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https://www.readbyqxmd.com/read/28922444/cholangiocarcinoma-in-wilson-s-disease-a-case-report
#1
Dániel Németh, Anikó Folhoffer, Gábor Smuk, Béla Kajtár, Tamás Tornóczky, Ferenc Szalay
It has been suggested that hepatobiliary carcinomas are less frequent in Wilson's disease (WD) than in liver diseases of other etiology. However, the protective role of copper against malignancies is debated. Only a few cases of cholangiocarcinoma (CCC) in WD have been published. Here we report on a case of a 47-year-old male H1069Q homozygous, Kayser-Fleischer ring positive WD patient with a low ceruloplasmin level who was followed up and treated with chelating agents throughout nine years. The patient presented with neurological symptoms and liver cirrhosis at diagnosis...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28922436/genetic-risk-factors-for-autoimmune-thyroid-disease-might-affect-the-susceptibility-to-and-modulate-the-progression-of-primary-biliary-cholangitis
#2
Aleksander Kuś, Magdalena Arłukowicz-Grabowska, Konrad Szymański, Ewa Wunsch, Małgorzata Milkiewicz, Rafał Płoski, Zakera Shums, Gary L Norman, Piotr Milkiewicz, Tomasz Bednarczuk, Marcin Krawczyk
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD). METHODS: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28922103/budd-chiari-syndrome
#3
Tomáš Grus, Lukáš Lambert, Gabriela Grusová, Rohan Banerjee, Andrea Burgetová
Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28919805/a-pilot-evaluation-of-a-computer-based-psychometric-test-battery-designed-to-detect-impairment-in-patients-with-cirrhosis
#4
Nicola A Cook, Jin Un Kim, Yasmin Pasha, Mary Me Crossey, Adrian J Schembri, Brian T Harel, Torben Kimhofer, Simon D Taylor-Robinson
BACKGROUND: Psychometric testing is used to identify patients with cirrhosis who have developed hepatic encephalopathy (HE). Most batteries consist of a series of paper-and-pencil tests, which are cumbersome for most clinicians. A modern, easy-to-use, computer-based battery would be a helpful clinical tool, given that in its minimal form, HE has an impact on both patients' quality of life and the ability to drive and operate machinery (with societal consequences). AIM: We compared the Cogstate™ computer battery testing with the Psychometric Hepatic Encephalopathy Score (PHES) tests, with a view to simplify the diagnosis...
2017: International Journal of General Medicine
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#5
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28914955/alcohol-related-mortality-in-patients-with-psoriasis-a-population-based-cohort-study
#6
Rosa Parisi, Roger T Webb, Matthew J Carr, Kieran J Moriarty, C Elise Kleyn, Christopher E M Griffiths, Darren M Ashcroft
Importance: People diagnosed with psoriasis have an increased risk of premature mortality, but the underlying reasons for this mortality gap are unclear. Objective: To investigate whether patients with psoriasis have an elevated risk of alcohol-related mortality. Design, Setting, and Participants: An incident cohort of patients with psoriasis aged 18 years and older was delineated for 1998 through 2014 using the Clinical Practice Research Datalink (CPRD) and linked to Hospital Episode Statistics (HES) and Office for National Statistics (ONS) mortality records...
September 15, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#7
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913620/a-brief-review-on-prognostic-models-of-primary-biliary-cholangitis
#8
REVIEW
Sha Chen, Weijia Duan, Hong You, Jidong Jia
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016...
September 14, 2017: Hepatology International
https://www.readbyqxmd.com/read/28905444/response-to-hepatitis-b-vaccination-in-patients-with-liver-cirrhosis
#9
REVIEW
Ioanna Aggeletopoulou, Panagiota Davoulou, Christos Konstantakis, Konstantinos Thomopoulos, Christos Triantos
Hepatitis B vaccination is strongly recommended for all infants and children but also for adults who are at risk of HBV infection. Attempts to immunize patients with liver cirrhosis have been proven relatively ineffective, and several strategies have already been used to improve the immune response in this group. The primary aim of this review is to examine, discuss, and summarize the immunogenicity of hepatitis B vaccination in patients with liver cirrhosis. MEDLINE search identified 11 studies (n = 961)...
September 14, 2017: Reviews in Medical Virology
https://www.readbyqxmd.com/read/28903508/effect-of-paritaprevir-ritonavir-ombitasvir-dasabuvir-and-ledipasvir-sofosbuvir-regimens-on-survival-compared-with-untreated-hepatitis-c-virus-infected-persons-results-from-erchives
#10
Adeel Ajwad Butt, Peng Yan, Tracey G Simon, Abdul-Badi Abou-Samra
Background: Interferon-based regimens are associated with a substantial survival benefit for persons infected with hepatitis C virus (HCV). Survival data with direct-acting antiviral agents are not available. We conducted this study to quantify the effect of paritaprevir/ritonavir, ombitasvir, dasabuvir (PrOD) and ledipasvir/sofosbuvir (LDV/SOF) regimens upon mortality. Methods: In the Electronically Retrieved Cohort of HCV Infected Veterans (ERCHIVES), a well-established national cohort of HCV-infected Veterans, we identified HCV-infected persons initiated on PrOD or LDV/SOF, excluding those with human immunodeficiency virus, hepatitis B surface antigen positivity, hepatocellular carcinoma, or missing HCV RNA or FIB-4 scores...
September 15, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28902040/long-term-rifaximin-therapy-as-a-primary-prevention-of-hepatorenal-syndrome
#11
El-Sayed Ibrahim, Ayman Alsebaey, Hassan Zaghla, Sabry Moawad Abdelmageed, Khalid Gameel, Eman Abdelsameea
BACKGROUND: Hepatorenal syndrome (HRS) is a severe complication of liver cirrhosis, with poor survival. Rifaximin is a gut-selective broad-spectrum antibiotic. AIM: The aim of this study was to evaluate the role of rifaximin as a primary prevention of HRS. PATIENTS AND METHODS: Eighty patients with liver cirrhosis and ascites were enrolled. They were randomized into two groups: control (n=40) and rifaximin group (n=40). Baseline liver function tests, renal function tests, complete blood count, international normalized ratio, urine analysis, and abdominal ultrasonography were carried out...
September 8, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28894136/soluble-intercellular-adhesion-molecule-1-is-associated-with-hepatocellular-carcinoma-risk-multiplex-analysis-of-serum-markers
#12
Vincent L Chen, An K Le, Ondrej Podlaha, Jacqueline Estevez, Biao Li, Philip Vutien, Ellen T Chang, Yael Rosenberg-Hasson, Stefan Pflanz, Zhaoshi Jiang, Dongliang Ge, Anuj Gaggar, Mindie H Nguyen
Individualized assessment of hepatocellular carcinoma (HCC) risk in chronic liver disease remains challenging. Serum biomarkers including cytokines may offer helpful adjuncts to standard parameters for risk prediction. Our aim was to identify markers associated with increased HCC incidence. This was a prospective cohort study of 282 patients with both viral or non-viral chronic liver disease. Baseline serum cytokines and other markers were measured in multiplex with a commercially-available Luminex-based system...
September 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28893941/viral-hepatitis-and-liver-cancer
#13
REVIEW
Marc Ringehan, Jane A McKeating, Ulrike Protzer
Hepatitis B and C viruses are a global health problem causing acute and chronic infections that can lead to liver cirrhosis and hepatocellular carcinoma (HCC). These infections are the leading cause for HCC worldwide and are associated with significant mortality, accounting for more than 1.3 million deaths per year. Owing to its high incidence and resistance to treatment, liver cancer is the second leading cause of cancer-related death worldwide, with HCC representing approximately 90% of all primary liver cancer cases...
October 19, 2017: Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#14
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28893365/coffee-consumption-and-prevention-of-cirrhosis-in-support-of-the-caffeine-hypothesis
#15
Jonathan Dranoff
It is widely acknowledged that coffee is the most widely used drug on Earth. While this is true, coffee is also a foodstuff with wide variation, so its use is often directed at satisfying dietary urges. When used as a drug, coffee is almost invariably used as a stimulant rather than to treat or prevent particular diseases. Thus, it has been an exciting revelation in recent years that coffee consumption is inversely related to progression of liver fibrosis to cirrhosis and even hepatocellular carcinoma.As is often the case, epidemiological observations have preceded pathophysiological explanations for this effect...
September 11, 2017: Gene Expression
https://www.readbyqxmd.com/read/28892963/autoimmune-hepatitis-primary-biliary-cirrhosis-overlap-syndrome
#16
Indira Bairy, Anupam Berwal, Shubha Seshadri
Autoimmune Hepatitis (AIH) and Primary Biliary Cirrhosis (PBC) are important immune mediated liver diseases. They are usually differentiated based on clinical, biochemical, serological and histological parameters. The presence of autoantibodies, clinical and serological findings can sometimes occur in different combinations leading to overlap syndromes, which is rare. Early recognition of such overlap syndromes is clinically significant from treatment point of view. Here, we report a case of AIH-PBC overlap syndrome with a brief review of literature on overlap syndromes...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28882625/drug-induced-bile-duct-injury
#17
REVIEW
Michele Visentin, Daniela Lenggenhager, Zhibo Gai, Gerd A Kullak-Ublick
Drug-induced liver injury includes a spectrum of pathologies, some related to the mode of injury, some to the cell type primarily damaged. Among these, drug-induced bile duct injury is characterized by the destruction of the biliary epithelium following exposure to a drug. Most of the drugs associated with bile duct injury cause immune-mediated lesions to the epithelium of interlobular ducts. These share common histopathological features with primary biliary cholangitis, such as inflammation and necrosis at the expense of cholangiocytes and, if the insult persists, bile duct loss and biliary cirrhosis...
September 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28880053/liver-fibrosis-the-2017-state-of-art
#18
Gian P Caviglia, Chiara Rosso, Sharmila Fagoonee, Giorgio M Saracco, Rinaldo Pellicano
Liver fibrosis is a wound-healing response to a wide spectrum of chronic liver injuries. It is characterized by loss of hepatocytes and alteration in hepatic architecture following an imbalance between extracellular matrix synthesis and degradation. Irrespectively of underlying etiology, fibrosis may progress to cirrhosis and specific pathogenetic mechanisms as well as different disease patterns may be identified according to etiology. Liver biopsy is still considered the gold standard for fibrosis assessment, despite the fact that it is invasive, has poor patient compliance and is not exempt of complications...
September 5, 2017: Panminerva Medica
https://www.readbyqxmd.com/read/28879168/the-oncopig-cancer-model-an-innovative-large-animal-translational-oncology-platform
#19
REVIEW
Kyle M Schachtschneider, Regina M Schwind, Jordan Newson, Nickolas Kinachtchouk, Mark Rizko, Nasya Mendoza-Elias, Paul Grippo, Daniel R Principe, Alex Park, Nana H Overgaard, Gregers Jungersen, Kelly D Garcia, Ajay V Maker, Laurie A Rund, Howard Ozer, Ron C Gaba, Lawrence B Schook
Despite an improved understanding of cancer molecular biology, immune landscapes, and advancements in cytotoxic, biologic, and immunologic anti-cancer therapeutics, cancer remains a leading cause of death worldwide. More than 8.2 million deaths were attributed to cancer in 2012, and it is anticipated that cancer incidence will continue to rise, with 19.3 million cases expected by 2025. The development and investigation of new diagnostic modalities and innovative therapeutic tools is critical for reducing the global cancer burden...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28877220/attenuated-lipotoxicity-and-apoptosis-is-linked-to-exogenous-and-endogenous-augmenter-of-liver-regeneration-by-different-pathways
#20
Thomas S Weiss, Madeleine Lupke, Sara Ibrahim, Christa Buechler, Julia Lorenz, Petra Ruemmele, Ute Hofmann, Michael Melter, Rania Dayoub
Nonalcoholic fatty liver disease (NAFLD) covers a spectrum from simple steatosis to nonalcoholic steatohepatitis (NASH) and cirrhosis. Free fatty acids (FFA) induce steatosis and lipo-toxicity and correlate with severity of NAFLD. In this study we aimed to investigate the role of exogenous and endogenous ALR (augmenter of liver regeneration) for FFA induced ER (endoplasmatic reticulum) -stress and lipoapoptosis. Primary human hepatocytes or hepatoma cells either treated with recombinant human ALR (rhALR, 15kDa) or expressing short form ALR (sfALR, 15kDa) were incubated with palmitic acid (PA) and analyzed for lipo-toxicity, -apoptosis, activation of ER-stress response pathways, triacylglycerides (TAG), mRNA and protein expression of lipid metabolizing genes...
2017: PloS One
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