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primary cholangitis

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https://www.readbyqxmd.com/read/29775793/analysis-of-dna-methylation-at-specific-loci-in-stool-samples-detects-colorectal-cancer-and-high-grade-dysplasia-in-patients-with-inflammatory-bowel-disease
#1
John B Kisiel, Pasquale Klepp, Hatim T Allawi, William R Taylor, Maria Giakoumopoulos, Tamara Sander, Tracy C Yab, Bjorn A Moum, Graham P Lidgard, Stephan Brackmann, Douglas W Mahoney, Arne Roseth, David A Ahlquist
BACKGROUND & AIMS: Patients with inflammatory bowel diseases (IBD), including ulcerative colitis (UC) and Crohn's disease (CD), are at increased risk for colorectal cancer (CRC). Analyses of DNA methylation patterns in stool samples have been reported to detect CRC in patients with IBD. We sought to validate these findings in larger cohorts and assess the accuracy of analysis of DNA methylation patterns in stool for detection of CRC and high-grade dysplasia (HGD) normalized to methylation level at ZDHHC1...
May 15, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29775791/an-imaging-biomarker-for-assessing-hepatic-function-in-patients-with-primary-sclerosing-cholangitis
#2
Jennifer Schulze, Henrike Lenzen, Jan B Hinrichs, Burckhardt Ringe, Michael P Manns, Frank Wacker, Kristina I Ringe
BACKGROUND & AIMS: We aimed to evaluate the potential of hepatobiliary phase magnetic resonance imaging (MRI) as parameter for assessment of hepatocellular function in patients with primary sclerosing cholangitis (PSC). METHODS: We collected data from 111 patients (83 male, 28 female; median, 44 years old), from March 2012 through March 2016, with a confirmed diagnosis of PSC who underwent MRI evaluation before and after injection (hepatobiliary phase) of a hepatocyte-specific contrast agent (gadoxetate disodium)...
May 15, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29774578/the-immunobiology-of-rankl-and-myeloid-derived-suppressor-cell-activation-in-igg4-related-sclerosing-cholangitis
#3
Min Lian, Qixia Wang, Xiang Jiang, Jun Zhang, Yiran Wei, Yanmei Li, Bo Li, Weihua Chen, Haiyan Zhang, Qi Miao, Yanshen Peng, Xiao Xiao, Li Sheng, Weici Zhang, Jingyuan Fang, Ruqi Tang, M Eric Gershwin, Xiong Ma
The primary function of myeloid derived suppressor cells (MDSCs) is reflected in their immune modulatory role in several immune-mediated diseases. In IgG4-related disease (IgG4-RD), it has been hypothesized that there are selective regulatory defects that lead to a Th2 bias immune response. Herein we have taken advantage of a large cohort of patients with IgG4-related sclerosing cholangitis (IgG4-SC), the most common extra-pancreatic involvement of IgG4-RD, as well as controls consisting of primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and healthy volunteers, to study MDSC...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29773854/genome-wide-haplotype-association-analysis-of-primary-biliary-cholangitis-risk-in-japanese
#4
Cindy Im, Yadav Sapkota, Wonjong Moon, Minae Kawashima, Minoru Nakamura, Katsushi Tokunaga, Yutaka Yasui
Primary biliary cholangitis (PBC) susceptibility loci have largely been discovered through single SNP association testing. In this study, we report genic haplotype patterns associated with PBC risk genome-wide in two Japanese cohorts. Among the 74 genic PBC risk haplotype candidates we detected with a novel methodological approach in a discovery cohort of 1,937 Japanese, nearly two-thirds were replicated (49 haplotypes, Bonferroni-corrected P < 6.8 × 10-4 ) in an independent Japanese cohort (N = 949)...
May 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29767269/-igg4-associated-cholangitis-clinical-presentation-of-an-overlooked-disease-entity
#5
REVIEW
T Herta, J Verheij, U Beuers
IgG4-associated cholangitis (IAC) is the hepatobiliary manifestation of immunoglobulin G4-related disease, which is an immune-mediated fibroinflammatory systemic disorder characterised by often elevated IgG4 serum levels and typical histopathological findings in affected tissues. IAC is frequently (>90%) accompanied by autoimmune pancreatitis type 1 (AIP), which is the pancreatic manifestation of immunoglobulin G4-related disease. In 80-85% of the cases patients with IAC are male, above 50 years of age and present with jaundice and weight loss...
May 16, 2018: Der Internist
https://www.readbyqxmd.com/read/29761166/ni-0801-an-anti-chemokine-c-x-c-motif-ligand-10-antibody-in-patients-with-primary-biliary-cholangitis-and-an-incomplete-response-to-ursodeoxycholic-acid
#6
Kathy L de Graaf, Geneviève Lapeyre, Florence Guilhot, Walter Ferlin, Stuart M Curbishley, Marco Carbone, Paul Richardson, Sulleman Moreea, C Anne McCune, Stephen D Ryder, Roger W Chapman, Annarosa Floreani, David E Jones, Cristina de Min, David H Adams, Pietro Invernizzi
NI-0801 is a fully human monoclonal antibody against chemokine (C-X-C motif) ligand 10 (CXCL10), which is involved in the recruitment of inflammatory T cells into the liver. The safety and efficacy of NI-0801 was assessed in patients with primary biliary cholangitis. In this open-label phase 2a study, patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid received six consecutive intravenous administrations of NI-0801 (10 mg/kg) every 2 weeks. Patients were followed up for 3 months after the last infusion...
May 2018: Hepatology Communications
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
#7
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
#8
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/29754140/improvement-of-primary-biliary-cholangitis-pbc-under-treatment-with-sulfasalazine-and-abatacept
#9
Florian Popp, David Semela, Johannes von Kempis, Ruediger B Mueller
No abstract text is available yet for this article.
May 12, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29753567/junctional-adhesion-molecules-jam-b-and-jam-c-promote-autoimmune-mediated-liver-fibrosis-in-mice
#10
Edith Hintermann, Monika Bayer, Clara Benedetta Conti, Sina Fuchs, Michel Fausther, Patrick S Leung, Michel Aurrand-Lions, Richard Taubert, Josef M Pfeilschifter, Mireen Friedrich-Rust, Detlef Schuppan, Jonathan A Dranoff, M Eric Gershwin, Michael P Manns, Beat A Imhof, Urs Christen
Fibrosis remains a serious health concern in patients with chronic liver disease. We recently reported that chemically induced chronic murine liver injury triggers increased expression of junctional adhesion molecules (JAMs) JAM-B and JAM-C by endothelial cells and de novo synthesis of JAM-C by hepatic stellate cells (HSCs). Here, we demonstrate that biopsies of patients suffering from primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) display elevated levels of JAM-C on portal fibroblasts (PFs), HSCs, endothelial cells and cholangiocytes, whereas smooth muscle cells expressed JAM-C constitutively...
May 9, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29751878/primary-biliary-cholangitis-and-autoimmune-hepatitis
#11
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/29743796/increased-incidence-of-post-transplant-lymphoproliferative-disorder-in-autoimmune-liver-disease-an-irish-national-experience
#12
Ahmed Abu-Shanab, Yasser Ged, Naeem Ullah, Diarmaid Houlihan, Aiden McCormick
Background: Post-Transplant Lymphoproliferative Disorder (PTLD) is a well-recognized complication post solid organs transplant. PTLD represents a broad spectrum of abnormalities ranging from an infectious mononucleosis like illness to malignant lymphoma. Methods: A retrospective study was performed by collecting data of orthotopic liver transplant (OLT) patients in the National Liver Unit in Ireland from December 1993 to December 2014. Data was analyzed to identify PTLD patients and determine their demographic details, the indication for liver transplant, presenting symptoms, immunosuppression regimens, Epstein"Barr virus (EBV) status and PTLD outcome...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29742811/primary-sclerosing-cholangitis-risk-estimate-tool-presto-predicts-outcomes-in-psc-a-derivation-validation-study-using-machine-learning
#13
John E Eaton, Mette Vesterhus, Bryan M McCauley, Elizabeth J Atkinson, Erik M Schlicht, Brian D Juran, Andrea A Gossard, Nicholas F LaRusso, Gregory J Gores, Tom H Karlsen, Konstantinos N Lazaridis
BACKGROUND & AIMS: Improved methods are needed to risk stratify and predict outcomes in patients with primary sclerosing cholangitis (PSC). Therefore, we sought to derive and validate a new prediction model and compare its performance to existing surrogate markers. METHODS: The model was derived using 509 subjects from a multicenter North American cohort and validated in an international multicenter cohort (n=278). Gradient boosting, a machine based learning technique, was used to create the model...
May 9, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29742724/a-case-report-of-immunoglobulin-g4-related-sclerosing-cholangitis-with-multiple-relapse
#14
Xiaoqin Dong, Na Huo, Zhao Wu, Guiqiang Wang, He Wang, Hong Zhao
RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital with neoplasms of the bile duct and pancreas at first visit in December 2011...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29741603/phenotype-and-clinical-course-of-inflammatory-bowel-disease-with-co-existent-celiac-disease
#15
Chung Sang Tse, Parakkal Deepak, Jaime De La Fuente, Adam C Bledsoe, Joseph J Larson, Joseph A Murray, Konstantinos A Papadakis
Background and Aims: Inflammatory bowel diseases, principally Crohn's disease and ulcerative colitis, and celiac disease are among the most common immune-mediated gastrointestinal diseases. We aim to elucidate the clinical course and outcomes of patients with concomitant inflammatory bowel disease and celiac disease, a unique population that remains scarcely studied to date. Methods: A retrospective matched case-control study of adults with coexistent inflammatory bowel disease and celiac disease was performed at a tertiary referral institution in North America...
May 7, 2018: Journal of Crohn's & Colitis
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
#16
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/29739227/efficacy-and-safety-of-fenofibrate-add-on-therapy-for-patients-with-primary-biliary-cholangitis-and-a-suboptimal-response-to-udca
#17
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/29737954/-90-y-radioembolization-for-hepatic-malignancy-in-patients-with-previous-biliary-intervention-multicenter-analysis-of-hepatobiliary-infections
#18
Kavi K Devulapalli, Nicholas Fidelman, Michael C Soulen, Matthew Miller, Matthew S Johnson, Eric Addo, Ghassan El-Haddad, Charles Nutting, James Morrison, Khashayar Farsad, R Peter Lokken, Ron C Gaba, Jacob Fleming, Daniel B Brown, Sharon W Kwan, Steven C Rose, Kevin A Pennycooke, David M Liu, Sarah B White, Ripal Gandhi, Ann A Lazar, Robert K Kerlan
Purpose To determine the frequency of hepatobiliary infections after transarterial radioembolization (TARE) with yttrium 90 (90 Y) in patients with liver malignancy and a history of biliary intervention. Materials and Methods For this retrospective study, records of all consecutive patients with liver malignancy and history of biliary intervention treated with TARE at 14 centers between 2005 and 2015 were reviewed. Data regarding liver function, 90 Y dosimetry, antibiotic prophylaxis, and bowel preparation prophylaxis were collected...
May 8, 2018: Radiology
https://www.readbyqxmd.com/read/29737419/langerhans-cell-histiocytosis-presenting-as-crohn-s-disease-a-case-report
#19
Amelie Therrien, Zaki El Haffaf, Claire Wartelle-Bladou, Justin Côté-Daigneault, Bich N Nguyen
PURPOSE: We describe an exceptional case of Langerhans cell histiocytosis (LCH) that presented as Crohn's disease and primary sclerosing cholangitis. METHODS: The patient's clinical, endoscopic, and histologic data from the Centre Hospitalier de l'Universite de Montreal were reviewed, as well as the literature on LCH involving the digestive tract and the liver, with a focus on the similarities with Crohn's disease and primary sclerosing cholangitis. RESULTS: A 39 years-old man first presented with anal fissures and deep punctiform colonic ulcers...
May 8, 2018: International Journal of Colorectal Disease
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
#20
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
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