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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28105357/immunoglobulin-g4-mediated-sclerosing-cholangitis-as-a-risk-factor-for-cholangiocarcinoma-a-case-report
#1
Karin E Koopman, Elisabeth Bloemena, Geert Kazemier, Michael Klemt-Kropp
Immunoglobulin (Ig)G4-mediated disease is a systemic autoimmune disease, which occasionally presents solely as sclerosing cholangitis (SC). IgG4-mediated SC is challenging to diagnose, as it may mimic cholangiocarcinoma radiologically, and carcinoma cells may produce IgG4. The diagnosis of IgG4-mediated disease is based on histological consensus criteria and response to corticosteroids. In addition to the radiological and histological overlap between IgG4-mediated SC and cholangiocarcinoma, IgG4-mediated SC may be considered as a risk factor for the development of cholangiocarcinoma...
December 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28103424/no-negative-impact-of-serum-igg4-levels-on-clinical-outcome-in-435-patients-with-primary-sclerosing-cholangitis-from-japan
#2
Atsushi Tanaka, Susumu Tazuma, Takahiro Nakazawa, Hiroyuki Isayama, Toshio Tsuyuguchi, Kazuo Inui, Hajime Takikawa
BACKGROUND AND AIMS: Several studies have demonstrated that elevated serum IgG4 levels are associated with poor outcomes of primary sclerosing cholangitis (PSC), but the impact of serum IgG4 levels on PSC remains controversial. In this study, we aimed to determine prognostic factors of patients with PSC and to investigate the association between serum IgG4 levels and the clinical features and prognosis of PSC in a Japanese cohort. METHODS: We retrospectively analysed follow-up data for 435 patients with PSC (UMIN000018438)...
January 19, 2017: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/28089354/primary-biliary-cholangitis-primary-sclerosing-cholangitis-in-an-evolving-overlap-syndrome-a-case-report
#3
Daniele Mandolesi, Marco Lenzi, Antonietta D'Errico, Davide Festi, Franco Bazzoli, Antonio Colecchia
No abstract text is available yet for this article.
January 12, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#4
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28086796/prophylaxis-of-post-erc-infectious-complications-in-patients-with-biliary-obstruction-by-adding-antimicrobial-agents-into-erc-contrast-media-a-single-center-retrospective-study
#5
Hella Wobser, Agnetha Gunesch, Frank Klebl
BACKGROUND: Patients with biliary obstruction are at high risk to develop septic complications after endoscopic retrograde cholangiography (ERC). We evaluated the benefits of local application of antimicrobial agents into ERC contrast media in preventing post-ERC infectious complications in a high-risk study population. METHODS: Patients undergoing ERC at our tertiary referral center were retrospectively included. Addition of vancomycin, gentamicin and fluconazol into ERC contrast media was evaluated in a case-control design...
January 13, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28079751/updates-on-primary-sclerosing-cholangitis
#6
Chantal Housset
No abstract text is available yet for this article.
January 12, 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28077782/serum-and-tissue-markers-in-hepatocellular-carcinoma-and-cholangiocarcinoma-clinical-and-prognostic-implications
#7
REVIEW
Massimiliano Berretta, Carla Cavaliere, Lara Alessandrini, Brigida Stanzione, Gaetano Facchini, Luca Balestreri, Tiziana Perin, Vincenzo Canzonieri
HCC represents the sixth most common cancer worldwide and the second leading cause of cancer-related death. Despite the high incidence, treatment options for advanced HCC remain limited and unsuccessful, resulting in a poor prognosis. Despite the major advances achieved in the diagnostic management of HCC, only one third of the newly diagnosed patients are presently eligible for curative treatments. Advances in technology and an increased understanding of HCC biology have led to the discovery of novel biomarkers...
December 14, 2016: Oncotarget
https://www.readbyqxmd.com/read/28073151/ccbe1-mutation-causing-sclerosing-cholangitis-expanding-the-spectrum-of-lymphedema-cholestasis-syndrome
#8
André Viveiros, Marion Reiterer, Benedikt Schaefer, Armin Finkenstedt, Stefan Schneeberger, Hubert Schwaighofer, Patrizia Moser, Rudolf Sprenger, Bernhard Glodny, Wolfgang Vogel, Andreas R Janecke, Heinz Zoller
No abstract text is available yet for this article.
January 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#9
REVIEW
Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis...
January 9, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28056976/a-novel-approach-to-genome-wide-association-analysis-identifies-genetic-associations-with-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis-in-polish-patients
#10
Agnieszka Paziewska, Andrzej Habior, Agnieszka Rogowska, Włodzimierz Zych, Krzysztof Goryca, Jakub Karczmarski, Michalina Dabrowska, Filip Ambrozkiewicz, Bozena Walewska-Zielecka, Marek Krawczyk, Halina Cichoz-Lach, Piotr Milkiewicz, Agnieszka Kowalik, Krzysztof Mucha, Joanna Raczynska, Joanna Musialik, Grzegorz Boryczka, Michal Wasilewicz, Irena Ciecko-Michalska, Malgorzata Ferenc, Maria Janiak, Alina Kanikowska, Rafal Stankiewicz, Marek Hartleb, Tomasz Mach, Marian Grzymislawski, Joanna Raszeja-Wyszomirska, Ewa Wunsch, Tomasz Bobinski, Michal Mikula, Jerzy Ostrowski
BACKGROUND: Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are forms of hepatic autoimmunity, and risk for both diseases has a strong genetic component. This study aimed to define the genetic architecture of PBC and PSC within the Polish population. METHODS: Subjects were 443 women with PBC, 120 patients with PSC, and 934 healthy controls recruited from Gastroenterology Departments in various Polish hospitals. Allelotyping employed a pooled-DNA sample-based genome-wide association study (GWAS) approach, using Illumina Human Omni2...
January 6, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28056490/knockout-of-the-primary-sclerosing-cholangitis-risk-gene-fut2-causes-liver-disease-in-mice
#11
Luca Maroni, Simon D Hohenester, Stan F J van de Graaf, Dagmar Tolenaars, Krijn van Lienden, Joanne Verheij, Marco Marzioni, Tom H Karlsen, Ronald P J Oude Elferink, Ulrich Beuers
: The etiopathogenesis of primary sclerosing cholangitis (PSC) is unknown. Genetic variants of fucosyltransferase 2 (FUT2) have been identified in genome-wide association studies as risk factor for PSC. We investigated the role of Fut2 in murine liver pathophysiology by studying Fut2(-/-) mice. Fut2(-/-) mice were viable and fertile, had lower body weight than wild type (wt) littermates and grey fur, as described earlier. Half of the Fut2(-/-) mice showed serum bile salt levels 40 times higher than wt (Fut2(-/-)(high) ), whereas the remainder were normocholanemic (Fut2(-/-)(low) )...
January 5, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#12
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#13
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28050782/colorectal-cancer-in-inflammatory-bowel-diseases-a-population-based-study-in-utah
#14
N Jewel Samadder, John F Valentine, Stephen Guthery, Harminder Singh, Charles N Bernstein, Yuan Wan, Jathine Wong, Kenneth Boucher, Lisa Pappas, Kerry Rowe, Mary Bronner, Cornelia M Ulrich, Randall W Burt, Karen Curtin, Ken R Smith
BACKGROUND AND AIMS: The molecular, endoscopic, and histological features of IBD-associated CRC differ from sporadic CRC. The objective of this study was to describe the prevalence, clinical features, and prognosis of IBD-associated CRC compared to patients with sporadic CRC in a US statewide population-based cohort. METHODS: All newly diagnosed cases of CRC between 1996 and 2011 were obtained from Utah Cancer Registry. IBD was identified using a previously validated algorithm, from statewide databases of Intermountain Healthcare, University of Utah Health Sciences, and the Utah Population Database...
January 3, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28050039/impact-of-crohn-s-disease-duration-on-the-risk-of-cholangiocarcinoma-in-patients-with-primary-sclerosing-cholangitis
#15
De-Jun Cui, Xun Zhao, Mei Hu
No abstract text is available yet for this article.
January 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28042931/retraction-notice-to-ps124-efficacy-and-safety-of-induction-dosing-of-vedolizumab-for-reducing-biliary-inflammation-in-primary-sclerosing-cholangitis-psc-in-individuals-with-inflammatory-bowel-disease-j-hepatol-64-2016-s199
#16
B Eksteen, J Heatherington, J Oshiomogo, R Panaccione, G Kaplan, S Ghosh
No abstract text is available yet for this article.
January 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28030370/the-evolution-of-natural-history-of-primary-sclerosing-cholangitis
#17
Will R Takakura, James H Tabibian, Christopher L Bowlus
PURPOSE OF REVIEW: Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. RECENT FINDINGS: PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis...
December 24, 2016: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28030369/intestinal-microbiota-in-primary-sclerosing-cholangitis
#18
Johannes R Hov, Martin Kummen
PURPOSE OF REVIEW: Alterations of the gut-liver axis have been linked to the pathogenesis of primary sclerosing cholangitis (PSC) since the disease was first described. The purpose of this review is to discuss multiple recent studies on the intestinal microbiota in human PSC and experimental models of this disease. RECENT FINDINGS: Data are available from eight cross-sectional studies of human PSC, which include a variable number of patients (n = 11-85), material (mucosal or fecal), and microbiota profiling methodology...
December 24, 2016: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28028930/an-update-on-cancer-risk-and-surveillance-in-primary-sclerosing-cholangitis
#19
Jennifer L Horsley-Silva, Eduardo A Rodriguez, Diana L Franco, Keith D Lindor
Malignancy represents substantial morbidity and mortality in patients with primary sclerosing cholangitis (PSC). This subset of patients has been proven to be at increased risk for developing cholangiocarcinoma, gallbladder carcinoma, and colorectal cancer in those with overlapping inflammatory bowel disease. Herein we review the prevalence of these malignancies and recommend screening tools and current knowledge to reduce the disease burden in this population. Cholangiocarcinoma is the most dominant malignancy affecting PSC patients, with a lifetime risk ranging from 5-20%...
December 27, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28027592/outcomes-of-liver-retransplantation-in-patients-with-primary-sclerosing-cholangitis
#20
Jacqueline B Henson, Yuval A Patel, Lindsay Y King, Jiayin Zheng, Shein-Chung Chow, Andrew J Muir
: Liver retransplantation in patients with primary sclerosing cholangitis (PSC) has not been well studied. The aims of this study were to characterize patients with PSC listed for and undergoing retransplantation and to describe the outcomes in these patients. The United Network for Organ Sharing/Organ Procurement and Transplantation Network database was used to identify all primary liver transplantations and subsequent relistings and first retransplantations in adults with PSC between 1987 and 2015...
December 27, 2016: Liver Transplantation
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