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alpha-1 liver disease

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https://www.readbyqxmd.com/read/29326427/severe-steatosis-induces-portal-hypertension-by-systemic-arterial-hyporeactivity-and-hepatic-vasoconstrictor-hyperreactivity-in-rats
#1
Denise Van der Graaff, Wilhelmus J Kwanten, Filip J Couturier, Jesse S Govaerts, Wim Verlinden, Isabel Brosius, Michiel D'Hondt, Ann Driessen, Benedicte Y De Winter, Joris G De Man, Peter P Michielsen, Sven M Francque
Non-alcoholic fatty liver disease (NAFLD) has become the most prevalent chronic liver disease. The presence of portal hypertension has been demonstrated in NAFLD prior to development of inflammation or fibrosis, and is a result of extrahepatic and intrahepatic factors, principally driven by vascular dysfunction. An increased intrahepatic vascular resistance potentially contributes to progression of NAFLD via intralobular hypoxia. However, the exact mechanisms underlying vascular dysfunction in NAFLD remain unknown...
January 11, 2018: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/29315431/receptor-conversion-in-distant-breast-cancer-metastases-a-systematic-review-and-meta-analysis
#2
Willemijne A M E Schrijver, Karijn P M Suijkerbuijk, Carla H van Gils, Elsken van der Wall, Cathy B Moelans, Paul J van Diest
Background: In metastatic breast cancer, hormone and/or human epidermal growth factor receptor 2 (HER2)-targeted therapy decision-making is still largely based on tissue characteristics of the primary tumor. However, a change of estrogen receptor alpha (ERα), progesterone receptor (PR), and HER2 status in distant metastases has frequently been reported. The actual incidence of this phenomenon has been debated. Methods: We performed a meta-analysis including 39 studies assessing receptor conversion from primary breast tumors to paired distant breast cancer metastases...
January 5, 2018: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/29312973/critical-appraisal-of-chinese-2017-guideline-on-the-management-of-hepatocellular-carcinoma
#3
REVIEW
Di-Yang Xie, Zheng-Gang Ren, Jian Zhou, Jia Fan, Qiang Gao
Hepatocellular carcinoma (HCC) is the fourth most common and the third most lethal cancer in China. An updated version of consensus-based recommendations on the management of HCC has been recently published by a multidisciplinary group of Chinese experts including liver surgeons, hepatic oncologists, radiologists and pathologists. Major changes have been made to the diagnostic criteria. In addition to dynamic multi-detector computed tomography (CT) and magnetic resonance imaging (MRI), gadoxetic acid-enhanced MRI and contrast-enhanced ultrasound (CEUS) are added to the diagnostic imaging tests...
December 2017: Hepatobiliary Surgery and Nutrition
https://www.readbyqxmd.com/read/29307587/cd8-t-cells-and-ifn-%C3%AE-induce-autoimmune-myelofibrosis-in-mice
#4
Yuan Yao, Liang Li, Shu-Han Yang, Cai-Yue Gao, Liang-Huan Liao, Yu-Qing Xie, Xue-Ying Yin, Yan-Qing Yang, Yun-Yun Fei, Zhe-Xiong Lian
Myelofibrosis usually occurs either as a part of a myelodysplastic syndrome or in conjunction with neoplasia. It is not commonly thought of an autoimmune disease. We reported that p40-/-IL-2Rα-/- (interleukin-12p40 and interleukin-2 receptor alpha double knockout) mice, a mouse model of human primary biliary cholangitis, exhibited features consistent with autoimmune myelofibrosis, including anemia associated with bone marrow fibrosis, and extramedullary hematopoiesis (EMH) including LSK (Lineage-c-Kit+Sca-1+) cells in spleen, liver and peripheral blood...
January 4, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29284589/serum-alpha-fetoprotein-level-per-total-tumor-volume-as-a-predictor-of-recurrence-of-hepatocellular-carcinoma-after-resection
#5
Yeshika Sharma, Michael J Weaver, Daniel R Ludwig, Kathryn Fowler, Neeta Vachharajani, William C Chapman, Jeffrey S Crippin
BACKGROUND: Alpha-fetoprotein has been used as a predictor of recurrence for hepatocellular carcinoma and disease-free survival post-resection. Studies in East Asia have shown that serum alpha-fetoprotein per total tumor volume ratio is a better prognostic indicator than alpha-fetoprotein alone. Similar studies in the United States evaluating serum alpha-fetoprotein to total tumor volume ratio have not been conducted. Its relevance is incompletely understood. METHODS: Consecutive patients undergoing resection for hepatocellular carcinoma at a single tertiary center between 2000 and 2013 were identified for inclusion in this retrospective cohort study...
December 25, 2017: Surgery
https://www.readbyqxmd.com/read/29259521/nuclease-mediated-gene-therapies-for-inherited-metabolic-diseases-of-the-liver%C3%A2-%C3%A2
#6
REVIEW
Taylor E Bryson, Caitlin M Anglin, P Hudson Bridges, Renee N Cottle
Inherited metabolic diseases (IMDs) of the liver represent a vast and diverse group of rare genetic diseases characterized by the loss or dysfunction of enzymes or proteins essential for metabolic pathways in the liver. Conventional gene therapy involving adeno-associated virus (AAV) serotype 8 vectors provide therapeutically high levels of hepatic transgene expression facilitating the correction of the disease phenotype in pre-clinical studies and are currently being evaluated in clinical trials for multiple IMDs...
December 2017: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/29249928/the-antioxidant-cofactor-alpha-lipoic-acid-may-control-endogenous-formaldehyde-metabolism-in-mammals
#7
Anastasia V Shindyapina, Tatiana V Komarova, Ekaterina V Sheshukova, Natalia M Ershova, Vadim N Tashlitsky, Alexander V Kurkin, Ildar R Yusupov, Garik V Mkrtchyan, Murat Y Shagidulin, Yuri L Dorokhov
The healthy human body contains small amounts of metabolic formaldehyde (FA) that mainly results from methanol oxidation by pectin methylesterase, which is active in a vegetable diet and in the gastrointestinal microbiome. With age, the ability to maintain a low level of FA decreases, which increases the risk of Alzheimer's disease and dementia. It has been shown that 1,2-dithiolane-3-pentanoic acid or alpha lipoic acid (ALA), a naturally occurring dithiol and antioxidant cofactor of mitochondrial α-ketoacid dehydrogenases, increases glutathione (GSH) content and FA metabolism by mitochondrial aldehyde dehydrogenase 2 (ALDH2) thus manifests a therapeutic potential beyond its antioxidant property...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29248802/eef1a1-overexpression-enhances-tumor-progression-and-indicates-poor-prognosis-in-hepatocellular-carcinoma
#8
Shi-Lu Chen, Shi-Xun Lu, Li-Li Liu, Chun-Hua Wang, Xia Yang, Zhi-Yi Zhang, Hui-Zhong Zhang, Jing-Ping Yun
Liver is a major contributor of protein production physiologically. The aberrant state of protein synthesis leads to tumor progression. Eukaryotic elongation factor 1 alpha 1 (eEF1A1) is a major member of the eukaryotic elongation factor family that regulates protein synthesis. Although eEF1A1 plays an essential role in controlling the cell fate, its clinical significance in tumor development and progression has not been reported. Here, we aimed to uncover the expression and prognostic significance of eEF1A1 in hepatocellular carcinoma (HCC)...
December 14, 2017: Translational Oncology
https://www.readbyqxmd.com/read/29232161/characterization-of-novel-missense-variants-of-serpina1-gene-causing-alpha-1-antitrypsin-deficiency
#9
Nerea Matamala, Beatriz Lara, Gema Gomez-Mariano, Selene Martínez, Diana Retana, Taiomara Fernandez, Ramona Angeles Silvestre, Irene Belmonte, Francisco Rodriguez-Frias, Marçal Vilar, Raquel Sáez, Igor Iturbe, Silvia Castillo, María Molina-Molina, Anna Texido, Gema Tirado-Conde, Jose Luis Lopez-Campos, Manuel Posada, Ignacio Blanco, Sabina Janciauskiene, Beatriz Martinez-Delgado
SERPINA1 gene is highly polymorphic, with more than one hundred variants described in databases. The SERPINA1 encodes alpha-1 antitrypsin (AAT) protein, and the severe deficiency of AAT is a major contributor to pulmonary emphysema and liver diseases. We report seven new variants in Spanish patients with AAT deficiency. All variants involved amino acid substitutions in different exons: PiSDonosti (S+Ser14Phe), PiTijarafe (Ile50Asn), PiSevilla (Ala58Asp), PiCadiz (Glu151Lys), PiTarragona (Phe227Cys), PiPuerto Real (Thr249Ala) and PiValencia (Lys328Glu)...
December 12, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29229313/emerging-role-of-adipocytokines-in-type-2-diabetes-as-mediators-of-insulin-resistance-and-cardiovascular-disease
#10
REVIEW
Ravindran Jaganathan, Rajeswari Ravindran, Sugapriya Dhanasekaran
Adipose tissue is an enormously active endocrine organ, secreting various hormones, such as adiponectin, leptin, resistin and visfatin, together with classical cytokines, such as tumor necrosis factor-alpha (TNF-α) and interleukin-6 (IL-6). All these adipocytokines plays significant roles in the regulation of energy metabolism, glucose and lipid metabolism, reproduction, cardiovascular function and immunity. Adipocytokines are significantly regulated by nutritional status and can directly influence other organ systems, including brain, liver and skeletal muscle...
December 8, 2017: Canadian Journal of Diabetes
https://www.readbyqxmd.com/read/29227477/the-deubiquitinating-enzyme-tnfaip3-mediates-inactivation-of-hepatic-ask1-and-ameliorates-nonalcoholic-steatohepatitis
#11
Peng Zhang, Pi-Xiao Wang, Ling-Ping Zhao, Xin Zhang, Yan-Xiao Ji, Xiao-Jing Zhang, Chun Fang, Yue-Xin Lu, Xia Yang, Mao-Mao Gao, Yan Zhang, Song Tian, Xue-Yong Zhu, Jun Gong, Xin-Liang Ma, Feng Li, Zhihua Wang, Zan Huang, Zhi-Gang She, Hongliang Li
Activation of apoptosis signal-regulating kinase 1 (ASK1) in hepatocytes is a key process in the progression of nonalcoholic steatohepatitis (NASH) and a promising target for treatment of the condition. However, the mechanism underlying ASK1 activation is still unclear, and thus the endogenous regulators of this kinase remain open to be exploited as potential therapeutic targets. In screening for proteins that interact with ASK1 in the context of NASH, we identified the deubiquitinase tumor necrosis factor alpha-induced protein 3 (TNFAIP3) as a key endogenous suppressor of ASK1 activation, and we found that TNFAIP3 directly interacts with and deubiquitinates ASK1 in hepatocytes...
December 11, 2017: Nature Medicine
https://www.readbyqxmd.com/read/29225784/diagnosing-alpha-1-antitrypsin-deficiency-the-first-step-in-precision-medicine
#12
REVIEW
Craig P Hersh
Severe alpha-1 antitrypsin (AAT) deficiency is one of the most common serious genetic diseases in adults of European descent. Individuals with AAT deficiency have a greatly increased risk for emphysema and liver disease. Other manifestations include bronchiectasis, necrotizing panniculitis and granulomatosis with polyangiitis. Despite the frequency and potential severity, AAT deficiency remains under-recognized, and there is often a delay in diagnosis. This review will focus on three recent updates that should serve to encourage testing and diagnosis of AAT deficiency: first, the publication of a randomized clinical trial demonstrating the efficacy of intravenous augmentation therapy in slowing the progression of emphysema in AAT deficiency; second, the mounting evidence showing an increased risk of lung disease in heterozygous PI MZ genotype carriers; last, the recent publication of a clinical practice guideline, outlining diagnosis and management...
2017: F1000Research
https://www.readbyqxmd.com/read/29207969/pre-treatment-alphafeto-protein-in-hepatocellular-carcinoma-with-non-viral-aetiology-a-prospective-study
#13
Siriwardana Rohan Chaminda, Thilakarathne Suchintha, Niriella Madunil Anuk, Dassanayake Anuradha Supun, Gunathilake Mahen Bhagya, Liyanage Chandika Anuruddha Habarakada, De Silva Hithadurage Janaka
BACKGROUND: Alpha-fetoprotein (AFP) is a biomarker for hepatocellular carcinoma (HCC). The significance of pre-treatment AFP (pt-AFP) in non-viral HCC (nvHCC) is not clear. METHODS: Patients with nvHCC, referred to a Hepatobiliary Clinic from September 2011-2015 were screened. HCC was diagnosed using American Association for the Study of Liver Disease guidelines, and TNM staged. nvHCC was diagnosed when HBsAg and anti-HCVAb was negative. Child-Turcotte-Pugh (CTP) and Model for End-stage Liver Disease (MELD) scores were calculated...
December 6, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/29201040/caffeoylquinic-acid-rich-extract-of-aster-glehni-f-schmidt-ameliorates-nonalcoholic-fatty-liver-through-the-regulation-of-ppar%C3%AE-and-adiponectin-in-apoe-ko-mice
#14
Yong-Jik Lee, Yoo-Na Jang, Yoon-Mi Han, Hyun-Min Kim, Jong-Min Jeong, Min Jeoung Son, Chang Bae Jin, Hyoung Ja Kim, Hong Seog Seo
Aster glehni is well known for its therapeutic properties. This study was performed to investigate the effects of A. glehni on nonalcoholic fatty liver disease (NAFLD) in atherosclerotic condition, by determining the levels of biomarkers related to lipid metabolism and inflammation in serum, liver, and adipose tissue. Body and abdominal adipose tissue weights and serum triglyceride level decreased in all groups treated with A. glehni. Serum adiponectin concentration and protein levels of peroxisome proliferator-activated receptor δ, 5' adenosine monophosphate-activated protein kinase, acetyl-CoA carboxylase, superoxide dismutase, and PPARγ coactivator 1-alpha in liver tissues increased in the groups treated with A...
2017: PPAR Research
https://www.readbyqxmd.com/read/29170515/prognostic-value-of-long-non-coding-rna-hulc-and-malat1-following-the-curative-resection-of-hepatocellular-carcinoma
#15
Fuminori Sonohara, Yoshikuni Inokawa, Masamichi Hayashi, Suguru Yamada, Hiroyuki Sugimoto, Tsutomu Fujii, Yasuhiro Kodera, Shuji Nomoto
Long non-coding RNAs (lncRNAs) were shown to be the crucial regulators of the many diseases. In this study, the expressions of lncRNAs were investigated in resected 158 hepatocellular carcinomas (HCCs) to evaluate the effects of their expression levels on prognosis. The expression levels of HULC and MALAT1 were shown to be significantly higher in the normal background tissue of HCC than those in the normal liver tissue of metastatic liver tumor without hepatitis (HULC: fold change 14.9, P = 1.7e-06; MALAT1: fold change 17...
November 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29159910/patients-with-advanced-hepatocellular-carcinoma-need-a-personalized-management-a-lesson-from-clinical-practice
#16
Edoardo G Giannini, Laura Bucci, Francesca Garuti, Matteo Brunacci, Barbara Lenzi, Matteo Valente, Eugenio Caturelli, Giuseppe Cabibbo, Fabio Piscaglia, Roberto Virdone, Martina Felder, Francesca Ciccarese, Francesco Giuseppe Foschi, Rodolfo Sacco, Gianluca Svegliati Baroni, Fabio Farinati, Gian Lodovico Rapaccini, Andrea Olivani, Antonio Gasbarrini, Maria Di Marco, Filomena Morisco, Marco Zoli, Alberto Masotto, Franco Borzio, Luisa Benvegnù, Fabio Marra, Antonio Colecchia, Gerardo Nardone, Mauro Bernardi, Franco Trevisani
The Barcelona Clinic Liver Cancer advanced stage (BCLC C) of hepatocellular carcinoma (HCC) includes a heterogeneous population, where sorafenib alone is the recommended treatment. In this study our aim was to assess treatment and overall survival (OS) of BCLC C patients sub-classified according to clinical features (Performance Status [PS], macro-vascular invasion [MVI], extra-hepatic spread [EHS] or MVI+EHS) determining their allocation to this stage. From the Italian Liver Cancer database, we analysed 835 consecutive BCLC C patients diagnosed between 2008 and 2014...
November 21, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29142479/medication-beliefs-predict-medication-adherence-in-ambulatory-patients-with-decompensated-cirrhosis
#17
Kelly L Hayward, Patricia C Valery, Jennifer H Martin, Antara Karmakar, Preya J Patel, Leigh U Horsfall, Caroline J Tallis, Katherine A Stuart, Penny L Wright, David D Smith, Katharine M Irvine, Elizabeth E Powell, W Neil Cottrell
AIM: To investigate the impact of medication beliefs, illness perceptions and quality of life on medication adherence in people with decompensated cirrhosis. METHODS: One hundred adults with decompensated cirrhosis completed a structured questionnaire when they attended for routine outpatient hepatology review. Measures of self-reported medication adherence (Morisky Medication Adherence Scale), beliefs surrounding medications (Beliefs about Medicines Questionnaire), perceptions of illness and medicines (Brief Illness Perception Questionnaire), and quality of life (Chronic Liver Disease Questionnaire) were examined...
October 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29137099/chemoembolization-for-hepatocellular-carcinoma-fed-by-right-internal-thoracic-artery
#18
Tao Shen, Zhongzhi Jia, Yuanquan Huang, Shaoqin Li, Guomin Jiang, Lihua Cheng
The purpose of the study was to evaluate the value of transarterial chemoembolization (TACE) via right internal thoracic artery (RITA) for patients with unresectable hepatocellular carcinoma (HCC).From January 2000 to June 2016, a retrospective study was conducted of all patients with unresectable HCC who underwent TACE via RITA across 3 medical centers. The technical success, serum alpha-fetoprotein (AFP) level changes, major complications, disease control rate, and survival were evaluated and analyzed.During the study peroid, in all, 21 patients (men 21; mean age 57...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29134552/intranasal-delivery-of-%C3%AE-asarone-to-the-brain-with-lactoferrin-modified-mpeg-pla-nanoparticles-prepared-by-premix-membrane-emulsification
#19
Linmei Pan, Jing Zhou, Feng Ju, Huaxu Zhu
Alpha-asarone is a bioactive component of Acorus tatarincuii Schott with low bioavailability, which is often used for treatments of various brain diseases in clinical setting. This study was to formulate biodegradable methoxy polyethylene glycol-polylactic acid (mPEG-PLA) nanoparticles (NPs) surface-modified by lactoferrin (Lf), for delivering α-asarone into the brain following intranasal administration. Alpha-asarone NPs were prepared by premix membrane emulsification. The relative parameters were optimized by a Box-Behnken experimental design...
November 13, 2017: Drug Delivery and Translational Research
https://www.readbyqxmd.com/read/29131698/the-anti-inflammatory-and-antioxidant-effects-of-salvia-officinalis-on-lipopolysaccharide-induced-inflammation-in-rats
#20
Umut Kerem Kolac, Mehmet Cengiz Ustuner, Neslihan Tekin, Derya Ustuner, Emine Colak, Emre Entok
Salvia officinalis, which has a high phenolic acid and flavonoid content, is a powerful antioxidant and anti-inflammatory herb. Inflammation plays an important role in the pathophysiology of many diseases and could cause damage by means of oxidative stress. The aim of this study was to investigate the anti-inflammatory and antioxidant activity of S. officinalis formed lipopolysaccharide (LPS)-induced experimental inflammation model. Four- to five-month-old 42 female Wistar albino rats were divided into six groups...
November 13, 2017: Journal of Medicinal Food
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