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https://www.readbyqxmd.com/read/28003588/effectiveness-of-medium-chain-triglyceride-oil-therapy-in-two-japanese-citrin-deficient-siblings-evaluation-using-oral-glucose-tolerance-tests
#1
Hiroki Otsuka, Hideo Sasai, Elsayed Abdelkreem, Norio Kawamoto, Minako Kawamoto, Toshiya Kamiya, Yasuo Tanimoto, Atsuo Kikuchi, Shigeo Kure, Chikahiko Numakura, Kiyoshi Hayasaka, Toshiyuki Fukao
Citrin deficiency, an inherited defect of the liver-type mitochondrial aspartate/glutamate carrier isoform (citrin), may cause impairment of glycolysis because of an increase in the cytosolic NADH/NAD(+) ratio. We report a Japanese boy whose main complaint was recurrent hypoglycemic episodes. He was suspected as having citrin deficiency because of his peculiar preference for protein- and fat-rich food. His young sister also had a similar food preference. Both siblings were diagnosed with citrin deficiency by genetic analysis...
2016: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27999529/exogenous-ketone-supplements-reduce-anxiety-related-behavior-in-sprague-dawley-and-wistar-albino-glaxo-rijswijk-rats
#2
Csilla Ari, Zsolt Kovács, Gabor Juhasz, Cem Murdun, Craig R Goldhagen, Andrew M Koutnik, Angela M Poff, Shannon L Kesl, Dominic P D'Agostino
Nutritional ketosis has been proven effective for seizure disorders and other neurological disorders. The focus of this study was to determine the effects of ketone supplementation on anxiety-related behavior in Sprague-Dawley (SPD) and Wistar Albino Glaxo/Rijswijk (WAG/Rij) rats. We tested exogenous ketone supplements added to food and fed chronically for 83 days in SPD rats and administered sub-chronically for 7 days in both rat models by daily intragastric gavage bolus followed by assessment of anxiety measures on elevated plus maze (EPM)...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27935709/physical-and-chemical-stability-of-curcumin-in-aqueous-solutions-and-emulsions-impact-of-ph-temperature-and-molecular-environment
#3
Mahesh Kharat, Zheyuan Du, Guodong Zhang, David Julian McClements
The utilization of curcumin as a nutraceutical in food and supplement products is often limited because of its low water solubility, poor chemical stability, and low oral bioavailability. This study examined the impact of pH, storage temperature, and molecular environment on the physical and chemical stability of pure curcumin in aqueous solutions and in oil-in-water emulsions. Unlike naturally occurring curcuminoid mixtures (that contain curcumin, demethoxy-curcumin, and bisdemethoxy-curcumin), pure curcumin was highly unstable to chemical degradation in alkaline aqueous solutions (pH ≥7...
February 16, 2017: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/27777763/dietary-stimulation-of-the-endogenous-somatotropic-axis-in-weaner-and-grower-finisher-pigs-using-medium-chain-triglycerides-and-cysteamine-hydrochloride
#4
David W Miller, Zoe Prosser, Edward Y W Chee, Christian F Hansen, Frank R Dunshea, Bruce P Mullan, John R Pluske
BACKGROUND: Three experiments were conducted to examine the overall hypothesis that addition of medium chain triglycerides (MCT) and cysteamine hydrochloride (CSH) into the diets of young and growing pigs would stimulate the endogenous somatotropic axis to improve growth and performance. RESULTS: In Experiment 1, weaner pigs were given either a 5 d dietary supplement of 5 % MCT (n = 8) or a control diet (n = 8). MCT increased the plasma concentration of growth hormone (GH; P < 0...
2016: Journal of Animal Science and Biotechnology
https://www.readbyqxmd.com/read/27530817/metabolic-modulation-by-medium-chain-triglycerides-reduces-oxidative-stress-and-ameliorates-cd36-mediated-cardiac-remodeling-in-spontaneously-hypertensive-rat-in-initial-and-established-stages-of-hypertrophy
#5
Saifudeen Ismael, Lakshmi S, Remani K, R Renuka Nair
BACKGROUND: Left ventricular hypertrophy is characterized by decrease in oxidation of long-chain fatty acids, possibly mediated by reduced expression of the cell-surface protein Cluster of differentiation-36 (CD36). Spontaneously hypertensive rat (SHR) was therefore supplemented with Medium-chain triglycerides (MCT), a substrate that bypasses CD36; based on the assumption that the metabolic modulation will ameliorate ventricular remodeling. METHODS: The diet of 2-month-old and 6-month-old SHR was supplemented with 5% MCT (Tricaprylin), for 4 months...
August 12, 2016: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/27517611/cognition-and-synaptic-plasticity-related-changes-in-aged-rats-supplemented-with-8-and-10-carbon-medium-chain-triglycerides
#6
Dongmei Wang, Ellen S Mitchell
Brain glucose hypometabolism is a common feature of Alzheimer's disease (AD). Previous studies have shown that cognition is improved by providing AD patients with an alternate energy source: ketones derived from either ketogenic diet or supplementation with medium chain triglycerides (MCT). Recently, data on the neuroprotective capacity of MCT-derived medium chain fatty acids (MCFA) suggest 8-carbon and 10-carbon MCFA may have cognition-enhancing properties which are not related to ketone production. We investigated the effect of 8 week treatment with MCT8, MCT10 or sunflower oil supplementation (5% by weight of chow diet) in 21 month old Wistar rats...
2016: PloS One
https://www.readbyqxmd.com/read/27334895/management-of-an-lchadd-patient-during-pregnancy-and-high-intensity-exercise
#7
D C D van Eerd, I A Brussé, V F R Adriaens, R T Mankowski, S F E Praet, M Michels, M Langeveld
In this report we describe a female Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHADD) patient who suffered from severe exercise intolerance. At age 34, the patient became pregnant for the first time. After an uneventful first 32 weeks of pregnancy she developed sinus tachycardia (resting heart rate 120-134 bpm) and lactate and creatinine kinase levels increased (3.3 mmol/L and 264 U/L, respectively). Increasing MCT supplementation (dose and frequency of administration) lowered heart rate and improved biochemical parameters...
June 23, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27198626/improvement-of-%C3%AE-carotene-bioaccessibility-from-dietary-supplements-using-excipient-nanoemulsions
#8
Laura Salvia-Trujillo, David Julian McClements
The influence of excipient nanoemulsions on β-carotene bioaccessibility from commercial dietary supplements (tablets or soft gels) was studied employing an in vitro gastrointestinal tract (GIT) model. Excipient nanoemulsions were formulated from long or medium chain triglycerides (LCT or MCT) to determine the impact of lipid type on carotenoid bioaccessibility. Dietary supplements were tested using the GIT model in the absence or presence of excipient nanoemulsions. β-carotene bioaccessibility from tablets (0...
June 8, 2016: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/27153855/dicarboxylic-acid-excretion-in-normal-formula-fed-and-breastfed-infants
#9
Maria Anderson, Kathrin Eliot, Patrick Kelly, James Shoemaker
PURPOSE: Infant formulas are often supplemented with medium-chain triglycerides (MCTs) to optimize calories for small for gestational age or preterm infants. High amounts of MCTs have been associated with an increase in dicarboxylic acid (DCA) in the urine. Elevated DCA in the urine is also a clinical indicator for fatty acid metabolism disorders. The purpose of this study was to identify if there is an amount of MCTs that can be provided without elevating urinary DCA excretion. METHODS: A metabolic screening laboratory provided urinary DCA excretion data for 175 infants...
May 6, 2016: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/27143902/the-application-of-nutrition-support-in-conservative-treatment-of-chylous-ascites-after-abdominal-surgery
#10
Wu Pan, Shen-Yang Cai, Hai-Long Luo, Shu-Rui Ouyang, Wen-Duo Zhang, Zai-Rong Wei, Da-Li Wang
BACKGROUND: Chylous ascites is the pathologic leakage of triglycerides-rich lymphatic fluid into the peritoneal cavity. Chylous ascites is a rare complication in abdominal surgery. This study aimed to find a relatively better method for nutrition support in the treatment of chylous ascites after abdominal surgery. METHODS: This study was a retrospective study. This study retrospectively reviewed patients who underwent abdominal surgery and developed chylous ascites, from the year 2010 to 2014, at the West China Hospital of Sichuan University and the Affiliated Hospital of Zunyi Medical College...
2016: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/27117294/diagnosis-treatment-and-clinical-outcome-of-patients-with-mitochondrial-trifunctional-protein-long-chain-3-hydroxy-acyl-coa-dehydrogenase-deficiency
#11
Irene De Biase, Krista S Viau, Aiping Liu, Tatiana Yuzyuk, Lorenzo D Botto, Marzia Pasquali, Nicola Longo
Deficiency of the mitochondrial trifunctional protein (TFP) and long-chain 3-Hydroxy Acyl-CoA dehydrogenase (LCHAD) impairs long-chain fatty acid oxidation and presents with hypoglycemia, cardiac, liver, eye, and muscle involvement. Without treatment, both conditions can be life-threatening. These diseases are identified by newborn screening (NBS), but the impact of early treatment on long-term clinical outcome is unknown. Moreover, there is lack of consensus on treatment, particularly on the use of carnitine supplementation...
April 28, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27075909/medium-chain-triglycerides-in-combination-with-leucine-and-vitamin-d-increase-muscle-strength-and-function-in-frail-elderly-adults-in-a-randomized-controlled-trial
#12
Sakiko Abe, Osamu Ezaki, Motohisa Suzuki
BACKGROUND: Sarcopenia, the loss of skeletal muscle mass, strength, and function, is common in elderly individuals but difficult to treat. OBJECTIVE: A combination of nutrients was investigated to treat sarcopenia in very frail elderly adults. METHODS: We enrolled 38 elderly nursing home residents (11 men and 27 women with a mean ± SD age of 86.6 ± 4.8 y) in a 3-mo randomized, controlled, single-blind, parallel group trial. The participants were randomly allocated to 3 groups...
May 2016: Journal of Nutrition
https://www.readbyqxmd.com/read/27014572/successful-therapy-for-protein-losing-enteropathy-caused-by-chronic-neuronopathic-gaucher-disease
#13
A A Mhanni, M Kozenko, J N Hartley, M Deneau, W El-Matary, C Rockman-Greenberg
Gaucher disease (OMIM #230800) is caused by β-glucosidase deficiency and primarily involves the mononuclear phagocyte system (also called Reticuloendothelial System or Macrophage System). The disease is classified into three main phenotypes based on the presence or absence of neurological manifestations: non-neuronopathic (type 1), acute neuronopathic (type 2) and chronic neuronopathic (type 3). Typical manifestations include hepatosplenomegaly, skeletal deformities, hematological abnormalities, interstitial lung fibrosis and neurodegeneration in neuronopathic cases...
March 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/26962779/primary-intestinal-lymphangiectasia-manifested-as-unusual-edemas-and-effusions-a-case-report
#14
Xuefeng Wang, Hong Jin, Weilu Wu
Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The main symptom is variable degrees of pitting edemas of bilateral lower limbs. But edemas of any other parts of body, and mild serous effusions may also occur sometimes...
March 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26911722/quantitative-comparison-of-pet-performance-siemens-biograph-mct-and-mmr
#15
Anna M Karlberg, Oddbjørn Sæther, Live Eikenes, Pål Erik Goa
BACKGROUND: Integrated clinical whole-body PET/MR systems were introduced in 2010. In order to bring this technology into clinical usage, it is of great importance to compare the performance with the well-established PET/CT. The aim of this study was to evaluate PET performance, with focus on image quality, on Siemens Biograph mMR (PET/MR) and Siemens Biograph mCT (PET/CT). METHODS: A direct quantitative comparison of the performance characteristics between the mMR and mCT system was performed according to National Electrical Manufacturers Association (NEMA) NU 2-2007 protocol...
December 2016: EJNMMI Physics
https://www.readbyqxmd.com/read/26907176/unique-plasma-metabolomic-signatures-of-individuals-with-inherited-disorders-of-long-chain-fatty-acid-oxidation
#16
Colin S McCoin, Brian D Piccolo, Trina A Knotts, Dietrich Matern, Jerry Vockley, Melanie B Gillingham, Sean H Adams
Blood and urine acylcarnitine profiles are commonly used to diagnose long-chain fatty acid oxidation disorders (FAOD: i.e., long-chain hydroxy-acyl-CoA dehydrogenase [LCHAD] and carnitine palmitoyltransferase 2 [CPT2] deficiency), but the global metabolic impact of long-chain FAOD has not been reported. We utilized untargeted metabolomics to characterize plasma metabolites in 12 overnight-fasted individuals with FAOD (10 LCHAD, two CPT2) and 11 healthy age-, sex-, and body mass index (BMI)-matched controls, with the caveat that individuals with FAOD consume a low-fat diet supplemented with medium-chain triglycerides (MCT) while matched controls consume a typical American diet...
May 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/26881704/thermal-degradation-and-isomerization-of-%C3%AE-carotene-in-oil-in-water-nanoemulsions-supplemented-with-natural-antioxidants
#17
Jiang Yi, Yuting Fan, Wallace Yokoyama, Yuzhu Zhang, Liqing Zhao
The goal of this study was to see the impact on the retention and isomerization of encapsulated β-carotene (BC) in nanoemulsions fortified with natural antioxidants (α-tocopherol (AT) and l-ascorbic acid (AA)). The physical stability of nanoemulsion, oxidative stability, and isomerization of all-trans-β-carotene (BC) in oil-in-water (O/W) nanoemulsions were determined in the presence or absence of natural antioxidants at 25 and 50 °C at certain intervals of time by high-performance liquid chromatography (HPLC)...
March 9, 2016: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/26878786/medium-chain-triglycerides-and-monounsaturated-fatty-acids-potentiate-the-beneficial-effects-of-fish-oil-on-selected-cardiovascular-risk-factors-in-rats
#18
Vijay Kumar Reddy Kondreddy, Maheswaraiah Anikisetty, Kamatham Akhilender Naidu
Fish oil (FO) rich in eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) is known to reduce the risk of cardiovascular diseases (CVDs). Little information is known regarding the influence of lipid composition in the background diet on the modulatory effect of FO supplementation on CVDs. The present study was designed to investigate the influence of various background dietary lipids and FO on selected cardiovascular risk factors in rats. Adult Wistar rats were fed semisynthetic diet with FO at 1.0% or 2...
February 2016: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/26855664/effects-of-exogenous-ketone-supplementation-on-blood-ketone-glucose-triglyceride-and-lipoprotein-levels-in-sprague-dawley-rats
#19
Shannon L Kesl, Angela M Poff, Nathan P Ward, Tina N Fiorelli, Csilla Ari, Ashley J Van Putten, Jacob W Sherwood, Patrick Arnold, Dominic P D'Agostino
BACKGROUND: Nutritional ketosis induced by the ketogenic diet (KD) has therapeutic applications for many disease states. We hypothesized that oral administration of exogenous ketone supplements could produce sustained nutritional ketosis (>0.5 mM) without carbohydrate restriction. METHODS: We tested the effects of 28-day administration of five ketone supplements on blood glucose, ketones, and lipids in male Sprague-Dawley rats. The supplements included: 1,3-butanediol (BD), a sodium/potassium β-hydroxybutyrate (βHB) mineral salt (BMS), medium chain triglyceride oil (MCT), BMS + MCT 1:1 mixture, and 1,3 butanediol acetoacetate diester (KE)...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/26547562/anaplerotic-treatment-of-long-chain-fat-oxidation-disorders-with-triheptanoin-review-of-15-years-experience
#20
Charles R Roe, Henri Brunengraber
BACKGROUND: The treatment of long-chain mitochondrial β-oxidation disorders (LC-FOD) with a low fat-high carbohydrate diet, a diet rich in medium-even-chain triglycerides (MCT), or a combination of both has been associated with high morbidity and mortality for decades. The pathological tableau appears to be caused by energy deficiency resulting from reduced availability of citric acid cycle (CAC) intermediates required for optimal oxidation of acetyl-CoA. This hypothesis was investigated by diet therapy with carnitine and anaplerotic triheptanoin (TH)...
December 2015: Molecular Genetics and Metabolism
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