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https://www.readbyqxmd.com/read/29768383/treatable-massive-pericardial-effusion-and-hypertrophic-cardiomyopathy-in-an-infant-with-a-novel-homozygous-acadvl-mutation-a-case-report
#1
Yoo-Mi Kim, Geena Kim, Hoon Ko, Han-Wook Yoo, Hyoung Doo Lee
RATIONALE: Infantile-onset hypertrophic cardiomyopathy (HCMP) should be considered a largely genetic condition, although its onset is most often triggered by infection. Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a rare autosomal recessive inborn error of mitochondrial fatty acid β-oxidation that often causes severe cardiomyopathy and/or sudden death during the neonatal period. PATIENT CONCERNS: Herein, we report an infant with VLCAD deficiency who presented with severe cardiac manifestations, including massive pericardial effusion and HCMP...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29740314/effect-of-toxicants-on-fatty-acid-metabolism-in-hepg2-cells
#2
David Grünig, Urs Duthaler, Stephan Krähenbühl
Impairment of hepatic fatty acid metabolism can lead to liver steatosis and injury. Testing drugs for interference with hepatic fatty acid metabolism is therefore important. To find out whether HepG2 cells are suitable for this purpose, we investigated the effect of three established fatty acid metabolism inhibitors and of three test compounds on triglyceride accumulation, palmitate metabolism, the acylcarnitine pool and dicarboxylic acid accumulation in the cell supernatant and on ApoB-100 excretion in HepG2 cells...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29730893/immunoglobulin-a-and-protein-content-of-low-fat-human-milk-prepared-for-the-treatment-of-chylothorax
#3
Michelle Drewniak, Chris C M Waterhouse, Andrew W Lyon, Tanis R Fenton
BACKGROUND: Several case studies report successful recovery from chylothorax while infants were fed low-fat human milk. The reported growth rates were inadequate despite milk supplementation with added medium-chain triglycerides (MCTs). The objective was to determine the effect that various human milk fat separating methods, refrigerated centrifuge, room temperature centrifuge, and refrigeration have on the loss of immunoglobulin A (IgA) and protein in the preparation of low-fat human milk...
December 14, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/29709912/influence-of-polysorbate-60-on-formulation-properties-and-bioavailability-of-morin-loaded-nanoemulsions-with-and-without-low-saponification-degree-polyvinyl-alcohol
#4
Yuri Ikeuchi-Takahashi, Ayaka Kobayashi, Chizuko Ishihara, Takumi Matsubara, Hiroaki Matsubara, Hiraku Onishi
The aim of the present study was to investigate the influence of polysorbate 60 (Tween 60) on the development of morin-loaded nanoemulsions to improve the oral bioavailability of morin. Nanoemulsions were prepared using Tween 60 and polyvinyl alcohol (PVA) as emulsifiers, and medium chain triglycerides (MCT) as the lipid base. Low-saponification-degree PVA (LL-810) was also added to stabilize dispersed droplets. MCT-LL810 nanoemulsion containing LL-810 was prepared with a reduced amount of Tween 60. However, the area under the blood concentration-time curve (AUC) of MCT-LL810 (0...
2018: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/29701491/effects-of-shiitake-intake-on-serum-lipids-in-rats-fed-different-high-oil-or-high-fat-diets
#5
Norihiko Asada, Rumi Kairiku, Mika Tobo, Akifumi Ono
Shiitake (Lentinula edodes) extract, eritadenine, has been shown to reduce cholesterol levels, and its hypocholesterolemic actions are involved in the metabolism of methionine. However, the mechanisms by which eritadenine affects cholesterol metabolism in animals fed a high-fat diet containing different sources of lipids have not yet been elucidated in detail. This study was conducted to investigate the effects of shiitake supplementation on serum lipid concentrations in rats fed a diet including a high amount of a plant oil (HO [high oil] and HOS [high oil with shiitake] groups), animal fat (HF [high fat] and HFS [high fat with shiitake] groups), or MCT- (medium-chain triglyceride-) rich plant oil (HM [high MCT] and HMS [high MCT with shiitake] groups)...
April 27, 2018: Journal of Dietary Supplements
https://www.readbyqxmd.com/read/29690945/a-protocol-to-decrease-postoperative-chylous-effusion-duration-in-children
#6
Melissa M Winder, Aaron W Eckhauser, Claudia Delgado-Corcoran, Randall J Smout, Jennifer Marietta, David K Bailly
OBJECTIVE: Our primary goal was to decrease time to resolution of postoperative chylothorax as demonstrated by total days of chest tube utilisation through development and implementation of a management protocol. METHODS: A chylothorax management protocol was implemented as a quality improvement project at a tertiary-care paediatric hospital in July, 2015. Retrospective analysis was completed on patients aged 0-17 years diagnosed with chylothorax within 30 days of cardiac surgery in a pre-protocol cohort (February, 2014 to June, 2015, n=20) and a post-protocol cohort (July, 2015 to March, 2016, n=22)...
April 25, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29683102/solid-lipid-particles-as-drug-carriers-effects-of-particle-preparation-methods-and-lipid-excipients-on-particle-characteristics
#7
Cintia Kieffer Gamboa, Ranoda Samir, Chengyu Wu, Huiling Mu
BACKGROUND: Oral delivery of biopharmaceuticals is limited by the absorption barriers in the gastrointestinal tract (GIT) such as low permeability across the biological membranes and the enzymatic degradation by proteases. In this study, lipid based drug delivery systems were proposed to overcome these obstacles. OBJECTIVE: The aim of this study was to evaluate the effect of particle preparation methods and excipients on the characteristics of solid lipid microparticles (SLM) and solid lipid nanoparticles (SLN)...
April 20, 2018: Pharmaceutical Nanotechnology
https://www.readbyqxmd.com/read/29651749/medium-chain-triglycerides-supplement-therapy-with-a-low-carbohydrate-formula-can-supply-energy-and-enhance-ammonia-detoxification-in-the-hepatocytes-of-patients-with-adult-onset-type-ii-citrullinemia
#8
Kiyoshi Hayasaka, Chikahiko Numakura, Mitsunori Yamakawa, Tetsuo Mitsui, Hisayoshi Watanabe, Hiroaki Haga, Masahide Yazaki, Hiromasa Ohira, Yasuo Ochiai, Toshiyuki Tahara, Tamio Nakahara, Noriyo Yamashiki, Takahiro Nakayama, Takashi Kon, Hiroshi Mitsubuchi, Hiroshi Yoshida
Citrin, encoded by SLC25A13, constitutes the malate-aspartate shuttle, the main NADH-shuttle in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Citrin deficiency is predicted to impair hepatic glycolysis and de novo lipogenesis, resulting in hepatic energy deficit. Secondary decrease in hepatic argininosuccinate synthetase (ASS1) expression has been considered a cause of hyperammonemia in CTLN2. We previously reported that medium-chain triglyceride (MCT) supplement therapy with a low-carbohydrate formula was effective in CTLN2 to prevent a relapse of hyperammonemic encephalopathy...
April 12, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29649104/coordination-of-gpr40-and-ketogenesis-signaling-by-medium-chain-fatty-acids-regulates-beta-cell-function
#9
Julien Benjamin Pujol, Nicolas Christinat, Yann Ratinaud, Claudia Savoia, Siobhan E Mitchell, El Hadji M Dioum
Diabetes prevalence increases with age, and β-cell dysfunction contributes to the incidence of the disease. Dietary lipids have been recognized as contributory factors in the development and progression of the disease. Unlike long chain triglycerides, medium chain triglycerides (MCT) increase fat burning in animal and human subjects as well as serum C-peptide in type 2 diabetes patients. We evaluated the beneficial effects of MCT on β-cells in vivo and in vitro. MCT improved glycemia in aged rats via β-cell function assessed by measuring insulin secretion and content...
April 12, 2018: Nutrients
https://www.readbyqxmd.com/read/29643556/a-prospective-observational-study-of-injection-pain-in-children-with-medium-plus-long-chain-triglyceride-and-long-chain-triglyceride-propofol-premixed-with-lignocaine
#10
Bhavika Singla, Anila D Malde
Background and Aims: Propofol injection pain is an unresolved problem in children. Although medium and long chain triglyceride (MCT-LCT) propofol has shown promising results in adults, its efficacy in children is not proven. In a prospective observational study the incidence and severity of pain with MCT-LCT and LCT propofol in children was compared. Methods: After obtaining approval from the Institutional Ethics Committee, 170 children (age group 6 months to 8 years) scheduled for various surgeries were included in this study...
March 2018: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/29625812/scn2a-mutation-in-an-infant-presenting-with-migrating-focal-seizures-and-infantile-spasm-responsive-to-a-ketogenic-diet
#11
Da-Jyun Su, Jyh-Feng Lu, Li-Ju Lin, Jao-Shwann Liang, Kun-Long Hung
SCN2A mutations have been identified in various encephalopathy phenotypes, ranging from benign familial neonatal-infantile seizure (BFNIS) to more severe forms of epileptic encephalopathy such as Ohtahara syndrome or epilepsy of infancy with migrating focal seizure (EIMFS). Thus far, no particularly effective treatment is available for severe epileptic encephalopathy caused by SCN2A mutations in children. We present the case of a boy who developed seizures on the third day of life and received a diagnosis of EIMFS based on his clinical presentations and electroencephalography reports...
April 3, 2018: Brain & Development
https://www.readbyqxmd.com/read/29596218/dietary-supplementation-with-medium-chain-triglycerides-reduces-candida-gastrointestinal-colonization-in-preterm-infants
#12
Amanda B Arsenault, Kearney T W Gunsalus, Sonia S Laforce-Nesbitt, Lynn Przystac, Erik J DeAngelis, Michaela E Hurley, Ethan S Vorel, Richard Tucker, Nirupa R Matthan, Alice H Lichtenstein, Carol A Kumamoto, Joseph M Bliss
BACKGROUND: Candida is an important cause of infections in premature infants. Gastrointestinal colonization with Candida is a common site of entry for disseminated disease. The objective of this study was to determine whether a dietary supplement of medium-chain triglycerides (MCTs) reduces Candida colonization in preterm infants. METHODS: Preterm infants with Candida colonization (n=12) receiving enteral feedings of either infant formula (n=5) or breastmilk (n=7) were randomized to MCT supplementation (n=8) or no supplementation (n=4)...
March 24, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29576959/the-use-of-nutritional-supplements-to-induce-ketosis-and-reduce-symptoms-associated-with-keto-induction-a-narrative-review
#13
Cliff J D C Harvey, Grant M Schofield, Micalla Williden
Background: Adaptation to a ketogenic diet (keto-induction) can cause unpleasant symptoms, and this can reduce tolerability of the diet. Several methods have been suggested as useful for encouraging entry into nutritional ketosis (NK) and reducing symptoms of keto-induction. This paper reviews the scientific literature on the effects of these methods on time-to-NK and on symptoms during the keto-induction phase. Methods: PubMed, Science Direct, CINAHL, MEDLINE, Alt Health Watch, Food Science Source and EBSCO Psychology and Behavioural Sciences Collection electronic databases were searched online...
2018: PeerJ
https://www.readbyqxmd.com/read/29559402/synergistic-effect-of-plga-nanoparticles-and-submicron-triglyceride-droplets-in-enhancing-the-intestinal-solubilisation-of-a-lipophilic-weak-base
#14
Paul Joyce, Clive A Prestidge
A novel hybrid microparticulate system composed of poly(lactic-co-glycolic) acid (PLGA) nanoparticles and submicron medium-chain triglyceride (MCT) droplets was fabricated to overcome the pH-dependent solubility and precipitation challenges associated with a model poorly water-soluble weak base, cinnarizine (CIN). Molecular CIN was confined within both the lipid and polymer phase of PLGA-lipid hybrid (PLH) and PLGA-lipid-mannitol hybrid (PLMH) particles, which offered significant biopharmaceutical advantages in comparison to the unformulated drug, submicron MCT droplets and PLGA nanoparticles...
March 17, 2018: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29556839/proposal-for-an-individualized-dietary-strategy-in-patients-with-very-long-chain-acyl-coa-dehydrogenase-deficiency
#15
Jeannette C Bleeker, Irene L Kok, Sacha Ferdinandusse, Maaike de Vries, Terry G J Derks, Margot F Mulder, Monique Williams, Estela Rubio Gozalbo, Annet M Bosch, Dorine T van den Hurk, Monique G M de Sain-van der Velden, Hans R Waterham, Frits A Wijburg, Gepke Visser
BACKGROUND: Patients with very long chain acyl-CoA dehydrogenase deficiency (VLCADD), a long chain fatty acid oxidation disorder, are traditionally treated with a long chain triglyceride (LCT) restricted and medium chain triglyceride (MCT) supplemented diet. Introduction of VLCADD in newborn screening (NBS) programs has led to the identification of asymptomatic newborns with VLCADD, who may have a more attenuated phenotype and may not need dietary adjustments. OBJECTIVE: To define dietary strategies for individuals with VLCADD based on the predicted phenotype...
March 19, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29529462/oil-in-water-emulsion-impregnated-electrospun-poly-ethylene-terephthalate-fiber-mat-as-a-novel-tool-for-optical-fiber-cleaning
#16
Dries J G Devlaminck, Md Mahbubor Rahman, Mamoni Dash, Sangram Keshari Samal, Jan Watté, Sandra Van Vlierberghe, Peter Dubruel
HYPOTHESIS: The complete removal of remaining polymer debris after stripping of optical fiber cables is essential for high precision connection between two fibers. It can be anticipated that electrospun porous membranes as cleaning wipes are able to trap and retain polymer debris within their pores. Impregnation of an oil-in-water emulsion as cleaning agent lowers the interfacial tension between debris and the optical fiber thereby enabling the straightforward removal of polymer debris from the optical fiber...
June 15, 2018: Journal of Colloid and Interface Science
https://www.readbyqxmd.com/read/29520223/anxiolytic-effect-of-exogenous-ketone-supplementation-is-abolished-by-adenosine-a1-receptor-inhibition-in-wistar-albino-glaxo-rijswijk-rats
#17
Zsolt Kovács, Dominic P D'Agostino, Csilla Ari
Anxiety disorders are one of the most common mental health problems worldwide, but the exact pathophysiology remains largely unknown. It has been demonstrated previously that administration of exogenous ketone supplement KSMCT (ketone salt/KS + medium chain triglyceride/MCT oil) by intragastric gavage for 7 days decreased the anxiety level in genetically absence epileptic Wistar Albino Glaxo/Rijswijk (WAG/Rij) rats. To investigate the potential role of the adenosinergic system in the pathomechanism of anxiety we tested whether the inhibition of adenosine A1 receptors (A1 Rs) influence the anxiolytic effect of the exogenous ketone supplement...
2018: Frontiers in Behavioral Neuroscience
https://www.readbyqxmd.com/read/29508361/surgical-chylothorax-in-neonates-management-and-outcomes
#18
Karina Miura Costa, Amulya Kumar Saxena
BACKGROUND: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. METHODS: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). RESULTS: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23...
April 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29505711/mpeg-5k-b-plga-2k-pcl-3-4k-mct-mixed-micelles-as-carriers-of-disulfiram-for-improving-plasma-stability-and-antitumor-effect-in-vivo
#19
Linlin Miao, Jia Su, Xuezhi Zhuo, Lifeng Luo, Yihan Kong, Jingxin Gou, Tian Yin, Yu Zhang, Haibing He, Xing Tang
The clinical application of disulfiram (DSF) in cancer treatments is hindered by its rapid degradation in the blood circulation. In this study, methoxy poly(ethylene glycol)- b-poly(lactide- co-glycolide)/poly(ε-caprolactone) (mPEG5k - b-PLGA2k /PCL3.4k ) micelles were developed for encapsulation of DSF by using the emulsification-solvent diffusion method. Medium chain triglyceride (MCT) was incorporated into the mixed polymeric micelles to improve drug loading by reducing the core crystallinity. Differential scanning calorimetry (DSC) results implied that DSF is likely present in an amorphous form within the micelles, and is well dispersed...
April 2, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29505002/case-report-of-primary-intestinal-lymphangiectasia-diagnosed-in-an-octogenarian-by-ileal-intubation-and-by-push-enteroscopy-after-missed-diagnosis-by-standard-colonoscopy-and-egd
#20
Mitchell S Cappell, Ahmed Edhi, Mitual Amin
RATIONALE: Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation...
January 2018: Medicine (Baltimore)
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