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https://www.readbyqxmd.com/read/28079250/use-of-the-ketogenic-diet-in-the-neonatal-intensive-care-unit-safety-and-tolerability
#1
Lindsey Thompson, Erin Fecske, Mohammad Salim, Ara Hall
Drug-resistant epilepsy poses a challenge in neonatal patients, especially those in the neonatal intensive care unit (NICU), who have various secondary comorbidities. We present results of four children with a history of drug-resistant epilepsy for whom a ketogenic diet was initiated and used in the NICU. A nonfasting induction into ketosis over 1-2 weeks was utilized, with gradual increases in the ketogenic ratio every 2-3 days. Data were collected retrospectively from a database, which included medical history, daily progress notes, relevant laboratory data, and imaging and diagnostic information...
January 12, 2017: Epilepsia
https://www.readbyqxmd.com/read/28076316/changes-of-thyroid-hormonal-status-in-patients-receiving-ketogenic-diet-due-to-intractable-epilepsy
#2
Engin Kose, Orkide Guzel, Korcan Demir, Nur Arslan
BACKGROUND: Ketogenic diet (KD), which is high in fat and low in carbohydrates, mimics the metabolic state of starvation and is used therapeutically for pharmacoresistant epilepsy. It is known that generation of triiodothyronine (T3) from thyroxine (T4) decreases during fasting periods. The aim of this study was to evaluate the thyroid function of children receiving KD for at least 1 year due to drug-resistant epilepsy. METHODS: A total of 120 patients [63 males, 52...
January 11, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28074323/changes-in-cerebral-metabolism-during-ketogenic-diet-in-patients-with-primary-brain-tumors-1-h-mrs-study
#3
Moran Artzi, Gilad Liberman, Nachum Vaisman, Felix Bokstein, Faina Vitinshtein, Orna Aizenstein, Dafna Ben Bashat
Normal brain cells depend on glucose metabolism, yet they have the flexibility to switch to the usage of ketone bodies during caloric restriction. In contrast, tumor cells lack genomic and metabolic flexibility and are largely dependent on glucose. Ketogenic-diet (KD) was suggested as a therapeutic option for malignant brain cancer. This study aimed to detect metabolic brain changes in patients with malignant brain gliomas on KD using proton magnetic-resonance-spectroscopy ((1)H-MRS). Fifty MR scans were performed longitudinally in nine patients: four patients with recurrent glioblastoma (GB) treated with KD in addition to bevacizumab; one patient with gliomatosis-cerebri treated with KD only; and four patients with recurrent GB who did not receive KD...
January 10, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28065757/fats-for-thoughts-an-update-on-brain-fatty-acid-metabolism
#4
Adele Romano, Justyna Barbara Koczwara, Cristina Anna Gallelli, Daniele Vergara, Maria Vittoria Micioni Di Bonaventura, Silvana Gaetani, Anna Maria Giudetti
Brain fatty acid (FA) metabolism deserves a close attention not only for its energetic aspects but also because FAs and their metabolites/derivatives are able to influence many neural functions, contributing to brain pathologies or representing potential targets for pharmacological and/or nutritional interventions. Glucose is the preferred energy substrate for the brain, whereas the role of FAs is more marginal. In conditions of decreased glucose supply ketone bodies, mainly formed by FA oxidation, are the alternative main energy source...
January 5, 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/28057897/review-and-update-of-the-hong-kong-epilepsy-guideline-on-status-epilepticus
#5
REVIEW
E Lw Fung, B Bh Fung
Convulsive status epilepticus is the most extreme form of seizure. It is a medical and neurological emergency that requires prompt and appropriate treatment. Treatment of convulsive status epilepticus is usually divided into stages/steps. The International League Against Epilepsy has released a new definition of status epilepticus that may help to unify the definition in future studies. Over the last few years new information has become available regarding its management. The Rapid Anticonvulsant Medication Prior to Arrival Trial demonstrated non-inferiority of intramuscular midazolam in early status epilepticus compared with intravenous lorazepam...
January 6, 2017: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
https://www.readbyqxmd.com/read/28053859/is-there-a-role-for-diet-monotherapy-in-adult-epilepsy
#6
Mackenzie C Cervenka, Bobbie J Henry-Barron, Eric H Kossoff
Ten adults were treated with ketogenic diet monotherapy for epilepsy. Four patients were naïve to antiseizure drugs (ASDs), and six previously tried and stopped ASDs. Of four treatment-naïve participants, two (50%) were free from disabling seizures on Modified Atkins Diet (MAD) monotherapy for > 1 year. Two (50%) stopped. Four of six patients (67%) who had previously tried ASDs became seizure-free on diet monotherapy, and two experienced > 50% seizure reduction. Side effects included amenorrhea, weight loss, osteoporosis, and hyperlipidemia...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28043175/the-promising-potential-role-of-ketones-in-inflammatory-dermatologic-disease-a-new-frontier-in-treatment-research
#7
Daren A Fomin, Brianna McDaniel, Jonathan Crane
The ketogenic diet has been shown to be beneficial for numerous diseases across different organ systems, but a dearth of information exists regarding these benefits for skin disease. Here, we searched the literature for known mechanisms behind inflammation in dermatologic disease and correlated that with suggested mechanisms of anti-inflammatory activity of ketones and a ketogenic state in the human body to observe how ketones and ketosis might aid in the treatment of inflammatory skin diseases based on these mechanisms...
January 3, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/28042592/paroxysmal-nonepileptic-events-in-glut1-deficiency
#8
Joerg Klepper, Baerbel Leiendecker, Christin Eltze, Nicole Heussinger
View Supplementary Video Movement disorders are a major feature of Glut1 deficiency. As recently identified in adults with paroxysmal exercise-induced dystonia, similar events were reported in pediatric Glut1 deficiency. In a case series, parent videos of regular motor state and paroxysmal events were requested from children with Glut1 deficiency on clinical follow-up. A questionnaire was sent out to 60 families. Videos of nonparoxysmal/paroxysmal states in 3 children illustrated the ataxic-dystonic, choreatiform, and dyskinetic-dystonic nature of paroxysmal events...
November 2016: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28038385/changes-in-quality-of-life-as-a-result-of-ketogenic-diet-therapy-a-new-approach-to-assessment-with-the-potential-for-positive-therapeutic-effects
#9
Susan Bruce, Anita Devlin, Linda Air, Lucy Cook
There are difficulties inherent in measuring Quality of life (QoL) in patients with chronic illness, including agreement on definitions of quality of life and the type of measure used, disease specific or generic. Well validated QoL instruments for epilepsy exist but focus on capturing common themes pertinent to children and families as a group instead of focusing on themes important to individual patients and their families/carers. In addition, it is common for numerous items on these inventories to be left incomplete or responded to with "not applicable" since many of the items are not suitable for children with disabilities and their families...
December 27, 2016: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28031249/transcription-factor-assisted-loading-and-enhancer-dynamics-dictate-the-hepatic-fasting-response
#10
Ido Goldstein, Songjoon Baek, Diego M Presman, Ville Pakkinaho, Erin E Swinstead, Gordon L Hager
Fasting elicits transcriptional programs in hepatocytes leading to glucose and ketone production. This transcriptional program is regulated by many transcription factors (TFs). To understands how this complex network regulates the metabolic response to fasting we aimed at isolating the enhancers and TFs dictating it. Measuring chromatin accessibility revealed that fasting massively reorganizes liver chromatin, exposing numerous fasting-induced enhancers. By utilizing computational methods in combination with dissecting enhancers features and TF cistromes, we implicated four key TFs regulating the fasting response: glucocorticoid receptor (GR), cAMP responsive element binding protein 1 (CREB1), peroxisome proliferator activated receptor alpha (PPARA) and CCAAT/enhancer binding protein beta (CEBPB)...
December 28, 2016: Genome Research
https://www.readbyqxmd.com/read/28030918/to-treat-or-not-to-treat-drug-refractory-epilepsy-by-the-ketogenic-diet-that-is-the-question
#11
REVIEW
Marzena Ułamek-Kozioł, Ryszard Pluta, Anna Bogucka-Kocka, Stanisław J Czuczwar
Epilepsy is a serious neurologic disorder worldwide which affects about 1% of the population (ca. 50 million people), the highest prevalence occurring in both children and elderly. Apart from idiopathic forms, etiology of the disease involves multiple brain risk factors - the most frequent being cerebrovascular diseases, tumours and traumatic injuries. Several treatment options exist, including, for instance, pharmacotherapy, vagal nerve stimulation or epilepsy surgery. In spite of treatment, about 30% of patients with epilepsy still have seizures and become drug-refractory...
23, 2016: Annals of Agricultural and Environmental Medicine: AAEM
https://www.readbyqxmd.com/read/28012184/low-carbohydrate-high-fat-diet-impairs-exercise-economy-and-negates-the-performance-benefit-from-intensified-training-in-elite-race-walkers
#12
Louise M Burke, Megan L Ross, Laura A Garvican-Lewis, Marijke Welvaert, Ida A Heikura, Sara G Forbes, Joanne G Mirtschin, Louise E Cato, Nicki Strobel, Avish P Sharma, John A Hawley
We investigated the effects of adaptation to a ketogenic low-carbohydrate (CHO), high-fat diet (LCHF) during 3 wk of intensified training on metabolism and performance of world-class endurance athletes. We controlled three isoenergetic diets in elite race walkers: High CHO availability (8.6 g.kg(-) 1.d(-1) CHO, 2.1 g.kg(-) 1.d(-1) protein; 1.2 g.kg(-) 1.d(-1) fat) consumed before/during/after training (HCHO, n = 9): identical macronutrient intake, periodised within/between days to alternate between low and high CHO availability (PCHO, n = 10); LCHF (<50 g...
December 23, 2016: Journal of Physiology
https://www.readbyqxmd.com/read/28008324/vitamin-d3-for-the-treatment-of-epilepsy-basic-mechanisms-animal-models-and-clinical-trials
#13
REVIEW
Kevin Pendo, Christopher M DeGiorgio
There is increasing evidence supporting dietary and alternative therapies for epilepsy, including the ketogenic diet, modified Atkins diet, and omega-3 fatty acids. Vitamin D3 is actively under investigation as a potential intervention for epilepsy. Vitamin D3 is fat-soluble steroid, which shows promise in animal models of epilepsy. Basic research has shed light on the possible mechanisms by which Vitamin D3 may reduce seizures, and animal data support the efficacy of Vitamin D3 in rat and mouse models of epilepsy...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/28003429/molecular-cloning-of-rat-acss3-and-characterization-of-mammalian-propionyl-coa-synthetase-in-the-liver-mitochondrial-matrix
#14
Yukihiro Yoshimura, Aya Araki, Hitomi Maruta, Yoshitaka Takahashi, Hiromi Yamashita
Among the three acyl-CoA synthetase short-chain family members (ACSS), ACSS3 is poorly characterized. To characterize ACSS3, we performed molecular cloning and protein expression of rat acss3 and determined its intracellular localization, tissue distribution, and substrate specificity. Transient expression of rat ACSS3 in HeLa cells resulted in a 10-fold increase of acetyl-CoA synthetase activity compared with that in control cells. The acss3 transcripts are expressed in a wide range of tissues, with the highest levels observed in liver tissue followed by kidney tissue...
December 21, 2016: Journal of Biochemistry
https://www.readbyqxmd.com/read/27999180/a-ketogenic-diet-rescues-hippocampal-memory-defects-in-a-mouse-model-of-kabuki-syndrome
#15
Joel S Benjamin, Genay O Pilarowski, Giovanni A Carosso, Li Zhang, David L Huso, Loyal A Goff, Hilary J Vernon, Kasper D Hansen, Hans T Bjornsson
Kabuki syndrome is a Mendelian intellectual disability syndrome caused by mutations in either of two genes (KMT2D and KDM6A) involved in chromatin accessibility. We previously showed that an agent that promotes chromatin opening, the histone deacetylase inhibitor (HDACi) AR-42, ameliorates the deficiency of adult neurogenesis in the granule cell layer of the dentate gyrus and rescues hippocampal memory defects in a mouse model of Kabuki syndrome (Kmt2d(+/βGeo)). Unlike a drug, a dietary intervention could be quickly transitioned to the clinic...
January 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27982131/creb3l3-controls-fatty-acid-oxidation-and-ketogenesis-in-synergy-with-ppar%C3%AE
#16
Yoshimi Nakagawa, Aoi Satoh, Hitomi Tezuka, Song-Iee Han, Kenta Takei, Hitoshi Iwasaki, Shigeru Yatoh, Naoya Yahagi, Hiroaki Suzuki, Yasumasa Iwasaki, Hirohito Sone, Takashi Matsuzaka, Nobuhiro Yamada, Hitoshi Shimano
CREB3L3 is involved in fatty acid oxidation and ketogenesis in a mutual manner with PPARα. To evaluate relative contribution, a combination of knockout and transgenic mice was investigated. On a ketogenic-diet (KD) that highlights capability of hepatic ketogenesis, Creb3l3(-/-) mice exhibited reduction of expression of genes for fatty oxidation and ketogenesis comparable to Ppara(-/-) mice. Most of the genes were further suppressed in double knockout mice indicating independent contribution of hepatic CREB3L3...
December 16, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27975114/the-ketogenic-diet-a-practical-guide-for-pediatricians
#17
Aimee F Luat, Leigh Coyle, Deepak Kamat
The ketogenic diet is an effective treatment for drug-resistant epilepsies in children. In addition, it is the first-line treatment for some metabolic disorders, such as glucose transporter 1 deficiency syndrome. This article discusses the proposed mechanisms of a ketogenic diet's antiseizure action, its clinical indications, and its contraindications. The steps involved in ketogenic diet initiation, monitoring, and management of its side effects are also discussed. This review provides general pediatricians with the necessary skills to provide comprehensive care of children using the ketogenic diet and counsel their families and caregivers...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27935584/ketogenesis-contributes-to-intestinal-cell-differentiation
#18
Qingding Wang, Yuning Zhou, Piotr Rychahou, Teresa W-M Fan, Andrew N Lane, Heidi L Weiss, B Mark Evers
The intestinal epithelium undergoes a continual process of proliferation, differentiation and apoptosis. Previously, we have shown that the PI3K/Akt/mTOR pathway has a critical role in intestinal homeostasis. However, the downstream targets mediating the effects of mTOR in intestinal cells are not known. Here, we show that the ketone body β-hydroxybutyrate (βHB), an endogenous inhibitor of histone deacetylases (HDACs) induces intestinal cell differentiation as noted by the increased expression of differentiation markers (Mucin2 (MUC2), lysozyme, IAP, sucrase-isomaltase, KRT20, villin, Caudal-related homeobox transcription factor 2 (CDX2) and p21(Waf1))...
December 9, 2016: Cell Death and Differentiation
https://www.readbyqxmd.com/read/27928725/the-efficacy-of-the-ketogenic-diet-in-infants-and-young-children-with-refractory-epilepsies-using-a-formula-based-powder
#19
Mahmoud Reza Ashrafi, Seyed Ahmad Hosseini, Gholam Reza Zamani, Mahmoud Mohammadi, Alireza Tavassoli, Reza Shervin Badv, Morteza Heidari, Parviz Karimi, Reza Azizi Malamiri
To evaluate the efficacy, safety, and tolerability of a classic 4:1 ketogenic diet using a formula-based powder in infants and children with refractory seizures who are reluctant to eat homemade foods. We conducted an open label trial and administered a ketogenic diet using formula-based power (Ketocal(®)). Twenty-seven infants and children aged between 12 months and 5 years were enrolled who had refractory seizures and were reluctant to eat homemade foods. Of 27 children, 5 were lost to follow-up and 22 were remained at the end of the study...
December 7, 2016: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/27927575/novel-mutation-in-a-patient-with-late-onset-glut1-deficiency-syndrome
#20
Sandra Juozapaite, Ruta Praninskiene, Birute Burnyte, Laima Ambrozaityte, Birute Skerliene
Glucose transporter 1 deficiency syndrome (GLUT1-DS) is an inborn error of metabolism caused by impaired glucose transport through blood brain barrier due to mutation in SLC2A1 gene, encoding transporter protein. Clinical spectrum includes various signs and symptoms, ranging from severe epileptic encephalopathy to movement disorders. The diagnosis of GLUT1-DS requires hypoglycorrhachia in the presence of normoglycaemia with a reduced cerebrospinal fluid (CSF):plasma glucose ratio. The absence of pathogenic mutation in SLC2A1 gene does not exclude the diagnosis...
December 5, 2016: Brain & Development
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