1,080,993 results match your criteria Metabolic Disease & Stroke - MELAS


[Study of epidemiological characteristics of metabolic syndrome and influencing factors in elderly people in China].

Zhonghua Liu Xing Bing Xue Za Zhi 2019 Mar;40(3):284-289

National Center for Chronic and Non-communicable Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing 100050, China.

To estimate the prevalence of MS in elderly people aged ≥60 years and its related factors in China and provide scientific evidence for prevention and control of MS in the elderly. Data used in this study were obtained from the 2013 Chinese Chronic Diseases and Risk Factor Surveillance Program. A total of 50 497 people aged ≥60 years were selected and interviewed through multistage stratified cluster sampling at 298 surveillance sites in 31 provinces. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0254-6450.2019.03.006DOI Listing

Establishment of gene-edited pigs expressing human blood coagulation factor VII and albumin for bioartificial liver use.

J Gastroenterol Hepatol 2019 Mar 18. Epub 2019 Mar 18.

Department of Hepatobiliary Surgery II, Guangdong Provincial Research Center for Artificial Organ and Tissue Engineering, Institute of Regenerative Medicine, Zhujiang Hospital, Southern Medical University.

Background And Aim: Bioartificial livers (BALs) are considered as a solution to bridge patients with acute liver failure to liver transplantation or to assist in spontaneous recovery for patients with end-stage liver disease. Pig is the best donor of hepatocytes for BALs in clinical trials, because metabolic and detoxification function of its liver are close to human. However, using pig hepatocytes for BALs remains controversial for safety concern owing to nonhuman proteins secretion. Read More

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http://dx.doi.org/10.1111/jgh.14666DOI Listing

Misery perfusion and amyloid deposition in atherosclerotic major cerebral artery disease.

Neuroimage Clin 2019 Mar 12;22:101762. Epub 2019 Mar 12.

Division of PET Imaging, Shiga Medical Centre Research Institute, Moriyama, Japan; National Institute of Radiological Sciences, National Institutes of Quantum and Radiological Science and Technology, Chiba, Japan.

Although experimental studies have shown that global cerebral hypoperfusion leads to amyloid deposition in the hemisphere with carotid artery occlusion in rodents, the results of such occurrence are controversial in humans. Hence, we aim to determine whether global cerebral hypoperfusion leading to decreased blood flow relative to metabolic demand [increased oxygen extraction fraction (OEF), misery perfusion] is associated with increases in amyloid deposition in the hemisphere with atherosclerotic major cerebral artery disease in patients. We evaluated the distribution of β-amyloid plaques using positron emission tomography and a [18F]-pyridylbenzofuran derivative (F-FPYBF-2) in 13 patients with unilateral atherosclerotic disease of the internal carotid artery (ICA) or middle cerebral artery (MCA) disease and no cortical infarction. Read More

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http://dx.doi.org/10.1016/j.nicl.2019.101762DOI Listing

Clinical Utility of Carotid Ultrasonography - Application for the Management of Patients with Diabetes.

J Diabetes Investig 2019 Mar 18. Epub 2019 Mar 18.

Department of Metabolic Medicine, Osaka University Graduate School of Medicine.

Carotid ultrasonography is a noninvasive, simple, and inexpensive modality to assess the severity of atherosclerosis. This article reviews related articles, summarizes the rationale for the application of carotid ultrasonography in clinical practice, and addresses the features and the limitations of carotid ultrasonography in cardiovascular risk prediction. Numerous large studies have confirmed that various carotid ultrasound measures, such as carotid intima-media thickness (CIMT), presence or absence of carotid plaque, plaque number, and plaque area, can be independent predictors of cardiovascular diseases (CVD) in subjects with and without diabetes mellitus. Read More

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http://dx.doi.org/10.1111/jdi.13042DOI Listing

Chinese clinical guidelines for continuous glucose monitoring (2018 edition).

Diabetes Metab Res Rev 2019 Mar 18:e3152. Epub 2019 Mar 18.

Changhai Hospital Affiliated to the Second Military Medical University.

Blood glucose monitoring is an important part of diabetes management. Continuous glucose monitoring (CGM) technology has become an effective complement to conventional blood glucose monitoring methods and has been widely applied in clinical practice. The indications for its use, the accuracy of the generated data, the interpretation of the CGM results and the application of the results must be standardized. Read More

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http://dx.doi.org/10.1002/dmrr.3152DOI Listing

Mechanisms for the maintenance and regulation of axonal energy supply.

J Neurosci Res 2019 Mar 18. Epub 2019 Mar 18.

The Porter Neuroscience Research Center, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.

The unique polarization and high-energy demand of neurons necessitates specialized mechanisms to maintain energy homeostasis throughout the cell, particularly in the distal axon. Mitochondria play a key role in meeting axonal energy demand by generating adenosine triphosphate through oxidative phosphorylation. Recent evidence demonstrates how axonal mitochondrial trafficking and anchoring are coordinated to sense and respond to altered energy requirements. Read More

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http://dx.doi.org/10.1002/jnr.24411DOI Listing

The role of telenursing in the management of diabetes:A systematic review and meta-analysis.

Public Health Nurs 2019 Mar 18. Epub 2019 Mar 18.

Anhui Medical College, Hefei, China.

Background: Diabetes is a common chronic disease that requires a long-term regimen. However, the management of diabetes by telenursing is limited and inconclusive.

Objectives: To determine the effectiveness of telenursing on control in diabetes. Read More

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http://dx.doi.org/10.1111/phn.12603DOI Listing

Lin28a expression protects against streptozotocin-induced β-cell destruction and prevents diabetes in mice.

Cell Biochem Funct 2019 Mar 18. Epub 2019 Mar 18.

Department of Food Science and Nutrition, Kyungpook National University, Sangju, Republic of Korea.

Lin28, which is highly expressed during embryogenesis, has been shown to play an important role in cell growth and embryonic development. Meanwhile, Lin28 represses let-7 miRNA biogenesis and block pre-let-7 processing in the cytoplasm. The let-7 family of miRNAs is known to repress oncogenesis and cell cycle progression by targeting oncogenic genes and signalling pathways. Read More

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http://dx.doi.org/10.1002/cbf.3376DOI Listing

Circulating Adipokine VASPIN Is Associated with Serum Lipid Profiles in Humans.

Lipids 2019 Mar 18. Epub 2019 Mar 18.

Department of Medicine, Division of Endocrinology and Nephrology, University of Leipzig, Liebigstrasse 21, 04103, Leipzig, Germany.

VASPIN, visceral adipose tissue-derived serpin, is an adipokine ameliorating insulin resistance in obesity. Here, we investigated the role of VASPIN and its genetic variants in lipid metabolism. We measured serum VASPIN concentrations by ELISA in 823 metabolically well-characterized Caucasian subjects (Sorbs from Germany). Read More

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http://dx.doi.org/10.1002/lipd.12139DOI Listing

Challenging metabolic tissues with fructose: Tissue-specific and sex-specific responses.

J Physiol 2019 Mar 18. Epub 2019 Mar 18.

Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford.

Excessive consumption of free sugars (which typically includes a composite of glucose and fructose) is associated with an increased risk of developing chronic metabolic diseases including obesity, non-alcoholic fatty liver disease (NAFLD), type 2 diabetes, and cardiovascular disease. Determining the utilisation, storage and fate of dietary sugars in metabolically relevant tissues is fundamental to understanding their contribution to metabolic disease risk. To date, the study of fructose metabolism has primarily focussed on the liver, where it has been implicated in impaired insulin sensitivity, increased fat accumulation and dyslipidaemia. Read More

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http://dx.doi.org/10.1113/JP277115DOI Listing

Association of SOST gene polymorphisms with peak bone mineral density in Chinese nuclear families with male-offspring.

Acta Biochim Biophys Sin (Shanghai) 2019 Mar;51(3):341-343

The Third Affiliated Hospital of Soochow University, Changzhou, China.

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http://dx.doi.org/10.1093/abbs/gmy175DOI Listing

Metabolomic analysis of male combat veterans with post traumatic stress disorder.

PLoS One 2019 18;14(3):e0213839. Epub 2019 Mar 18.

Department of Psychiatry and UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA, United States of America.

Posttraumatic stress disorder (PTSD) is associated with impaired major domains of psychology and behavior. Individuals with PTSD also have increased co-morbidity with several serious medical conditions, including autoimmune diseases, cardiovascular disease, and diabetes, raising the possibility that systemic pathology associated with PTSD might be identified by metabolomic analysis of blood. We sought to identify metabolites that are altered in male combat veterans with PTSD. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213839PLOS

Platelet Indices Alterations in Children With Type 1 Diabetes Mellitus.

J Pediatr Hematol Oncol 2019 Mar 15. Epub 2019 Mar 15.

Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht.

Platelet (PLT) hyperactivity is a key factor which contributes to cardiovascular complications in patients with type 2 diabetes mellitus even in preclinical stages of disease. To the best of our knowledge, there is limited researches in this regard among patients with type 1 diabetes. The aim of this study was to evaluate hematologic indices indicating PLT activity in children with type 1 diabetes. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001454DOI Listing

Neuronal Apolipoprotein E4 Expression Results in Proteome-Wide Alterations and Compromises Bioenergetic Capacity by Disrupting Mitochondrial Function.

J Alzheimers Dis 2019 Mar 11. Epub 2019 Mar 11.

Gladstone Institute of Neurological Disease, San Francisco, CA, USA.

Apolipoprotein (apo) E4, the major genetic risk factor for Alzheimer's disease (AD), alters mitochondrial function and metabolism early in AD pathogenesis. When injured or stressed, neurons increase apoE synthesis. Because of its structural difference from apoE3, apoE4 undergoes neuron-specific proteolysis, generating fragments that enter the cytosol, interact with mitochondria, and cause neurotoxicity. Read More

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http://dx.doi.org/10.3233/JAD-181184DOI Listing

The lysosomal storage disorders mucolipidosis type II, type III alpha/beta and type III gamma: Update on GNPTAB and GNPTG mutations.

Hum Mutat 2019 Mar 18. Epub 2019 Mar 18.

Section Biochemistry, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Mutations in the GNPTAB and GNPTG genes cause mucolipidosis (ML) type II, type III alpha/beta and type III gamma, which are autosomal recessively inherited lysosomal storage disorders. GNPTAB and GNPTG encode the α/β-precursor and the γ-subunit of N-acetylglucosamine (GlcNAc)-1-phosphotransferase, respectively, the key enzyme for the generation of mannose 6-phosphate targeting signals on lysosomal enzymes. Defective GlcNAc-1-phosphotransferase results in missorting of lysosomal enzymes and accumulation of non-degradable macromolecules in lysosomes, strongly impairing cellular function. Read More

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http://dx.doi.org/10.1002/humu.23748DOI Listing

MST kinases and metabolism.

Endocrinology 2019 Mar 18. Epub 2019 Mar 18.

Departamento de Fisioloxía and Centro de Investigación en Medicina Molecular e Enfermidades Crónicas (CIMUS), Instituto de Investigación Sanitaria de Santiago (IDIS), Universidade de Santiago de Compostela, Spain.

Since the discovery of the MST kinase family of proteins (MST1/STK4, MST2/STK3, MST3/STK24 and SOK1/STK25), much has been done that adds to our knowledge of their structure, regulation and function. In the last few years, a series of articles have unveiled a previous unknown relation of these kinases with metabolic regulation and the homeostasis of metabolic tissues. The aim of this review is to bring together this body of data to have a detailed picture of the current knowledge related with these proteins, metabolism, and some of its associated diseases. Read More

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http://dx.doi.org/10.1210/en.2018-00898DOI Listing

Nonalcoholic fatty liver disease represents a greater metabolic burden in patients with atherosclerosis: A cross-sectional study.

Medicine (Baltimore) 2019 Mar;98(11):e14896

Department of Endocrinology and Metabolism, Shandong Provincial Hospital affiliated to Shandong University, Jinan.

How nonalcoholic fatty liver disease (NAFLD) is linked to atherosclerosis is still disputed. This study aimed to explore the association between NAFLD and atherosclerosis among adults in Shandong province, China.A total of 6849 individuals were enrolled in the final analyses for a community-based study. Read More

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http://dx.doi.org/10.1097/MD.0000000000014896DOI Listing

Latin American Consensus on the management of hypertension in the patient with diabetes and the metabolic syndrome.

J Hypertens 2019 Mar 14. Epub 2019 Mar 14.

Istituto Auxologico Italiano, IRCCS, and Università degli Studi of Milan, Italy.

: The prevalence of hypertension, type 2 diabetes mellitus (DM2) and the metabolic syndrome continues to increase in Latin America, while the rates of diagnosis, treatment and control of these disorders remain low. The frequency of the risk factors that constitute the metabolic syndrome and are associated with an increased risk of cardiovascular disease has not diminished since the publication of the previous consensus. This document discusses the socioeconomic, demographic, environmental and cultural characteristics of most associated Latin American countries and partially explains the lack of better results in improving clinical and public health actions that allow high morbidity and mortality rates caused by cardiovascular diseases and DM2 to be reduced through programs aligned with the so-called precision medicine, which should be predictive, preventive, personalized and participatory. Read More

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http://dx.doi.org/10.1097/HJH.0000000000002072DOI Listing

Risk of Stroke in Liver Cirrhosis: A Systematic Review and Meta-Analysis.

J Clin Gastroenterol 2019 Mar 13. Epub 2019 Mar 13.

Liver Cirrhosis Study Group, Department of Gastroenterology, General Hospital of Northern Theater Command (formerly General Hospital of Shenyang Military Area), Shenyang.

Background And Aims: Liver cirrhosis can lead to abnormal coagulation, rendering patients at risk for bleeding but also thrombotic complications. We conducted a systematic review and meta-analysis to explore the epidemiology of stroke in liver cirrhosis and the potential association between them.

Materials And Methods: Studies were searched through the PubMed, EMBASE, and Cochrane Library databases. Read More

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http://dx.doi.org/10.1097/MCG.0000000000001201DOI Listing

Insulin at the Crossroads of Metabolic, Neurologic, and Vasculature Disease.

Authors:
Steven K Malin

Exerc Sport Sci Rev 2019 Apr;47(2):64-65

Department of Kinesiology, University of Virginia Charlottesville, VA.

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http://dx.doi.org/10.1249/JES.0000000000000187DOI Listing

Effects of Canagliflozin on Heart Failure Outcomes Associated with Preserved and Reduced Ejection Fraction in Type 2 Diabetes: Results from the CANVAS Program.

Circulation 2019 Mar 17. Epub 2019 Mar 17.

Senior Director's Office, The George Institute for Global Health, Australia.

Patients with type 2 diabetes mellitus are at high risk of developing heart failure (HF). Sodium glucose co-transporter 2 (SGLT2) inhibitors have been demonstrated, in large scale trials, to reduce the risk of HF events in patients with type 2 diabetes deemed to be at high risk based on established cardiovascular disease or multiple risk factors. However, it is unclear whether benefits are experienced across the broad spectrum of HF patients that includes those with preserved (HFpEF) as well as reduced ejection fraction (HFrEF). Read More

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https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.119.0
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http://dx.doi.org/10.1161/CIRCULATIONAHA.119.040057DOI Listing
March 2019
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A Metabolomic Study on the Intervention of Traditional Chinese Medicine Qushi Huayu Decoction on Rat Model of Fatty Liver Induced by High-Fat Diet.

Biomed Res Int 2019 7;2019:5920485. Epub 2019 Feb 7.

Institute of Liver Disease, Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai 201203, China.

Qushi Huayu Decoction (QHD), an important clinically proved herbal formula, has been reported to be effective in treating fatty liver induced by high-fat diet in rats. However, the mechanism of action has not been clarified at the metabolic level. In this study, a urinary metabolomic method based on gas chromatography-mass spectrometry (GC-MS) coupled with pattern recognition analysis was performed in three groups (control, model, and QHD group), to explore the effect of QHD on fatty liver and its mechanism of action. Read More

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http://dx.doi.org/10.1155/2019/5920485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383432PMC
February 2019

Effectiveness of health education-based conventional intervention method to reduce noncommunicable diseases risk factors among rural population.

Cardiovasc Diagn Ther 2019 Feb;9(1):30-34

Department of Noncommunicable Diseases, Bangladesh University of Health Sciences (BUHS), Dhaka, Bangladesh.

Background: Noncommunicable diseases (NCDs) are the major public health challenges that can be averted through prevention and control of behavioral risk factors. The study aimed to assess the effectiveness of health education-based conventional intervention method to reduce NCD risk factors among a selected rural population of Bangladesh.

Methods: A quasi-experimental study was conducted among 80 adults of Basulia village. Read More

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http://dx.doi.org/10.21037/cdt.2018.10.09DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6382656PMC
February 2019

Quinolone-isoniazid hybrids: synthesis and preliminary cytotoxicity and anti-tuberculosis evaluation.

Medchemcomm 2019 Feb 11;10(2):326-331. Epub 2019 Jan 11.

Faculty of Science , Department of Chemistry , Rhodes University , Grahamstown 6140 , South Africa . Email:

Herein, we propose novel quinolones incorporating an INH moiety as potential drug templates against TB. The quinolone-based compounds bearing an INH moiety attached a hydrazide-hydrazone bond were synthesised and evaluated against H37Rv (MTB). The compounds were also evaluated for cytotoxicity against HeLa cell lines. Read More

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http://dx.doi.org/10.1039/c8md00480cDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390685PMC
February 2019

A Novel Heterozygous Mutation of the Gene Causes a Peculiar Phenotype without Hematuria and Renal Function Impairment in a Chinese Family.

Dis Markers 2019 10;2019:8705989. Epub 2019 Feb 10.

Department of Otolaryngology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, No. 600, Yishan Road, Xuhui District, 200233 Shanghai, China.

Mutations in the gene are frequently reported to be associated with various types of hereditary nephropathy. encodes the 3 chain of type IV collagen, which is the main structural protein in the basement membrane. Mutations in this gene are always related to kidney performance, and deafness and ocular lesion have also been reported. Read More

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http://dx.doi.org/10.1155/2019/8705989DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6387717PMC
February 2019

Determination of the complexity and diversity of the TCR β-chain CDR3 repertoire in bladder cancer using high-throughput sequencing.

Oncol Lett 2019 Apr 5;17(4):3808-3816. Epub 2019 Feb 5.

Nephrology Department, Guangxi Key Laboratory of Metabolic Diseases Research, Guilin No. 181 Hospital (The Affiliated Guilim Hospital of Southern Medical University), Guilin, Guangxi 541002, P.R. China.

The present study aimed to investigate the complexity and diversity of the T lymphocyte immune repertoire in patients with bladder cancer. To do so, the immune state of patients was assessed. The study also aimed to elucidate the aetiology and pathogenesis of bladder cancer to provide a novel theoretical basis for disease prevention, diagnosis, treatment and prognosis monitoring. Read More

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http://dx.doi.org/10.3892/ol.2019.10015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6403507PMC

Age and Gender Specific Thyroid Hormones and Their Relationships with Body Mass Index in a Large Chinese Population.

Int J Endocrinol Metab 2019 Jan 23;17(1):e66450. Epub 2019 Jan 23.

Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China.

Objectives: To investigate the associations of thyroid-stimulating hormone (TSH), free triiodothyronine (FT3) and free thyroxine (FT4) with body mass index (BMI) and the effect of age and gender on these relationships in a large Chinese population.

Methods: A total of 97,997 individuals from a health examination center were selected. The medians (25th and 75th) of TSH, FT3 and FT4 were used to explore the trends based on gender, 10-year age groups and BMI. Read More

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http://dx.doi.org/10.5812/ijem.66450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408740PMC
January 2019

Is there a relationship between serum vaspin levels and insulin resistance in chronic renal failure?

Pak J Med Sci 2019 Jan-Feb;35(1):230-235

Huseyin Celiker Deparment of Nephrology, Firat University Medical Faculty, Elazig, Turkey.

Objective: Chronic kidney disease (CKD) patients have insulin secretion disorders and resistance to insulin effects, that is responsible for the development of cardiovascular events. Vaspin is an adipocytokine that regulates glucose and lipid metabolism. We aimed to determine the serum vaspin levels and its relationship with insulin resistance in CKD patients. Read More

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http://dx.doi.org/10.12669/pjms.35.1.96DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408677PMC

Plant-Derived Polyphenols Modulate Human Dendritic Cell Metabolism and Immune Function via AMPK-Dependent Induction of Heme Oxygenase-1.

Front Immunol 2019 1;10:345. Epub 2019 Mar 1.

School of Biochemistry and Immunology and School of Medicine, Trinity Biomedical Sciences Institute, Trinity College Dublin, The University of Dublin, Dublin, Ireland.

Polyphenols are important immunonutrients which have been investigated in the context of inflammatory and autoimmune disease due to their significant immunosuppressive properties. However, the mechanism of action of many polyphenols is unclear, particularly in human immune cells. The emerging field of immunometabolism has highlighted the significance of metabolic function in the regulation of immune cell activity, yet the effects of polyphenols on immune cell metabolic signaling and function has not been explored. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00345DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6405514PMC

Endocrine Disrupting Chemicals: An Occult Mediator of Metabolic Disease.

Front Endocrinol (Lausanne) 2019 1;10:112. Epub 2019 Mar 1.

Department of Endocrinology & Diabetes, Hygeia Hospital, Athens, Greece.

Endocrine disrupting chemicals (EDCs), a heterogeneous group of exogenous chemicals that can interfere with any aspect of endogenous hormones, represent an emerging global threat for human metabolism. There is now considerable evidence that the observed upsurge of metabolic disease cannot be fully attributed to increased caloric intake, physical inactivity, sleep deficit, and ageing. Among environmental factors implicated in the global deterioration of metabolic health, EDCs have drawn the biggest attention of scientific community, and not unjustifiably. Read More

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http://dx.doi.org/10.3389/fendo.2019.00112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6406073PMC

Platelet-rich plasma in the management of chronic low back pain: a critical review.

J Pain Res 2019 25;12:753-767. Epub 2019 Feb 25.

Department of Orthopaedic Surgery, University of California, San Diego, La Jolla, CA 92093-0863, USA.

Low back pain (LBP) is now regarded as the first cause of disability worldwide and should be a priority for future research on prevention and therapy. Intervertebral disc (IVD) degeneration is an important pathogenesis of LBP. Platelet-rich plasma (PRP) is an autologous blood concentrate that contains a natural concentration of autologous growth factors and cytokines and is currently widely used in the clinical setting for tissue regeneration and repair. Read More

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http://dx.doi.org/10.2147/JPR.S153085DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394242PMC
February 2019

Multiple phenotypic domains of Fabry disease and their relevance for establishing genotype- phenotype correlations.

Appl Clin Genet 2019 5;12:35-50. Epub 2019 Mar 5.

Department of Genetics, Faculty of Medicine, University of Porto, Alameda Hernâni Monteiro, 4200-319 Porto, Portugal,

Fabry disease (FD) is a rare X-linked glycosphingolipidosis resulting from deficient α-galactosidase A (AGAL) activity, caused by pathogenic mutations in the gene. In males, the multisystemic involvement and the severity of tissue injury are critically dependent on the level of AGAL residual enzyme activity (REA) and on the metabolic load of the disease, but organ susceptibility to damage varies widely, with heart appearing as the most vulnerable to storage pathology, even with relatively high REA. The expression of FD can be conceived as a multidomain phenotype, where each of the component domains is the laboratory or clinical expression of the causative mutation along a complex pathophysiologic cascade pathway. Read More

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http://dx.doi.org/10.2147/TACG.S146022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407513PMC

Retention of aberrant cortisol secretion in a patient with bilateral macronodular adrenal hyperplasia after unilateral adrenalectomy.

Ther Clin Risk Manag 2019 27;15:337-342. Epub 2019 Feb 27.

Division of Nephrology, Hypertension and Endocrinology, Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan,

Aberrant cortisol secretion responses after exogenous stimuli such as upright posture, eating a mixed meal or receiving agents influencing aberrant G-protein-coupled receptors in adrenal glands, are often observed in patients with bilateral macronodular adrenal hyperplasia (BMAH). However, little is known about whether this aberrant response is retained after unilateral adrenalectomy. Here, we describe a 61-year-old postmenopausal Japanese woman with unsatisfactorily controlled hypertension who was referred to us for further investigation due to her pre-obesity characteristics (body mass index 28. Read More

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http://dx.doi.org/10.2147/TCRM.S196171DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6398404PMC
February 2019

Effects of prenatal exposure to temephos on behavior and social interaction.

Neuropsychiatr Dis Treat 2019 11;15:669-673. Epub 2019 Mar 11.

Neurobiology Laboratory of Inflammatory and Metabolic Processes, University of Southern Santa Catarina, Tubarão, Santa Catarina, Brazil,

The neurodevelopment period is susceptible to alterations by genetic and environmental factors, such as the exposure to organophosphates (OP). The OP is neurotoxic and has been associated with neurological diseases pathophysiology. The OP temephos is widely used against in Brazil's public health programs. Read More

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http://dx.doi.org/10.2147/NDT.S193896DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6417020PMC

An Evaluation of the Association of Reproductive History and Multiple Births during Adolescence with Postmenopausal Osteoporosis.

Geburtshilfe Frauenheilkd 2019 Mar 11;79(3):300-307. Epub 2018 Oct 11.

Department of Physical Therapy and Rehabilitation, Duzce University Hospital, Duzce, Turkey.

Osteoporosis is the most common metabolic bone disease characterized by low bone mass. Reproductive factors are known to affect bone mineral density (BMD). Calcium loss from maternal bone and decreased BMD have been observed especially during pregnancy and lactation, although this loss has been reported to recover within 6 - 12 months. Read More

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http://dx.doi.org/10.1055/a-0743-7260DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414300PMC

Uric Acid Levels Correlate with Sensory Nerve Function in Healthy Subjects.

Can J Neurol Sci 2019 Mar 18:1-5. Epub 2019 Mar 18.

Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine,University Health Network, University of Toronto,Toronto,Canada.

Background: High levels of uric acid (UA) are associated with various peripheral neuropathies. Furthermore, uric acid levels have been found to correlate with both the clinical and electrophysiological severity of diabetic sensorimotor polyneuropathy, mainly with sensory functions.

Objectives: To determine whether higher UA levels are associated negatively with nerve function in healthy subjects. Read More

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http://dx.doi.org/10.1017/cjn.2019.9DOI Listing

Redifferentiation of a BRAF-K601E mutated poorly differentiated thyroid cancer patient with dabrafenib and trametinib treatment.

Thyroid 2019 Mar 18. Epub 2019 Mar 18.

Gustave Roussy, 46657, Department of Biology and Pathology, Villejuif, Île-de-France, France.

A 59 years old woman with locally invasive poorly differentiated thyroid cancer with synchronous lung, mediastinal and bone metastases and a BRAF-K601E somatic mutation with contra-indication for antiangiogneic drugs was treated with dabrafenib and trametinib. During treatment, serum levels of thyroglobulin increased as soon as day 7 up to 10 folds over baseline at week 4. Concurrently, clinical hyperthyroidism occurred, with free T3 and free T4 levels increasing to 6. Read More

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http://dx.doi.org/10.1089/thy.2018.0457DOI Listing

Cisplatin resistance involves a metabolic reprogramming through ROS and PGC-1α in NSCLC which can be overcome by OXPHOS inhibition.

Free Radic Biol Med 2019 Mar 14. Epub 2019 Mar 14.

Servicio de Oncología Médica, Instituto de Investigación Sanitaria Puerta de Hierro, Hospital Universitario Puerta de Hierro-Majadahonda, Madrid, Spain. Electronic address:

Background: Platinum-based chemotherapy remains the standard of care for most lung cancer cases. However chemoresistance is often developed during the treatment, limiting clinical utility of this drug. Recently, the ability of tumor cells to adapt their metabolism has been associated to resistance to therapies. Read More

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http://dx.doi.org/10.1016/j.freeradbiomed.2019.03.009DOI Listing

Genetic Factors and the Intestinal Microbiome Guide Development of Microbe-based Therapies for Inflammatory Bowel Diseases.

Gastroenterology 2019 Mar 14. Epub 2019 Mar 14.

Department of Pathology, University of California San Diego, La Jolla, California; Chiba University-UC San Diego Center for Mucosal Immunology, Allergy, and Vaccines (CU-UCSD cMAV), La Jolla, California, 92093, USA. Electronic address:

The intestinal microbiota is a dynamic community of bacteria, fungi, and viruses that mediates mucosal homeostasis and physiology. Imbalances in the microbiome and aberrant immune responses to gut bacteria can disrupt homeostasis and are associated with inflammatory bowel diseases (IBD) in humans and colitis in mice. We review genetic variants associated with IBD and their effects on the intestinal microbiome, the immune response, and disease pathogenesis. Read More

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http://dx.doi.org/10.1053/j.gastro.2019.03.017DOI Listing

Integrated radiofrequency array and animal holder design for minimizing head motion during awake marmoset functional magnetic resonance imaging.

Neuroimage 2019 Mar 14. Epub 2019 Mar 14.

Centre for Functional and Metabolic Mapping, Robarts Research Institute, University of Western Ontario, London, Ontario, Canada; Department of Physiology and Pharmacology, University of Western Ontario, London, Ontario, Canada. Electronic address:

Marmosets are small New World primates that are posited to become an important preclinical animal model for studying intractable human brain diseases. A critical step in the development of marmosets as a viable model for human brain dysfunction is to characterize brain networks that are homologous with human network topologies. In this regard, the use of functional magnetic resonance imaging (fMRI) holds tremendous potential for functional brain mapping in marmosets. Read More

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http://dx.doi.org/10.1016/j.neuroimage.2019.03.023DOI Listing

Androgen deprivation therapy and cardiovascular disease.

Urol Oncol 2019 Mar 14. Epub 2019 Mar 14.

Duke Clinical Research Institute, Duke University Medical Center, Durham, NC.

Prostate cancer (PCa) is the most common cancer among men. Advances in early detection and successful treatments have improved cancer-specific survival. With prolonged survival, PCa patients now suffer from the effects of aging and are at increasing risk for the development of cardiovascular (CV) risk factors and CV disease. Read More

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http://dx.doi.org/10.1016/j.urolonc.2019.02.010DOI Listing

Extremely low levels of low-density lipoprotein potentially suggestive of familial hypobetalipoproteinemia: A separate phenotype of NAFLD?

J Clin Lipidol 2019 Feb 14. Epub 2019 Feb 14.

Division of Gastroenterology, Hepatology and Nutrition, University of Cincinnati School of Medicine, Cincinnati, OH, USA.

Background: Low-density lipoprotein cholesterol (LDL-C) levels below 50 mg/dL may suggest familial hypobetalipoproteinemia, particularly in patients with hepatic steatosis. The prevalence of hypobetalipoproteinemia in cohorts with nonalcoholic fatty liver disease (NAFLD) is not known, and it is not clear whether the severity of liver disease of these patients is different. The objective of this study was to address these questions in a large pediatric NAFLD cohort. Read More

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http://dx.doi.org/10.1016/j.jacl.2019.02.002DOI Listing
February 2019

Permanent cardiac pacing for patients with iatrogenic or potentially reversible bradyarrhythmia.

Rev Port Cardiol 2019 Mar 14. Epub 2019 Mar 14.

Serviço de Cardiologia, Centro Hospitalar de Setúbal, Setúbal, Portugal.

Introduction: In iatrogenic or potentially reversible bradyarrhythmia, drug discontinuation or metabolic correction is recommended before permanent cardiac pacemaker (PM) implantation. These patients often have conduction system disease and there are few data on recurrence or the need for a permanent PM.

Objective: To analyze the need for PM implantation in patients with iatrogenic bradyarrhythmia or bradyarrhythmia associated with other potentially reversible causes. Read More

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http://dx.doi.org/10.1016/j.repc.2018.05.018DOI Listing

Lymphatic network remodeling after small bowel resection.

J Pediatr Surg 2019 Feb 28. Epub 2019 Feb 28.

Division of Pediatric Surgery, Department of Surgery, St. Louis Children's Hospital, Washington University in St. Louis School of Medicine, St. Louis, MO. Electronic address:

Background: Short gut syndrome (SGS) following massive small bowel resection (SBR) is a major cause of pediatric mortality and morbidity secondary to nutritional deficiencies and the sequelae of chronic total parenteral nutrition use, including liver steatosis. Despite the importance of lymphatic vasculature in fat absorption, lymphatic response after SBR has not been studied. We hypothesize that lymphatic vessel integrity is compromised in SGS, potentially contributing to the development of impaired lipid transport leading to liver steatosis and metabolic disease. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.02.026DOI Listing
February 2019

Metabolic Reprogramming as a Driver of Fibroblast Activation in Pulmonary Fibrosis.

Am J Med Sci 2019 Feb 12. Epub 2019 Feb 12.

Department of Medicine, Center for Translational Research and Division of Pulmonary, Allergy and Critical Care Medicine, and; Jane & Leonard Korman Respiratory Institute, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address:

Pulmonary fibrosis refers to a heterogeneous group of disorders that scar the lung, most often irreversibly. To date, there are limited effective treatments for these conditions, despite decades of research in this area of investigation. In pulmonary fibrosis, the principle cell responsible for producing the vast majority of scar tissue is the fibroblast, making these cells ideally suited for drug targeting. Read More

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http://dx.doi.org/10.1016/j.amjms.2019.02.003DOI Listing
February 2019

Liver Transplantation for Wilson's Disease in Non-adult Patients: A Systematic Review.

Transplant Proc 2019 Mar 4;51(2):443-445. Epub 2019 Jan 4.

2nd Department of Propaedeutic Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.

Introduction: Wilson's disease (WD) is a rare autosomal recessive disorder transmitted through a gene located on chromosome 13. Liver transplantation (LT) provides a therapeutic option for patients with WD presenting fulminant liver failure or drug resistance. LT in patients with WD has a twofold aim: to save the patient's life when the disorder has progressed to hepatic (or other organ) failure and to cure the underlying metabolic defect. Read More

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http://dx.doi.org/10.1016/j.transproceed.2019.01.017DOI Listing

Influence of Pre-renal Transplant Secondary Hyperparathyroidism on Later Evolution After Transplantation.

Transplant Proc 2019 Mar 12;51(2):344-349. Epub 2018 Dec 12.

Department of Nephrology, University Hospital Reina Sofía, Córdoba, Spain.

Persistence of secondary hyperparathyroidism (SHPT) is common after renal transplantation. Good diagnosis and treatment are important to avoid complications. The objective of our work was to perform a retrospective analysis of the evolution of SHPT after renal transplantation. Read More

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http://dx.doi.org/10.1016/j.transproceed.2018.12.012DOI Listing

Discovery of novel coumarin derivatives as potent and orally bioavailable BRD4 inhibitors based on scaffold hopping.

J Enzyme Inhib Med Chem 2019 Dec;34(1):808-817

a Key Laboratory of Neuropsychiatric Drug Research of Zhejiang Province , Institute of Materia Medica, Zhejiang Academy of Medical Sciences , Hangzhou , PR China.

The bromodomain and extra-terminal (BET) bromodomains, particularly BRD4, have been identified as promising therapeutic targets in the treatment of many human disorders such as cancer, inflammation, obesity, and cardiovascular disease. Recently, the discovery of novel BRD4 inhibitors has garnered substantial interest. Starting from scaffold hopping of the reported compound dihydroquinazolinone (PFI-1), a series of coumarin derivatives were designed and synthesised as a new chemotype of BRD4 inhibitors. Read More

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https://www.tandfonline.com/doi/full/10.1080/14756366.2019.1
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http://dx.doi.org/10.1080/14756366.2019.1587417DOI Listing
December 2019
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HDAC (Histone Deacetylase) Inhibitor Valproic Acid Attenuates Atrial Remodeling and Delays the Onset of Atrial Fibrillation in Mice.

Circ Arrhythm Electrophysiol 2019 Mar;12(3):e007071

Institute of Pharmacology and Toxicology, University of Münster, Germany (B.S., J.S.S., S.H., K.H., F.P., M.D.S., J.S., F.U.M.).

Background: A structural, electrical and metabolic atrial remodeling is central in the development of atrial fibrillation (AF) contributing to its initiation and perpetuation. In the heart, HDACs (histone deacetylases) control remodeling associated processes like hypertrophy, fibrosis, and energy metabolism. Here, we analyzed, whether the HDAC class I/IIa inhibitor valproic acid (VPA) is able to attenuate atrial remodeling in CREM-IbΔC-X (cAMP responsive element modulator isoform IbΔC-X) transgenic mice, a mouse model of extensive atrial remodeling with age-dependent progression from spontaneous atrial ectopy to paroxysmal and finally long-lasting AF. Read More

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http://dx.doi.org/10.1161/CIRCEP.118.007071DOI Listing

Inhibition of ER Stress by 2-Aminopurine Treatment Modulates Cardiomyopathy in a Murine Chronic Chagas Disease Model.

Biomol Ther (Seoul) 2019 Mar 13. Epub 2019 Mar 13.

Department of Microbiology, Biochemistry and Molecular Genetics, Public Health Research Institute, New Jersey Medical School, Newark, NJ 07103, USA.

infection results in debilitating cardiomyopathy, which is a major cause of mortality and morbidity in the endemic regions of Chagas disease (CD). The pathogenesis of Chagasic cardiomyopathy (CCM) has been intensely studied as a chronic inflammatory disease until recent observations reporting the role of cardio-metabolic dysfunctions. In particular, we demonstrated accumulation of lipid droplets and impaired cardiac lipid metabolism in the hearts of cardiomyopathic mice and patients, and their association with impaired mitochondrial functions and endoplasmic reticulum (ER) stress in CD mice. Read More

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http://dx.doi.org/10.4062/biomolther.2018.193DOI Listing
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