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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/29781738/diagnosis-of-cholangiocarcinoma-in-primary-sclerosing-cholangitis
#1
Seyed Alireza Taghavi, Ahad Eshraghian, Ramin Niknam, Gholam Reza Sivandzadeh, Kamran Bagheri Lankarani
Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the hepatobiliary system characterized by chronic inflammation, progressive fibrosis, stricture formation and destruction of extrahepatic and intrahepatic bile ducts. Areas covered: The increased incidence of cholangiocarcinoma (CCA) in PSC has been well documented and can be explained by the continuous inflammation in the biliary tree leading to an enhanced dysplasia-carcinoma sequence. Although PSC patients may progress to liver cirrhosis; CCA most commonly occurs between the ages of 30 and 45 years when cirrhosis has not yet developed...
May 21, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29774570/high-mobility-group-box-1-drives-fibrosis-progression-signaling-via-the-receptor-for-advanced-glycation-end-products-in-mice
#2
Xiaodong Ge, Elena Arriazu, Fernando Magdaleno, Daniel J Antoine, Rouchelle Dela Cruz, Neil Theise, Natalia Nieto
BACKGROUND & RATIONALE: High-mobility group box-1 (HMGB1) is a damage-associated molecular pattern (DAMP) increased in response to liver injury. Since HMGB1 is a ligand for the receptor for advanced glycation end-products (RAGE), we hypothesized that induction of HMGB1 could participate in the pathogenesis of liver fibrosis via RAGE cell-specific signaling mechanisms. RESULTS: liver HMGB1 protein expression correlated with fibrosis stage in patients with chronic Hepatitis C virus (HCV) infection, primary biliary cirrhosis (PBC) and alcoholic steatohepatitis (ASH)...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29762873/l-ornithine-l-aspartate-for-prevention-and-treatment-of-hepatic-encephalopathy-in-people-with-cirrhosis
#3
REVIEW
Ee Teng Goh, Caroline S Stokes, Sandeep S Sidhu, Hendrik Vilstrup, Lise Lotte Gluud, Marsha Y Morgan
BACKGROUND: Hepatic encephalopathy is a common complication of cirrhosis and has high associated morbidity and mortality. The condition is classified as overt if it is clinically apparent or minimal if only evident though psychometric testing. The exact pathogenesis of this syndrome is unknown although ammonia is thought to play a key role. L-ornithine L-aspartate has ammonia-lowering properties and may, therefore, benefit people with cirrhosis and hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of L-ornithine L-aspartate versus placebo, no intervention, or other active interventions in people with cirrhosis and hepatic encephalopathy...
May 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29761165/a-real-world-observational-cohort-of-patients-with-primary-biliary-cholangitis-target-primary-biliary-cholangitis-study-design-and-rationale
#4
Cynthia Levy, Christopher L Bowlus, Elizabeth Carey, Julie M Crawford, Karen Deane, Marlyn J Mayo, W Ray Kim, Michael W Fried
Primary biliary cholangitis (PBC) is a rare chronic cholestatic liver disease that may progress to biliary cirrhosis if left untreated. The first-line therapy for PBC is ursodeoxycholic acid (UDCA). Unfortunately, 1 of 3 patients does not respond to UDCA. These patients are at risk for developing clinical events, including cirrhosis, complications of portal hypertension, hepatocellular carcinoma, liver transplant, or death. Recently, the U.S. Food and Drug Administration approved obeticholic acid to be used in certain patients with PBC...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29758112/novel-and-emerging-therapies-for-cholestatic-liver-diseases
#5
Jordan Goldstein, Cynthia Levy
While bile acids are important for both digestion and signaling, hydrophobic bile acids can be harmful especially when in high concentrations. Mechanisms for protection of cholangiocytes against bile acid cytotoxicity include negative feedback loops via farnesoid X nuclear receptor (FXR) activation, the bicarbonate umbrella, cholehepatic shunting and anti-inflammatory signaling, among others. By altering or overwhelming these defense mechanisms, cholestatic diseases such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) can further progress to biliary cirrhosis, end-stage liver disease and death or liver transplantation...
May 14, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29751878/primary-biliary-cholangitis-and-autoimmune-hepatitis
#6
REVIEW
Raul S Gonzalez, Kay Washington
Primary biliary cholangitis and autoimmune hepatitis are common autoimmune diseases of the liver. Both have typical clinical presentations, including certain autoantibodies on serologic testing. Histologic features are also often typical: primary biliary cholangitis shows bile duct destruction (sometimes with granulomas), and autoimmune hepatitis shows prominent portal and lobular lymphoplasmacytic inflammation. Both have a wide differential diagnosis, including one another; they may also simultaneously occur within the same patient...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29745886/primary-myelofibrosis-but-not-autoimmune-myelofibrosis-accompanied-by-sj%C3%A3-gren-s-syndrome-and-primary-biliary-cirrhosis-in-a-patient-with-trisomy-8-mosaic-a-case-report-and-literature-review
#7
Chenyang Lu, Xiaoyan Wu, Hongyan Wen, Huiying Gao, Caihong Wang, Bo Yang, Zhipeng Liang, Chong Gao, Xiaofeng Li
Bone marrow fibrosis has been found to be associated with autoimmune disorders, and autoimmune myelofibrosis (AIMF) has been defined. Primary myelofibrosis (PMF), a clonal myeloproliferative disorder, should be distinguished from AIMF which has a good response to steroids, as the former has a high mortality and very bad response to conventional treatment. This case report describes a rare case of PMF accompanied with Sjögren's syndrome (SJS) and primary biliary cirrhosis (PBC), in a patient with trisomy 8 mosaic...
May 10, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29741240/follow-up-magnetic-resonance-imaging-3d-magnetic-resonance-cholangiopancreatography-in-patients-with-primary-sclerosing-cholangitis-challenging-for-experts-to-interpret
#8
R Zenouzi, T Liwinski, J Yamamura, C Weiler-Normann, M Sebode, S Keller, A W Lohse, C Schramm
BACKGROUND: In patients with primary sclerosing cholangitis follow-up magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP) is performed by many centres, particularly for the early detection of biliary malignancies and strictures. Clinically meaningful MRI-based definitions of primary sclerosing cholangitis related complications are, however, lacking. AIM: To investigate how primary sclerosing cholangitis experts interpret follow-up MRI/MRCP with a focus on conclusions that may impact clinical decision-making in primary sclerosing cholangitis...
May 9, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29740582/anti-high-density-lipoprotein-antibodies-and-antioxidant-dysfunction-in-immune-driven-diseases
#9
Javier Rodríguez-Carrio, Lourdes Mozo, Patricia López, Elena Nikiphorou, Ana Suárez
Introduction: Impaired high-density lipoprotein (HDL) levels and antioxidant functionality of HDL, mainly attributed to a decreased paraoxonase-1 (PON1) functionality, have been described in autoimmune conditions. In this setting, a role for humoral response in cardiovascular disease is emerging. This study evaluates the role of immunoglobulin G (IgG) antibodies against HDL and disease-related autoantibodies on HDL dysfunction in immune-driven diseases. Methods: Serum IgG anti-HDL antibodies, PON1 activity, and total antioxidant capacity (TAC) were quantified in 381 patients with different immune-driven diseases [18 mixed connective tissue disease (MCTD), 35 primary Sjögren syndrome (pSS), 38 systemic sclerosis (SSc), 33 ANCA-associated vasculitis (AAV), 60 diabetes mellitus 1, 29 autoimmune B12 deficiency/pernicious anemia, 29 primary biliary cirrhosis, 46 IBD/Crohn, 54 IBD/UC, and 39 celiac disease (CD)] and 138 healthy controls...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29739227/efficacy-and-safety-of-fenofibrate-add-on-therapy-for-patients-with-primary-biliary-cholangitis-and-a-suboptimal-response-to-udca
#10
Weijia Duan, Xiaojuan Ou, Xiaoming Wang, Yu Wang, Xinyan Zhao, Qianyi Wang, Xiaoning Wu, Wei Zhang, Hong Ma, Hong You, Jidong Jia
BACKGROUND: primary biliary cholangitis (PBC) patients with a suboptimal response to ursodeoxycholic acid (UDCA) have a significantly worse survival rate. Fenofibrate has been shown to improve the short-term biochemical response in this group of patients. However, there is limited data available on the safety and efficacy of its long-term use, especially in patients with cirrhosis. Methods:in this retrospective cohort study, fenofibrate was given to PBC patients with a suboptimal response to at least 12 months of UDCA (13-15 mg/kg/d) therapy...
May 9, 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29736833/are-clinicians-ready-for-safe-use-of-stratified-therapy-in-primary-biliary-cholangitis-pbc-a-study-of-educational-awareness
#11
Laura Jopson, Amardeep Khanna, Patricia Peterson, Elaine Rudell, Margaret Corrigan, David Jones
BACKGROUND: Primary Biliary Cholangitis (PBC, formerly cirrhosis), is a chronic cholestatic liver disease which until spring 2016 had a single licensed therapy, Ursodeoxycholic acid (UDCA). Approximately 30% of patients do not respond to UDCA, and are high-risk for progressing to end stage liver disease, transplantation or death. A new era of stratified medicine with second-line therapies to treat high-risk disease is emerging, with the first such second-line agent obeticholic acid recently receiving FDA and EMA approval and entering practice...
May 8, 2018: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/29710425/systemic-inflammation-and-immune-cell-phenotypes-are-associated-with-neuro-psychiatric-symptoms-in-patients-with-chronic-inflammatory-liver-diseases
#12
Amare Aregay, Meike Dirks, Verena Schlaphoff, Solomon Owusu Sekyere, Kim Haag, Christine Susanne Falk, Julia Hengst, Birgit Bremer, Ramona Schuppner, Michael P Manns, Henning Pflugrad, Markus Cornberg, Heiner Wedemeyer, Karin Weissenborn
BACKGROUND AND AIMS: Chronic inflammatory liver diseases are frequently associated with neuropsychiatric and cognitive dysfunctions. We hypothesized that symptomatic patients may show altered levels of soluble inflammatory mediators (SIMs) as well as changes in immune cell phenotypes. METHODS: A comprehensive immune-phenotyping including investigation of 50 SIMs as well as ex-vivo phenotypes of NK-cells, CD3+, CD4+, CD8+ and regulatory T cells in 40 patients with viral and autoimmune chronic liver diseases was performed...
April 30, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29707074/hepatobiliary-manifestations-and-complications-in-inflammatory-bowel-disease-a-review
#13
REVIEW
Fotios S Fousekis, Vasileios I Theopistos, Konstantinos H Katsanos, Epameinondas V Tsianos, Dimitrios K Christodoulou
Liver and biliary track diseases are common extraintestinal manifestations of inflammatory bowel disease (IBD), reported both in Crohn's disease and ulcerative colitis, and may occur at any time during the natural course of the disease. Their etiology is mainly related to pathophysiological changes induced by IBD, and secondary, due to drugs used in IBD. Fatty liver is considered as the most frequent hepatobiliary manifestation in IBD, while primary sclerosing cholangitis (PSC) is the most correlated hepatobiliary disorder and is more prevalent in patients with ulcerative colitis...
April 2018: Gastroenterology Research
https://www.readbyqxmd.com/read/29691599/-primary-biliary-cholangitis-established-and-novel-therapies
#14
REVIEW
M Vetter, A E Kremer
BACKGROUND: Patients with primary biliary cholangitis (PBC, formerly primary biliary cirrhosis) and insufficient treatment response or risk factors exhibit a remarkably increased risk for disease progression and associated complications. Furthermore, extrahepatic manifestations may considerably reduce quality of life in affected patients. OBJECTIVES: This article presents an overview on standard therapy with ursodeoxycholic acid (UDCA) and further therapeutic options in patients with insufficient treatment response...
April 24, 2018: Der Internist
https://www.readbyqxmd.com/read/29681633/secondary-sclerosing-cholangitis-in-critically-ill-patients
#15
Carlos Alberto Peña-Pérez, Juan Alberto Díaz Ponce-Medrano
Primary sclerosing cholangitis (PSC) is a rare idiopathic condition with immunopathogenic mechanisms where there is chronic progressive destruction of the biliary tree. Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, but is caused by specific processes which directly damage the biliary tree; examples include recurrent pancreatitis, bile duct malignancy, congenital bile duct abnormalities. A new cause of SSC has been described during or following significant critical illness associated with severe respiratory insufficiency, vasopressor requirement, shock and sepsis...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29678444/biliary-epithelium-a-neuroendocrine-compartment-in-cholestatic-liver-disease
#16
REVIEW
Laurent Ehrlich, Marinda Scrushy, Fanyin Meng, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis is characterized by abnormal accumulation of extracellular matrix (ECM) that can lead to ductopenia, cirrhosis, and even malignant transformation. In this review, we examine cholestatic liver diseases characterized by extensive biliary fibrosis such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), polycystic liver disease (PLD), and MDR2-/- and BDL mouse models. Following biliary injury, cholangiocytes, the epithelial cells that line the bile ducts, become reactive and adopt a neuroendocrine phenotype in which they secrete and respond to neurohormones and neuropeptides in an autocrine and paracrine fashion...
April 17, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29617396/the-impact-of-depression-and-antidepressant-usage-on-primary-biliary-cholangitis-clinical-outcomes
#17
Abdel-Aziz Shaheen, Gilaad G Kaplan, Wagdi Almishri, Isabelle Vallerand, Alexandra D Frolkis, Scott Patten, Mark G Swain
BACKGROUND: Depression is prevalent in primary biliary cholangitis (PBC) patients. Our aims were to examine the effects of depression and antidepressants on hepatic outcomes of PBC patients. METHODS: We used the UK Health Improvement Network database to identify PBC patients between 1974 and 2007. Our primary outcome was one of three clinical events: decompensated cirrhosis, liver transplantation and death. We assessed depression and each class of antidepressant medication in adjusted multivariate Cox proportional hazards models to identify independent predictors of outcomes...
2018: PloS One
https://www.readbyqxmd.com/read/29593060/the-british-society-of-gastroenterology-uk-pbc-primary-biliary-cholangitis-treatment-and-management-guidelines
#18
Gideon M Hirschfield, Jessica K Dyson, Graeme J M Alexander, Michael H Chapman, Jane Collier, Stefan Hübscher, Imran Patanwala, Stephen P Pereira, Collette Thain, Douglas Thorburn, Dina Tiniakos, Martine Walmsley, George Webster, David E J Jones
Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. Both genetic and environmental influences are presumed relevant to disease initiation. PBC is most prevalent in women and those over the age of 50, but a spectrum of disease is recognised in adult patients globally; male sex, younger age at onset (<45) and advanced disease at presentation are baseline predictors of poorer outcome...
March 28, 2018: Gut
https://www.readbyqxmd.com/read/29574435/autoimmune-pancreatitis-with-concomitant-autoimmune-haemolytic-anaemia
#19
Lauren Passby, Matthew Harris, Ahmed Al-Mukhtar
Autoimmune pancreatitis (AIP) is an infrequent cause of acute pancreatitis, being more commonly associated with chronic pancreatitis. AIP can be associated with other autoimmune manifestations, including Sjögren's, inflammatory bowel disease, primary biliary cirrhosis, rheumatoid arthritis, hypothyroidism and sarcoidosis. Rarely, concurrent autoimmune haemolytic anaemia (AIHA) is observed, as seen in our case report of a 33-year-old postpartum woman.
March 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29554146/evaluation-of-classical-and-novel-autoantibodies-for-the-diagnosis-of-primary-biliary-cholangitis-autoimmune-hepatitis-overlap-syndrome-pbc-aih-os
#20
Henry H Nguyen, Abdel Aziz Shaheen, Natalia Baeza, Ellina Lytvyak, Stefan J Urbanski, Andrew L Mason, Gary L Norman, Marvin J Fritzler, Mark G Swain
BACKGROUND AND AIMS: Up to 20% of Primary Biliary Cholangitis (PBC) patients are estimated to have features that overlap with Autoimmune Hepatitis (AIH). Patients with PBC-AIH overlap syndrome (PBC-AIH OS) have been reported to exhibit suboptimal responses to ursodeoxycholic acid therapy, and are more likely to progress to cirrhosis. Anti-double stranded DNA (anti-dsDNA) and anti-p53 have been previously suggested to be potential autoantibodies for identifying patients with PBC-AIH OS...
2018: PloS One
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