilepsy-syndrome-clinical-review-and-hypotheses-of-epileptogenesis
#1
Andreas van Baalen, Annamaria Vezzani, Martin Häusler, Gerhard Kluger
Febrile infection-related epilepsy syndrome (FIRES, AERRPS, or DESC) is one of the most severe, mostly irreversible, and presumably immune-mediated epileptic encephalopathies affecting healthy children. Refractory status epilepticus or a cluster of seizures start a few days after the onset of an acute febrile illness; however, encephalitis cannot be proved. Sequelae of FIRES are drug-resistant epilepsy and neuropsychological impairments occurring without latency. Clinical knowledge is limited because FIRES is sporadic and extremely rare...
December 5, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27913098/succinyl-coa-synthetase-sucla2-deficiency-in-two-siblings-with-impaired-activity-of-other-mitochondrial-oxidative-enzymes-in-skeletal-muscle-without-mitochondrial-dna-depletion
#2
Xiaoping Huang, Jirair K Bedoyan, Didem Demirbas, David J Harris, Alexander Miron, Simone Edelheit, George Grahame, Suzanne D DeBrosse, Lee-Jun Wong, Charles L Hoppel, Douglas S Kerr, Irina Anselm, Gerard T Berry
Mutations in SUCLA2 result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) (A-SCS) deficiency, a mitochondrial tricarboxylic acid cycle disorder. The phenotype associated with this gene defect is largely encephalomyopathy. We describe two siblings compound heterozygous for SUCLA2 mutations, c.985A>G (p.M329V) and c.920C>T (p.A307V), with parents confirmed as carriers of each mutation. We developed a new LC-MS/MS based enzyme assay to demonstrate the decreased SCS activity in the siblings with this unique genotype...
November 12, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27899882/tumor-metabolism-the-ketogenic-diet-and-%C3%AE-hydroxybutyrate-novel-approaches-to-adjuvant-brain-tumor-therapy
#3
REVIEW
Eric C Woolf, Nelofer Syed, Adrienne C Scheck
Malignant brain tumors are devastating despite aggressive treatments such as surgical resection, chemotherapy and radiation therapy. The average life expectancy of patients with newly diagnosed glioblastoma is approximately ~18 months. It is clear that increased survival of brain tumor patients requires the design of new therapeutic modalities, especially those that enhance currently available treatments and/or limit tumor growth. One novel therapeutic arena is the metabolic dysregulation that results in an increased need for glucose in tumor cells...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27891164/a-low-carbohydrate-high-fat-diet-decreases-lean-mass-and-impairs-cardiac-function-in-pair-fed-female-c57bl-6j-mice
#4
Jessica Nilsson, Madelene Ericsson, Masoumeh Motamedi Joibari, Fredrick Anderson, Leif Carlsson, Stefan K Nilsson, Anna Sjödin, Jonas Burén
BACKGROUND: Excess body fat is a major health issue and a risk factor for the development of numerous chronic diseases. Low-carbohydrate diets like the Atkins Diet are popular for rapid weight loss, but the long-term consequences remain the subject of debate. The Scandinavian low-carbohydrate high-fat (LCHF) diet, which has been popular in Scandinavian countries for about a decade, has very low carbohydrate content (~5 E %) but is rich in fat and includes a high proportion of saturated fatty acids...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/27884781/epigenetic-mechanisms-underlying-lifespan-and-age-related-effects-of-dietary-restriction-and-the-ketogenic-diet
#5
Cesar L Moreno, Charles V Mobbs
Aging constitutes the central risk factor for major diseases including many forms of cancer, neurodegeneration, and cardiovascular diseases. The aging process is characterized by both global and tissue-specific changes in gene expression across taxonomically diverse species. While aging has historically been thought to entail cell-autonomous, even stochastic changes, recent evidence suggests that modulation of this process can be hierarchal, wherein manipulations of nutrient-sensing neurons (e.g., in the hypothalamus) produce peripheral effects that may modulate the aging process itself...
November 21, 2016: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/27877138/a-ketogenic-diet-in-rodents-elicits-improved-mitochondrial-adaptations-in-response-to-resistance-exercise-training-compared-to-an-isocaloric-western-diet
#6
Hayden W Hyatt, Wesley C Kephart, A Maleah Holland, Petey Mumford, C Brooks Mobley, Ryan P Lowery, Michael D Roberts, Jacob M Wilson, Andreas N Kavazis
Purpose: Ketogenic diets (KD) can facilitate weight loss, but their effects on skeletal muscle remain equivocal. In this experiment we investigated the effects of two diets on skeletal muscle mitochondrial coupling, mitochondrial complex activity, markers of oxidative stress, and gene expression in sedentary and resistance exercised rats. Methods: Male Sprague-Dawley rats (9-10 weeks of age, 300-325 g) were fed isocaloric amounts of either a KD (17 g/day, 5.2 kcal/g, 20.2% protein, 10.3% CHO, 69.5% fat, n = 16) or a Western diet (WD) (20 g/day, 4...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27873289/the-effect-of-ketogenic-diet-on-serum-selenium-levels-in-patients-with-intractable-epilepsy
#7
Nur Arslan, Engin Kose, Orkide Guzel
The aim of the present study was to evaluate serum selenium levels in children receiving olive oil-based ketogenic diet (KD) for intractable seizures for at least 1 year. Out of 320 patients who were initiated on KD, patients who continued receiving KD for at least 12 months were enrolled. Sixteen patients who had selenium deficiency at the time of starting KD were excluded. Finally, a total of 110 patients (mean age 7.3 ± 4.2 years) were included. Serum selenium levels were measured at baseline and at 3, 6, and 12 months after treatment initiation by using atomic absorption spectroscopy...
November 21, 2016: Biological Trace Element Research
https://www.readbyqxmd.com/read/27871516/pediatric-patients-on-ketogenic-diet-undergoing-general-anesthesia-a-medical-record-review
#8
Elif Soysal, Heike Gries, Carter Wray
STUDY OBJECTIVE: To identify guidelines for anesthesia management and determine whether general anesthesia is safe for pediatric patients on ketogenic diet (KD). DESIGN: Retrospective medical record review. SETTING: Postoperative recovery area. PATIENTS: All pediatric patients who underwent general anesthesia while on KD between 2009 and 2014 were reviewed. We identified 24 patients who underwent a total of 33 procedures...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27868454/the-atp-sensitive-k-channel-is-seizure-protective-and-required-for-effective-dietary-therapy-in-a-model-of-mitochondrial-encephalomyopathy
#9
Keri J Fogle, J Ian Hertzler, Joy H Shon, Michael J Palladino
Effective therapies are lacking for mitochondrial encephalomyopathies (MEs). MEs are devastating diseases that predominantly affect the energy-demanding tissues of the nervous system and muscle, causing symptoms such as seizures, cardiomyopathy, and neuro- and muscular degeneration. Even common anti-epileptic drugs which are frequently successful in ameliorating seizures in other diseases tend to have a lower success rate in ME, highlighting the need for novel drug targets, especially those that may couple metabolic sensitivity to neuronal excitability...
November 21, 2016: Journal of Neurogenetics
https://www.readbyqxmd.com/read/27866088/is-ketogenic-diet-treatment-hepatotoxic-for-children-with-intractable-epilepsy
#10
Nur Arslan, Orkide Guzel, Engin Kose, Unsal Yılmaz, Pınar Kuyum, Betül Aksoy, Tansel Çalık
PURPOSE: Long-term ketogenic diet (KD) treatment has been shown to induce liver steatosis and gallstone formation in some in vivo and clinical studies. The aim of this retrospective study was to evaluate the hepatic side effects of KD in epileptic children. METHOD: A total of 141 patients (mean age: 7.1±4.1years [2-18 years], 45.4% girls), receiving KD at least one year for intractable epilepsy due to different diagnoses (congenital brain defects, GLUT-1 deficiency, West syndrome, tuberous sclerosis, hypoxic brain injury, etc...
November 13, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27851461/1826-subtle-symptoms-preceding-fulminant-pancreatitis-and-hyperacidosis-secondary-to-ketogenic-diet
#11
Juan Gutierrez, Colin O'Reilly
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27847463/metabolic-therapy-for-temporal-lobe-epilepsy-in-a-dish-investigating-mechanisms-of-ketogenic-diet-using-electrophysiological-recordings-in-hippocampal-slices
#12
REVIEW
Masahito Jr Kawamura, David N Ruskin, Susan A Masino
The hippocampus is prone to epileptic seizures and is a key brain region and experimental platform for investigating mechanisms associated with the abnormal neuronal excitability that characterizes a seizure. Accordingly, the hippocampal slice is a common in vitro model to study treatments that may prevent or reduce seizure activity. The ketogenic diet is a metabolic therapy used to treat epilepsy in adults and children for nearly 100 years; it can reduce or eliminate even severe or refractory seizures. New insights into its underlying mechanisms have been revealed by diverse types of electrophysiological recordings in hippocampal slices...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27846468/therapeutic-effects-of-the-ketogenic-diet-in-children-with-lennox-gastaut-syndrome
#13
Yunjian Zhang, Yi Wang, Yuanfeng Zhou, Linmei Zhang, Lifei Yu, Shuizhen Zhou
OBJECTIVE: The aim of this study was to evaluate the efficacy of the ketogenic diet (KD) on the clinical and electroencephalographic (EEG) features of Lennox-Gastaut syndrome (LGS) and explore the relationships between EEG changes and clinical efficacy. METHODS: We retrospectively studied 47 patients with LGS who accepted KD therapy between May 2011 and May 2015. Clinical efficacy and EEG features such as background activity, abnormal interictal epileptic discharges (IEDs) and the discharge location were evaluated prior to and at 3 and 6 months after therapy...
December 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27836684/ketogenic-diets-improve-behaviors-associated-with-autism-spectrum-disorder-in-a-sex-specific-manner-in-the-el-mouse
#14
David N Ruskin, Jessica A Fortin, Subrina N Bisnauth, Susan A Masino
The core symptoms of autism spectrum disorder are poorly treated with current medications. Symptoms of autism spectrum disorder are frequently comorbid with a diagnosis of epilepsy and vice versa. Medically-supervised ketogenic diets are remarkably effective nonpharmacological treatments for epilepsy, even in drug-refractory cases. There is accumulating evidence that supports the efficacy of ketogenic diets in treating the core symptoms of autism spectrum disorders in animal models as well as limited reports of benefits in patients...
January 1, 2017: Physiology & Behavior
https://www.readbyqxmd.com/read/27826689/%C3%AE-hydroxybutyrate-in-the-brain-one-molecule-multiple-mechanisms
#15
Lavanya B Achanta, Caroline D Rae
β-Hydroxybutyrate (βOHB), a ketone body, is oxidised as a brain fuel. Although its contribution to energy metabolism in the healthy brain is minimal, it is an interesting metabolite which is not only oxidised but also has other direct and collateral effects which make it a molecule of interest for therapeutic purposes. In brain βOHB can be produced in astrocytes from oxidation of fatty acids or catabolism of amino acids and is metabolised in the mitochondria of all brain cell types although uptake across the blood brain barrier is a metabolic control point...
November 8, 2016: Neurochemical Research
https://www.readbyqxmd.com/read/27822291/monitoring-for-compliance-with-a-ketogenic-diet-what-is-the-best-time-of-day-to-test-for-urinary-ketosis
#16
Paul Urbain, Hartmut Bertz
BACKGROUND: The ketogenic diet (KD) is a very low-carbohydrate, high-fat and adequate-protein diet with no calorie limit that induces a metabolic condition called "physiological ketosis". It was first introduced to treat epilepsy in the 1920s and has become quite popular recently as weight-loss and performance-enhancing diet. Its therapeutic use in a range of diseases is under investigation. During KD interventions people are supposed to monitor compliance with the dietary regimen by daily urine testing for ketosis...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/27817982/from-genotype-to-phenotype-in-dravet-disease
#17
Svetlana Gataullina, Olivier Dulac
Dravet syndrome combines clonic generalized, focal or unilateral seizures, beginning within the first year of life, often triggered by hyperthermia whatever its cause, including pertussis vaccination. Long-lasting febrile seizures are frequent in infancy and repeat status epilepticus (SE) has negative prognostic value. Massive myoclonus, rare absences, complex partial seizures and generalized spikes may appear several years later. Myoclonic status may occur in childhood, but acute encephalopathy with febrile SE followed by ischemic lesions and psychomotor impairment, the most severe condition, occurs mainly within the first five years of life...
October 21, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27816501/gapdh-mediated-posttranscriptional-regulations-of-sodium-channel-scn1a-and-scn3a-genes-under-seizure-and-ketogenic-diet-conditions
#18
Guo-Wang Lin, Ping Lu, Tao Zeng, Hui-Ling Tang, Yong-Hong Chen, Shu-Jing Liu, Mei-Mei Gao, Qi-Hua Zhao, Yong-Hong Yi, Yue-Sheng Long
Abnormal expressions of sodium channel SCN1A and SCN3A genes alter neural excitability that are believed to contribute to the pathogenesis of epilepsy, a long-term risk of recurrent seizures. Ketogenic diet (KD), a high-fat and low-carbohydrate treatment for difficult-to-control (refractory) epilepsy in children, has been suggested to reverse gene expression patterns. Here, we reveal a novel role of GAPDH on the posttranscriptional regulation of mouse Scn1a and Scn3a expressions under seizure and KD conditions...
November 2, 2016: Neuropharmacology
https://www.readbyqxmd.com/read/27815040/the-addition-of-ketone-bodies-alleviates-mitochondrial-dysfunction-by-restoring-complex-i-assembly-in-a-melas-cellular-model
#19
Samuel Frey, Guillaume Geffroy, Valerie Desquiret-Dumas, Naig Gueguen, Celine Bris, Sophie Belal, Patrizia Amati-Bonneau, Arnaud Chevrollier, Magalie Barth, Daniel Henrion, Guy Lenaers, Dominique Bonneau, Pascal Reynier, Vincent Procaccio
Ketogenic Diet used to treat refractory epilepsy for almost a century may represent a treatment option for mitochondrial disorders for which effective treatments are still lacking. Mitochondrial complex I deficiencies are involved in a broad spectrum of inherited diseases including Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes syndrome leading to recurrent cerebral insults resembling strokes and associated with a severe complex I deficiency caused by mitochondrial DNA (mtDNA) mutations...
November 1, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27803969/erratum-to-effects-of-twenty-days-of-the-ketogenic-diet-on-metabolic-and-respiratory-parameters-in-healthy-subjects
#20
Alessandro Rubini, Gerardo Bosco, Alessandra Lodi, Lorenzo Cenci, Andrea Parmagnani, Keith Grimaldi, Yang Zhongjin, Antonio Paoli
No abstract text is available yet for this article.
November 1, 2016: Lung
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