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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/28922440/real-life-use-of-3-direct-acting-antiviral-regimen-in-a-large-cohort-of-patients-with-genotype-1b-hcv-compensated-cirrhosis
#2
Liana Gheorghe, Speranta Iacob, Manuela Curescu, Ciprian Brisc, Cristina Cijevschi, Florin Caruntu, Carol Stanciu, Iulia Simionov, Ioan Sporea, Cristian Gheorghe, Razvan Iacob, Victoria Arama, Roxana Sirli, Anca Trifan
BACKGROUND AND AIMS: Ombitasvir/Paritaprevir/ritonavir/Dasabuvir (OBV/PTV/r+DSV) is one of the elective direct-acting antivirals (DAAs) recommended by international guidelines and the only one covered by the National Insurance System in Romania until November 2016. Our aim was to present the first prospective Romanian cohort evaluating the effectiveness and safety in clinical practice of this 3DAA combination in patients with HCV genotype-1b Child A liver cirrhosis. METHODS: 681 patients received OBV/PTV/r+DSV+RBV for 12 weeks and were assessed clinically and biologically at baseline, week 4, 8, 12 (end of treatment, EOT), and 12 weeks after therapy (sustained viral response, SVR)...
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
#3
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/28922227/incidence-and-risk-factors-of-coagulation-profile-derangement-after-liver-surgery-implications-for-the-use-of-epidural-analgesia-a-retrospective-cohort-study
#4
Pierre Jacquenod, Grégoire Wallon, Mathieu Gazon, Benjamin Darnis, Pierre Pradat, Victor Virlogeux, Olivier Farges, Frédéric Aubrun
BACKGROUND: Hepatic surgery is a major abdominal surgery. Epidural analgesia may decrease the incidence of postoperative morbidities. Hemostatic disorders frequently occur after hepatic resection. Insertion or withdrawal (whether accidental or not) of an epidural catheter during coagulopathic state may cause an epidural hematoma. The aim of the study is to determine the incidence of coagulopathy after hepatectomy, interfering with epidural catheter removal, and to identify the risk factors related to coagulopathy...
September 14, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28922103/budd-chiari-syndrome
#5
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/28921807/the-italian-compassionate-use-of-sofosbuvir-in-hcv-patients-waitlisted-for-liver-transplantation-a-national-real-life-experience
#6
Silvia Martini, Maria Francesca Donato, Chiara Mazzarelli, Maria Rendina, Ubaldo Visco-Comandini, Daniela Filì, Alice Gianstefani, Stefano Fagiuoli, Mario Melazzini, Simona Montilla, Luca Pani, Sandra Petraglia, Pierluigi Russo, Maria Paola Trotta, Paola Carrai, Paolo Caraceni
BACKGROUND & AIMS: This study aimed to assess the real-life clinical and virological outcomes of HCV waitlisted patients for liver transplantation (LT) who received sofosbuvir/ribavirin (SOF/R) within the Italian compassionate use program. METHODS: Clinical and virological data were collected in 224 patients with decompensated cirrhosis and/or hepatocellular carcinoma (HCC) receiving daily SOF/R until LT or up a maximum of 48 weeks. RESULTS: Of 100 transplanted patients, 51 were HCV-RNA negative for >4 weeks before LT (SVR12: 88%) and 49 negative for <4 weeks or still viremic at transplant: 34 patients continued treatment after LT (bridging therapy) (SVR12: 88%), while 15 stopped treatment (SVR12: 53%)...
September 16, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28921803/the-pathophysiology-of-arterial-vasodilatation-and-hyperdynamic-circulation-in-cirrhosis
#7
Søren Møller, Flemming Bendtsen
Patients with cirrhosis and portal hypertension often develop complications from a variety of organ systems leading to a multiple organ failure. The combination of liver failure and portal hypertension result in a hyperdynamic circulatory state partly owing to simultaneous splanchnic and peripheral arterial vasodilatation. Increases in circulatory vasodilators are believed to be due to portosystemic shunting and bacterial translocation leading to redistribution of the blood volume with central hypovolemia. Portal hypertension per se and increased splanchnic blood flow are mainly responsible for the development and perpetuation of the hyperdynamic circulation and the associated changes in cardiovascular function with development of cirrhotic cardiomyopathy, autonomic dysfunction, and renal dysfunction as part of a cardiorenal syndrome...
September 16, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28921677/clinical-profile-of-hepatitis-c-virus-infection-in-a-developing-country-india
#8
Ramit Mahajan, Vandana Midha, Omesh Goyal, Varun Mehta, Vikram Narang, Kirandeep Kaur, Arshdeep Singh, Dharmatma Singh, Rishu Bhanot, Ajit Sood
BACKGROUND: The epidemiology and clinical profile of hepatitis C virus (HCV) varies worldwide and data from developing countries is sparse. AIMS: To study the clinical profile of HCV infection in a developing country in South East Asia (India). METHODS: This observational study assessed patient demographics, viral characteristics, risk factors for virus acquisition and disease characteristics in HCV patients diagnosed between January 2004 and December 2015...
September 18, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28919595/a-case-of-hepatorenal-syndrome-and-abdominal-compartment-syndrome-with-high-renal-congestion
#9
Hiroteru Kamimura, Takayuki Watanabe, Tomoyuki Sugano, Nao Nakajima, Junji Yokoyama, Kenya Kamimura, Atsunori Tsuchiya, Masaaki Takamura, Hirokazu Kawai, Takashi Kato, Gen Watanabe, Satoshi Yamagiwa, Shuji Terai
BACKGROUND Hepatorenal syndrome (HRS) is a reversible renal impairment that occurs in patients with acute liver failure and advanced liver cirrhosis. HRS is due to a renal vasoconstriction that results from extreme vasodilatation. It is therefore a functional disorder, not associated with structural kidney damage. On the other hand, end-stage liver diseases are often complicated by massive ascites. Massive ascites may cause abdominal compartment syndrome (ACS), which includes impairment of renal blood flow, but there are no reports indicating that kidney lesions caused by ACS may pathologically contribute to end-stage liver diseases...
September 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28919365/hepatic-stellate-cell-specific-deletion-of-sirt1-exacerbates-liver-fibrosis-in-mice
#10
Min Li, Wenxuan Hong, Chenzhi Hao, Luyang Li, Huihui Xu, Ping Li, Yong Xu
Liver fibrosis is widely perceived as a host defense mechanism that aids tissue repair following liver injury. Excessive fibrogenesis, however, serves to disrupts normal liver structure and precedes such irrevocable human pathologies as cirrhosis and hepatocellular carcinoma. Activation of hepatic stellate cells (HSCs) is a hallmark event during liver fibrosis. In the present study we investigated the mechanism by which the lysine deacetylase SIRT1 regulates HSC activation. We report here that SIRT1 levels were decreased in the liver in different mouse models and in cultured HSCs undergoing activation...
September 14, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#11
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/28919164/integrated-analysis-of-microrna-and-gene-expression-profiles-reveals-a-functional-regulatory-module-associated-with-liver-fibrosis
#12
Wei Chen, Wenshan Zhao, Aiting Yang, Anjian Xu, Huan Wang, Min Cong, Tianhui Liu, Ping Wang, Hong You
BACKGROUND: Liver fibrosis, characterized with the excessive accumulation of extracellular matrix (ECM) proteins, represents the final common pathway of chronic liver inflammation. Ever-increasing evidence indicates microRNAs (miRNAs) dysregulation have important implications in the different stages of liver fibrosis. However, our knowledge of miRNA-gene regulation details pertaining to such disease remains unclear. METHODS: The publicly available Gene Expression Omnibus (GEO) datasets of patients suffered from cirrhosis were extracted for integrated analysis...
September 14, 2017: Gene
https://www.readbyqxmd.com/read/28918833/recurrence-of-barcelona-clinic-liver-cancer-stage-a-hepatocellular-carcinoma-after-hepatectomy
#13
Hao Zou, Cheng-Zhan Zhu, Chang Wang, Zu-Sen Wang, Xiang Ma, Bing Han, Li-Qun Wu
BACKGROUND: The Barcelona Clinic Liver Cancer (BCLC) staging system is widely used to classify hepatocellular carcinoma (HCC). This study was performed to investigate the prognostic factors for patients with BCLC stage A HCC after R0 hepatectomy. METHODS: A total of 592 patients with BCLC stage A HCC following R0 hepatectomy from 1997-2012 were enrolled in this study. Kaplan-Meier analysis and Cox regression were used to analyze the risk factors associated with recurrence...
September 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28918795/-acute-on-chronic-liver-failure
#14
Nikolaj Worm Ørntoft, Karen Louise Thomsen, Gitte Dam, Niels Kristian Aagaard, Henning Grønbæk
Acute-on-chronic liver failure (ACLF) is a clinical syndrome characterized by acute decompensation of liver cirrhosis, organ failure and high short-term mortality (20-80% at one month). The main precipitants are infections and excessive alcohol intake, and the mechanistic features include a high level of systemic inflammation, macrophage activation and liver injury. The severity of ACLF is graded according to the number and extent of organ failures. Prognostic scores help predict mortality and support decisions on intensive treatment or futility...
September 18, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28918403/successful-twice-interrupted-therapy-of-hcv-infection-in-patients-with-cirrhosis-with-hepatocellular-carcinoma-before-and-after-liver-transplantation
#15
Anna Szymanek-Pasternak, Karolina Rostkowska, Krzysztof Simon
We are presenting the case study of the patient diagnosed at the age of 37 with liver cirrhosis due to genotype 1b hepatitis C virus infection. At the age of 46, he was diagnosed with hepatocellular carcinoma with subsequent resection of the tumour in May 2015. In December 2015, the treatment was started with ombitasvir, paritaprevir/ritonavir and dasabuvir (3D) with ribavirin (RBV) 1000 mg per day. After 24 days of this treatment, the patient received a deceased donor liver transplantation, followed by 18-day interruption of 3D therapy...
September 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28918389/glucagon-like-peptide-1-receptor-agonists-for-the-management-of-obesity-and-non-alcoholic-fatty-liver-disease-a-novel-therapeutic-option
#16
REVIEW
Gauri Dhir, Kenneth Cusi
Obesity is a major risk factor for the development of type 2 diabetes mellitus (T2DM), and is associated with a cluster of metabolic factors that lead to poor cardiovascular outcomes. In non-alcoholic fatty liver disease (NAFLD), liver fat (triglyceride) accumulation closely mirrors adipose tissue dysfunction and insulin resistance in obesity and T2DM. It is now recognized as the most common chronic liver disease in Westernized societies, often progressing to more severe forms of the disease such as nonalcoholic steatohepatitis (NASH), or cirrhosis and hepatocellular carcinoma...
September 15, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28918191/development-and-validation-of-a-chronic-pancreatitis-prognosis-score-in-2-independent-cohorts
#17
Georg Beyer, Ujjwal M Mahajan, Christoph Budde, Thomas J Bulla, Thomas Kohlmann, Louise Kuhlmann, Kerstin Schütte, Ali A Aghdassi, Eckhard Weber, Ulrich Weiss, Asbjørn M Drewes, Søren S Olesen, Markus M Lerch, Julia Mayerle
BACKGROUND & AIMS: The clinical course of chronic pancreatitis is unpredictable. There is no model to assess disease severity or progression or predict patient outcomes. METHODS: We performed a prospective study of 91 patients with chronic pancreatitis; data were collected from patients seen at academic centers in Europe, from January 2011 through April 2014. We analyzed correlations between clinical, laboratory, and imaging data with number of hospital readmissions and in-hospital days over the next 12 months; the parameters with the highest degree of correlation were to develop 3-stage chronic pancreatitis prognosis score (COPPS)...
September 14, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28918131/cirrhotic-patients-with-portal-hypertension-related-bleeding-and-an-indication-for-early-tips-a-large-multicentre-audit-with-real-life-results
#18
Dominique Thabut, Arnaud Pauwels, Nicolas Carbonell, Andre Jean Remy, Pierre Nahon, Xavier Causse, Jean-Paul Cervoni, Jean-François Cadranel, Isabelle Archambeaud, Slim Bramli, Florent Ehrhard, Philippe Ah-Soune, Florian Rostain, Alexandre Pariente, Julien Vergniol, Jean-Pierre Dupuychaffray, Anne-Laure Pelletier, Florence Skinazi, Anne Guillygomarc'h, René-Louis Vitte, Jean Henrion, Stéphanie Combet, Marika Rudler, Christophe Bureau
BACKGROUND: The Baveno VI consensus meeting concluded that an early TIPS must be considered in high-risk cirrhotic patients presenting with variceal bleeding (VB) (Child B + active bleeding at endoscopy or Child C10-13 patients). Whether this therapeutic approach is feasible in a real-life setting remains unclear. AIMS: To determine (1) the proportion of patients eligible for early-TIPS among cirrhotic patients with VB, (2) the proportion of these patients who underwent early-TIPS placement and the main reasons for discarding TIPS, and (3) the outcomes of patients who experienced early-TIPS placement in a large, national, prospective, multicentre audit including academic and non-academic centres...
September 13, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28918026/anti-fibrotic-effects-of-synthetic-oligodeoxynucleotide-for-tgf-%C3%AE-1-and-smad-in-an-animal-model-of-liver-cirrhosis
#19
Jung-Yeon Kim, Hyun-Jin An, Woon-Hae Kim, Mi-Gyeong Gwon, Hyemin Gu, Yoon-Yub Park, Kwan-Kyu Park
Liver fibrosis is characterized by changes in tissue architecture and extracellular matrix composition. Liver fibrosis affects not only hepatocytes but also the non-parenchymal cells such as hepatic stellate cells (HSCs), which are essential for maintaining an intact liver structure and function. Transforming growth factor β1 (TGF-β1) is a multifunctional cytokine that induces liver fibrosis through activation of Smad signaling pathways. To improve a new therapeutic approach, synthetic TGF-β1/Smad oligodeoxynucleotide (ODN) was used to suppress both TGF-β1 expression and Smad transcription factor using a combination of antisense ODN and decoy ODN...
September 15, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28916899/impact-of-surgical-complications-on-the-risk-of-hepatocellular-carcinoma-recurrence-after-hepatic-resection
#20
Riccardo Pravisani, Umberto Baccarani, Miriam Isola, Gianluigi Adani, Dario Lorenzin, Giovanni Terrosu, Andrea Risaliti
Surgery-related morbidity has been identified as prognostic risk factor for tumor recurrence for several tumor types, but data regarding hepatocellular carcinoma (HCC) are limited and controversial. The aim of this study was to analyze the impact of surgical complications on the risk of HCC recurrence after hepatic resection (HR). A Retrospective study was conducted on a cohort of patients submitted to HR in a tertiary teaching hospital, between January 2006 and December 2015. 112 patients were submitted to HR during the study period...
September 15, 2017: Updates in Surgery
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