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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28432731/association-between-progressive-hepatic-morphology-changes-on-serial-mr-imaging-and-clinical-outcome-in-primary-sclerosing-cholangitis
#1
Yu Xuan Kitzing, Siobhan A Whitley, Sara S Upponi, Brijesh Srivastava, Graeme Jm Alexander, David J Lomas
INTRODUCTION: Hepatic morphology changes are well described in Primary Sclerosing Cholangitis and characterised by a combination of atrophy and hypertrophy changes. This study investigates the relationship between progression of these changes over time and clinical outcome in patients with PSC. METHODS: Fifty-three patients with PSC (mean age 44, 28 males and 25 females) who underwent serial MRI liver studies at least one year apart were identified. The first and the last MRI studies were selected for the retrospective analysis...
April 22, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28428344/vascular-adhesion-protein-1-is-elevated-in-primary-sclerosing-cholangitis-is-predictive-of-clinical-outcome-and-facilitates-recruitment-of-gut-tropic-lymphocytes-to-liver-in-a-substrate-dependent-manner
#2
Palak J Trivedi, Joseph Tickle, Mette Nåmdal Vesterhus, Peter J Eddowes, Tony Bruns, Jani Vainio, Richard Parker, David Smith, Evaggelia Liaskou, Liv Wenche Thorbjørnsen, Gideon M Hirschfield, Kaisa Auvinen, Stefan G Hubscher, Marko Salmi, David H Adams, Chris J Weston
OBJECTIVE: Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of IBD. This clinical association is linked pathologically to the recruitment of mucosal T cells to the liver, via vascular adhesion protein (VAP)-1-dependent enzyme activity. Our aim was to examine the expression, function and enzymatic activation of the ectoenzyme VAP-1 in patients with PSC. DESIGN: We examined VAP-1 expression in patients with PSC, correlated levels with clinical characteristics and determined the functional consequences of enzyme activation by specific enzyme substrates on hepatic endothelium...
April 20, 2017: Gut
https://www.readbyqxmd.com/read/28427764/role-of-endoscopy-in-primary-sclerosing-cholangitis-european-society-of-gastrointestinal-endoscopy-esge-and-european-association-for-the-study-of-the-liver-easl-clinical-guideline
#3
(no author information available yet)
This guideline is an official statement of the European Society of Gastrointestinal Endoscopy (ESGE) and of the European Association for the Study of the Liver (EASL) on primary sclerosing cholangitis. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) system was adopted to define the strength of recommendations and the quality of evidence. Main recommendations.
April 13, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28426778/apai-polymorphism-of-vitamin-d-receptor-affects-health-related-quality-of-life-in-patients-with-primary-sclerosing-cholangitis
#4
Agnieszka Kempinska-Podhorodecka, Malgorzata Milkiewicz, Dariusz Jabłonski, Piotr Milkiewicz, Ewa Wunsch
BACKGROUND: Polymorphisms of vitamin D receptor (VDR) contribute to the pathogenesis of multiple autoimmune conditions. METHODS: We investigated the incidence of VDR polymorphisms (rs1544410-BsmI; rs7975232-ApaI; rs731236-TaqI) in a group of patients with primary sclerosing cholangitis (PSC, n = 275) and in healthy controls (n = 376). Additionally, correlations of the VDR polymorphisms with clinical and biochemical factors of the disease were analysed. RESULTS: The genotype and allele distributions of these polymorphisms in PSC patients were similar to those observed in controls...
2017: PloS One
https://www.readbyqxmd.com/read/28422840/case-report-a-female-case-of-isolated-igg4-related-sclerosing-cholangitis-mimicking-cholangiocarcinoma
#5
Jianchun Xiao, Guanqiao Li, Gang Yang, Congwei Jia, Binglu Li
RATIONALE: IgG4-related disease is a newly recognized fibroinflammatory disorder, characterized by tumefactive lesions, storiform fibrosis and IgG4-positive plasma cells infiltration. IgG4-related sclerosing cholangitis (IgG4-SC) is the most common extrapancreatic manifestation of IgG4-related disease, but it is frequently associated with autoimmune pancreatitis(AIP). Only few case was reported to be diagnosed with IgG4-SC in the absence of AIP, with a striking male preponderance. Here we report a female case of isolated IgG4 related sclerosing cholangitis mimicking cholangiocarcinoma...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28420030/role-of-endoscopy-in-primary-sclerosing-cholangitis-european-society-of-gastrointestinal-endoscopy-esge-and-european-association-for-the-study-of-the-liver-easl-clinical-guideline
#6
Lars Aabakken, Tom H Karlsen, Jörg Albert, Marianna Arvanitakis, Olivier Chazouilleres, Jean-Marc Dumonceau, Martti Färkkilä, Peter Fickert, Gideon M Hirschfield, Andrea Laghi, Marco Marzioni, Michael Fernandez, Stephen P Pereira, Jürgen Pohl, Jan-Werner Poley, Cyriel Y Ponsioen, Christoph Schramm, Fredrik Swahn, Andrea Tringali, Cesare Hassan
1 ESGE/EASL recommend that, as the primary diagnostic modality for PSC, magnetic resonance cholangiography (MRC) should be preferred over endoscopic retrograde cholangiopancreatography (ERCP).Moderate quality evidence, strong recommendation. 2 ESGE/EASL suggest that ERCP can be considered if MRC plus liver biopsy is equivocal or contraindicated in patients with persisting clinical suspicion of PSC. The risks of ERCP have to be weighed against the potential benefit with regard to surveillance and treatment recommendations...
April 18, 2017: Endoscopy
https://www.readbyqxmd.com/read/28417463/pharmacological-interventions-for-primary-sclerosing-cholangitis-an-attempted-network-meta-analysis
#7
REVIEW
Francesca Saffioti, Kurinchi Selvan Gurusamy, Neil Hawkins, Clare D Toon, Emmanuel Tsochatzis, Brian R Davidson, Douglas Thorburn
BACKGROUND: Primary sclerosing cholangitis is a chronic cholestatic liver disease that is associated with both hepatobiliary and colorectal malignancies, which can result in liver cirrhosis and its complications. The optimal pharmacological treatment for patients with primary sclerosing cholangitis remains controversial. OBJECTIVES: To assess the comparative benefits and harms of different pharmacological interventions in people with primary sclerosing cholangitis by performing a network meta-analysis, and to generate rankings of available pharmacological interventions according to their safety and efficacy...
March 28, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28410344/preoperative-clostridium-difficile-infection-does-not-affect-pouch-outcomes-in-patients-with-ulcerative-colitis-who-undergo-ileal-pouch-anal-anastomosis
#8
Amy L Lightner, Chung Sang Tse, Kevin Quinn, John R Bergquist, Felicity Enders, Richard Pendegraft, Sahil Khanna, Laura Raffals
BACKGROUND: The operation of choice for patients with chronic ulcerative colitis (CUC) is restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). Pouchitis is the most common complication after IPAA. The incidence of Clostridium difficile infection (CDI) is higher in CUC patients than the general population and can lead to significant disease flares and higher rates of colectomy. We sought to determine the risk of pouchitis in patients with precolectomy CDI and 90-day postoperative IPAA complications...
April 13, 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28407375/h19-promotes-cholestatic-liver-fibrosis-by-preventing-zeb1-mediated-inhibition-of-epcam
#9
Yongfeng Song, Chune Liu, Xia Liu, Jocelyn Trottier, Michele Beaudoin, Li Zhang, Chad Pope, Guangyong Peng, Olivier Barbier, Xiaobo Zhong, Linheng Li, Li Wang
Based on our recent finding that disruption of bile acid (BA) homeostasis in mice results in the induction of hepatic lncRNA H19 expression, we sought to elucidate the role of H19 in cholestatic liver fibrosis. Hepatic overexpression of H19RNA augmented bile duct ligation (BDL)-induced liver fibrosis, which was accompanied by the elevation of serum ALT, AST, bilirubin, and BA levels. Multiple genes related to liver fibrosis, inflammation, and biliary hyperplasia were increased in H19-BDL vs Null-BDL mice, whereas genes in BA synthesis were decreased...
April 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28407274/the-riddle-of-juvenile-sclerosing-cholangitis
#10
EDITORIAL
Giorgina Mieli-Vergani, Diego Vergani
No abstract text is available yet for this article.
April 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28404115/advances-in-primary-sclerosing-cholangitis
#11
REVIEW
Jennifer L Horsley-Silva, Elizabeth J Carey, Keith D Lindor
Primary sclerosing cholangitis is a chronic, progressive cholangiopathy that frequently affects men and is associated with inflammatory bowel disease. Although the cause of the disease is still debated, a genetic association and link to immune-mediated disease triggered by environmental factors are thought to contribute. The disease can present as isolated imaging abnormalities, biochemical changes, cholangiocarcinoma, or end-stage complications such as cirrhosis. Symptoms of primary sclerosing cholangitis include fatigue, jaundice, pruritus, or steatorrhoea...
September 2016: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28403110/a-feasibility-study-of-digital-single-operator-cholangioscopy-for-diagnostic-and-therapeutic-procedure-with-videos
#12
Miyuki Imanishi, Takeshi Ogura, Yoshitaka Kurisu, Saori Onda, Wataru Takagi, Atsushi Okuda, Akira Miyano, Mio Amano, Nobu Nishioka, Daisuke Masuda, Kazuhide Higuchi
Recently, the novel SpyGlass DS Direct Visualization system (SPY DS) has become available. This system offers several advantages over the conventional SPYGlass system. This study evaluated the clinical feasibility and efficacy of diagnostic and therapeutic procedures for biliary disorder using SPY DS.In this retrospective study, consecutive patients who had biliary disorder were enrolled between November 2015 and February 2016. All patients could not be diagnosed or treated by standard endoscopic retrograde cholangiopancreatography in our hospital or at another hospital...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28393105/patients-with-primary-sclerosing-cholangitis-require-more-sedation-during-endoscopic-retrograde-cholangiography
#13
Carsten Keil, Lukas Aguirre Dávila, Theodor Framke, Henrike Lenzen, Michael P Manns, Tim O Lankisch, Torsten Voigtländer
Background and study aims Patients with primary sclerosing cholangitis (PSC) require repeated endoscopic retrograde cholangiography (ERC). Our aim was to evaluate whether patients with PSC require higher doses of sedation during ERC. Patients and methods We retrospectively analyzed all patients undergoing ERC from 2006 to 2013 who received conscious sedation with propofol and midazolam. The duration of the intervention and a potential progression of propofol consumption or intervention time by visit number were analyzed...
April 2017: Endoscopy International Open
https://www.readbyqxmd.com/read/28391417/differences-in-phenotypes-and-liver-transplantation-outcomes-by-age-group-in-patients-with-primary-sclerosing-cholangitis
#14
Jacqueline B Henson, Yuval A Patel, Julius M Wilder, Jiayin Zheng, Shein-Chung Chow, Lindsay Y King, Andrew J Muir
BACKGROUND: There is increasing evidence for a heterogeneity of phenotypes in primary sclerosing cholangitis (PSC), but differences across the age spectrum in adults with PSC have not been well characterized. AIMS: To characterize phenotypic variations and liver transplantation outcomes by age group in adults with PSC. METHODS: The United Network for Organ Sharing database was used to identify waitlist registrations for primary liver transplantation in adults with PSC...
April 8, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#15
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28384790/treatment-of-severe-hypercholesterolemia-in-a-woman-with-advanced-primary-sclerosing-cholangitis
#16
Changting Xiao, Robert A Hegele, Gary F Lewis
No abstract text is available yet for this article.
April 5, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28383652/th1-and-innate-lymphoid-cells-accumulate-in-primary-sclerosing-cholangitis-associated-inflammatory-bowel-disease
#17
Agnes Gwela, Priya Siddhanathi, Roger W Chapman, Simon Travis, Fiona Powrie, Carolina V Arancibia-Cárcamo, Alessandra Geremia
Background and Aims: Primary sclerosing cholangitis (PSC) is an idiopathic chronic disorder of the hepatobiliary system associated with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). Colitis in patients with PSC and UC (PSC-UC) exhibits characteristic features and is linked to increased colon cancer risk. Genetic studies have identified immune-related susceptibility genes that only partially overlap with those involved in IBD. These observations suggest that PSC-UC may represent a distinct form of IBD...
April 5, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28376583/inflammatory-bowel-disease-and-primary-sclerosing-cholangitis-a-review-of-the-phenotype-and-associated-specific-features
#18
REVIEW
Carolina Palmela, Farhad Peerani, Daniel Castaneda, Joana Torres, Steven H Itzkowitz
Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70% of cases. Although the pathogenesis is still unknown for both diseases, there is increasing evidence to indicate that they share a common underlying predisposition. Herein, we review the epidemiology, diagnosis, disease pathogenesis, and specific clinical features of the PSC-IBD phenotype. Patients with PSC-IBD have a distinct IBD phenotype with an increased incidence of pancolitis, backwash ileitis, and rectal sparing...
April 6, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28373776/severe-infection-with-multidrug-resistant-salmonella-choleraesuis-in-a-young-patient-with-primary-sclerosing-cholangitis
#19
Philip G Ferstl, Claudia Reinheimer, Katalin Jozsa, Stefan Zeuzem, Volkhard Aj Kempf, Oliver Waidmann, Georgios Grammatikos
Massive global spread of multidrug-resistant (MDR) Salmonella spp. expressing extended-spectrum beta-lactamase (ESBL) and additional resistance to fluoroquinolones has often been attributed to high international mobility as well as excessive use of oral antibiotics in livestock farming. However, MDR Salmonella spp. have not been mentioned as a widespread pathogen in clinical settings so far. We demonstrate the case of a 25-year-old male with primary sclerosing cholangitis who tested positive for MDR Salmonella enterica serotype Choleraesuis expressing ESBL and fluoroquinolone resistance...
March 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28371374/vitamin-a-deficiency-in-chronic-cholestatic-liver-disease-is-vitamin-a-therapy-beneficial
#20
Cora Freund, Daniel Nils Gotthardt
Chronic cholestatic diseases are progressive diseases of the biliary tract that cause hepatic fibrosis and ultimately lead to liver failure. Liver transplantation is the sole curative option currently available, and because of high morbidity and mortality rates of these diseases, new therapeutic approaches are needed. Vitamin A is a nutrient essential for health as it regulates many processes, including epithelial growth and immunological processes. Vitamin A is primarily stored in hepatic stellate cells, and during liver injury, through an unknown mechanism, these cells lose vitamin A and convert into collagen-producing myofibroblasts, which contributes to hepatic fibrosis...
March 29, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
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