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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28333202/risk-of-rectal-neoplasia-after-colectomy-and-ileorectal-anastomosis-for-ulcerative-colitis
#1
Mathieu Uzzan, Julien Kirchgesner, Nadia Oubaya, Aurélien Amiot, Jean-Marc Gornet, Philippe Seksik, Stéphane Nancey, Eddy Cotte, Matthieu Allez, Gilles Boschetti, David Laharie, Nicola de Angelis, Maria Nachury, Anne-Laure Pelletier, Vered Abitbol, Mathurin Fumery, Antoine Brouquet, Anthony Buisson, Romain Altwegg, Jacques Cosnes, Yves Panis, Xavier Treton
Introduction-: Colectomy can be required in the management of ulcerative colitis (UC). While ileal pouch anal-anastomosis (IPAA) is the recommended reconstruction technique, ileorectal anastomosis (IRA) is still performed and might present some advantages. However, the risk of rectal neoplasia might limit its indication. The aims of our study were to determine the incidence of rectal neoplasias following IRA for UC and to identify risk factors associated with rectal carcinomas. Methods-: We performed a multicenter retrospective study including patients who underwent IRA for UC from 1960 to 2014 in 13 centers...
February 21, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28328835/poor-response-of-initial-steroid-therapy-for-igg4-related-sclerosing-cholangitis-with-multiple-organs-affected
#2
Wei Liu, Weijie Chen, Xiaodong He, Qiang Qu, Tao Hong, Binglu Li
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare biliary manifestation in which many other organs might be affected. The purpose of our study was to investigate the different clinical characteristics and initial steroid response between IgG4-SC patients with and without other organs affected.A series of patients with IgG4-SC in the period from January 2006 to December 2015 at our hospital were included. The pancreas and major salivary glands were screened, and the initial corticosteroid therapy was given...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28302003/liver-transplant-for-nonhepatocellular-carcinoma-malignancy
#3
Nihan Haberal Reyhan
Liver transplant is now an acceptable and effective treatment for specific nonhepatocellular malignancies. Worldwide, hilar cholangiocarcinoma accounts for 3% of all primary gastrointestinal malignancies and for 10% of primary hepatobiliary malignancies. For patients who have early-stage, unresectable cholangiocarcinoma, liver transplant preceded by neoadjuvant radiotherapy can result in tumor-free margins, accomplish a radical resection, and treat the underlying primary sclerosing cholangitis when present...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28301681/biliary-reconstruction-in-liver-transplant-patients-with-primary-sclerosing-cholangitis-duct-to-duct-or-roux-en-y
#4
Alireza Shamsaeefar, Mohammad Shafiee, Saman Nikeghbalian, Kourosh Kazemi, Mohsenreza Mansorian, Nasrin Motazedian, Bita Geramizadeh, Seyed Ali Malekhosseini
INTRODUCTION: Roux-en-Y choledochojejunostomy and duct-to-duct anastomosis are biliary reconstruction methods for liver transplantation. However, there is a controversy over which method produces better results. We have compared the outcome of duct-to-duct anastomosis vs. Roux-en-Y hepaticojujenostomy in patients with primary sclerosing cholangitis who had undergone liver transplant in Shiraz Organ Transplant Center. MATERIALS: The medical records of 405 PSC patients who had undergone liver transplant from 1996-2015 were reviewed...
March 16, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28300822/human-%C3%AE-defensin-2-in-primary-sclerosing-cholangitis
#5
Cindy Chang, Ana Lleo, Anchasa Kananurak, Fabio Grizzi, Koichi Tsuneyama, Pietro Invernizzi, Charles L Bevins, Christopher L Bowlus
OBJECTIVES: Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts frequently associated with inflammatory bowel disease (IBD), suggesting an important role for the gut-liver axis. Defensins are small (3.5-4.5 kDa) anti-microbial peptides that contribute to innate immunity at mucosal surfaces and have been implicated in IBD. The aim of this study was to investigate copy number variation of the gene (DEFB4) encoding human β-defensin 2 (HBD2) and protein expression of HBD2 in PSC...
March 16, 2017: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/28297794/-research-advances-in-autoimmune-liver-diseases-in-2016
#6
B Li, Q X Wang, X Ma
Autoimmune liver diseases are a group of abnormal autoimmune-mediated inflammatory hepatobiliary injuries, mainly including autoimmune hepatitis(AIH), primary biliary cholangitis(PBC), and primary sclerosing cholangitis (PSC). The diagnosis and treatment of autoimmune liver diseases, an important type of non-viral liver disease, have become a prominent issue in hepatology. In 2016, many new advances have been achieved in the clinical and basic research on autoimmune liver diseases, including the phase 3 clinical trial of obeticholic acid, the proposal of UK-PBC risk score, and the research on gut microbiota associated with PSC...
February 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28293027/genetics-of-primary-sclerosing-cholangitis-and-pathophysiological-implications
#7
REVIEW
Xiaojun Jiang, Tom H Karlsen
Primary sclerosing cholangitis (PSC) is a chronic disease leading to fibrotic scarring of the intrahepatic and extrahepatic bile ducts, causing considerable morbidity and mortality via the development of cholestatic liver cirrhosis, concurrent IBD and a high risk of bile duct cancer. Expectations have been high that genetic studies would determine key factors in PSC pathogenesis to support the development of effective medical therapies. Through the application of genome-wide association studies, a large number of disease susceptibility genes have been identified...
March 15, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28288474/cholangiocellular-carcinoma
#8
Arndt Vogel, Anna Saborowski
BACKGROUND: Cholangiocarcinomas (CCAs) are aggressive malignancies that display features of biliary differentiation. According to their anatomical location, CCAs are commonly classified as intrahepatic and extrahepatic tumors, the latter entity being further subdivided into perihilar CCAs, also termed as Klatskin tumors, and distal tumors. While a majority of CCAs occur sporadically, established risk factors such as liver fluke infestation or primary sclerosing cholangitis exist. SUMMARY: Due to lack of efficient early screening markers, CCAs are frequently diagnosed at an advanced stage when curative surgical resection is not an option...
March 14, 2017: Digestion
https://www.readbyqxmd.com/read/28281846/autoimmune-liver-diseases-and-inflammatory-bowel-diseases-in-children-current-issues-and-future-perspectives
#9
Sabrina Cardile, Tommaso Alterio, Manila Candusso, Andrea Pietrobattista, Daniela Liccardo, Maria Sole Basso, Bronislava Papadatou, Fiammetta Bracci, Daniela Knafelz, Giuliano Torre
Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children...
March 10, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28276816/emerging-treatments-for-primary-sclerosing-cholangitis
#10
Eduardo A Rodriguez, Elizabeth J Carey, Keith D Lindor
Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease that can progress to end-stage liver disease, cirrhosis and cholangiocarcinoma. PSC is an uncommon and highly heterogeneous disease, associated with inflammatory bowel disease and a complex pathophysiology. To date, no medical therapies have proved effective. The only available treatment for end-stage PSC is liver transplant, but recurrence is a significant complication. Areas covered: This review will explore previously tested treatments, discuss current treatment strategies and present viewpoints about future emerging therapies in PSC...
February 22, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28275292/common-controversies-in-management-of-biliary-strictures
#11
EDITORIAL
Mansour A Parsi
Biliary strictures are caused by a heterogeneous group of benign and malignant conditions, each requiring a specific treatment approach. Management of biliary strictures often involves endoscopy either for definite treatment, as a bridge to surgery or for palliative purposes. Endoscopic treatment of various types of biliary strictures is not standardized and there are multiple areas of controversy regarding the best treatment options. These controversies are mainly due to lack of well-designed comparative studies to support a specific therapy...
February 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28274849/patient-age-sex-and-inflammatory-bowel-disease-phenotype-associate-with-course-of-primary-sclerosing-cholangitis
#12
Tobias J Weismüller, Palak J Trivedi, Annika Bergquist, Mohamad Imam, Henrike Lenzen, Cyriel Y Ponsioen, Kristian Holm, Daniel Gotthardt, Martti A Färkkilä, Hanns-Ulrich Marschall, Douglas Thorburn, Rinse K Weersma, Johan Fevery, Tobias Mueller, Olivier Chaouillères, Kornelius Schulze, Konstantinos N Lazaridis, Sven Almer, Stephen P Pereira, Cynthia Levy, Andrew Mason, Sigrid Naess, Christopher L Bowlus, Annarosa Floreani, Emina Halilbasic, Kidist K Yimam, Piotr Milkiewicz, Ulrich Beuers, Dep K Huynh, Albert Pares, Christine N Manser, George N Dalekos, Bertus Eksteen, Pietro Invernizzi, Christoph P Berg, Gabi I Kirchner, Christoph Sarrazin, Vincent Zimmer, Luca Fabris, Felix Braun, Marco Marzioni, Brian D Juran, Karouk Said, Christian Rupp, Kalle Jokelainen, Maria Benito de Valle, Francesca Saffioti, Angela Cheung, Michael Trauner, Christoph Schramm, Roger W Chapman, Tom H Karlsen, Erik Schrumpf, Christian P Strassburg, Michael P Manns, Keith D Lindor, Gideon M Hirschfield, Bettina E Hansen, Kirsten M Boberg
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large, international cohort of patients with PSC. METHODS: We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries...
March 5, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28272287/risk-factors-and-outcomes-of-de-novo-cancers-excluding-nonmelanoma-skin-cancer-after-liver-transplantation-for-primary-sclerosing-cholangitis
#13
Mohamad A Mouchli, Siddharth Singh, Edward V Loftus, Lisa Boardman, Jayant Talwalkar, Charles B Rosen, Julie K Heimbach, Russell H Wiesner, Bashar Hasan, John J Poterucha, Kymberly D Watt
BACKGROUND: Patients with primary sclerosing cholangitis (PSC) may be at higher of risk of malignancy after liver transplantation (LT) compared to other LT recipients. We aimed to determine the cumulative incidence of/risk factors for and long-term cancer-related mortality in patients with PSC after LT. METHODS: All adult patients who underwent LT for PSC without cholangiocarcinoma from 1984-2012, with follow-up through June 2015. We estimated cumulative incidence, risk factors and mortality from de novo malignancies after LT RESULTS: 293 patients were identified (mean age, 47±12 years; 63...
March 8, 2017: Transplantation
https://www.readbyqxmd.com/read/28267887/enhanced-liver-fibrosis-test-predicts-transplant-free-survival-in-primary-sclerosing-cholangitis-a-multi-center-study
#14
Elisabeth M G de Vries, Martti Färkkilä, Piotr Milkiewicz, Johannes R Hov, Bertus Eksteen, Douglas Thorburn, Olivier Chazouillères, Albert Pares, Ståle Nygård, Odd Helge Gilja, Ewa Wunsch, Pietro Invernizzi, Marco Carbone, Francesca Bernuzzi, Kirsten Muri Boberg, Helge Røsjø, William Rosenberg, Ulrich H Beuers, Cyriel Y Ponsioen, Tom H Karlsen, Mette Vesterhus
BACKGROUND AND AIMS: Biomarkers reflecting disease activity and prognosis in primary sclerosing cholangitis (PSC) have not been firmly established. Enhanced Liver Fibrosis (ELF) test was previously reported to predict outcome in PSC. We aimed to validate the prognostic utility of ELF test in an independent, multicenter, retrospective PSC study population. METHODS: We collected serum samples from PSC patients from seven countries. We estimated rates of transplant-free survival by the Kaplan-Meier method, used Cox proportional hazards regression to explore the association between ELF test and clinical outcome and determined prognostic performance of ELF test by computing the area under the receiver operating characteristic (AUC-ROC) curve...
March 7, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28267268/late-choledochojejunostomy-dysfunction-after-liver-transplantation-for-primary-sclerosing-cholangitis
#15
EDITORIAL
D Zamora-Valdes, C B Rosen, J K Heimbach, T Taner
No abstract text is available yet for this article.
December 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/28263100/decreased-serum-dnase1-activity-in-patients-with-autoimmune-liver-diseases
#16
Nikolaos K Gatselis, Aigli G Vakrakou, Kalliopi Zachou, Theodoros Androutsakos, Kalliopi Azariadis, Gregorios Hatzis, Menelaos N Manoussakis, George N Dalekos
Deoxyribonuclease1 (DNase1) is involved in chromatin degradation of apoptotic cells. Its deficiency results in accumulation of self-DNA, which in turn may induce inflammation and autoimmunity. We assessed for the first time serum DNase1-activity in a large consecutive cohort of treatment-naïve patients with autoimmune liver diseases (ALD). DNase1-activity was determined by single radial enzyme-diffusion (SRED) at diagnosis of 224 patients with autoimmune hepatitis (AIH), 249 with primary biliary cirrhosis (PBC) and 36 with primary sclerosing cholangitis (PSC)...
March 2017: Autoimmunity
https://www.readbyqxmd.com/read/28260715/immunoglobulin-g4-related-sclerosing-cholangitis-diagnosed-by-liver-biopsy-a-case-report
#17
Mitsuhiro Furuta, Hiroki Eguchi, Yoshiya Takeda, Kunihiro Fushiki, Takeshi Yasuda, Yuriko Onozawa, Masanobu Katayama, Motoo Tanaka, Tadashi Shigematsu, Masamichi Bamba
Patients with immunoglobulin (Ig) G4-related sclerosing cholangitis typically have a high serum IgG4 level. However, here we describe our experience of a patient with a normal serum IgG4 level for whom the cholangitis was diagnosed by liver biopsy. A 61-year-old male presented with elevated liver enzymes and a normal serum IgG4 level. The hilar, intrahepatic, and upper extrahepatic bile ducts were stenotic, with no evidence of a pancreatic lesion. We therefore performed a liver biopsy to differentiate between cholangiocarcinoma and primary sclerosing cholangitis...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28257308/emerging-pharmacologic-therapies-for-primary-sclerosing-cholangitis
#18
Angela C Cheung, Konstantinos N Lazaridis, Nicholas F LaRusso, Gregory J Gores
PURPOSE OF REVIEW: The only currently approved treatment for primary sclerosing cholangitis (PSC) is liver transplantation, with a median time to transplant of 12-18 years after diagnosis. There are a number of emerging drugs that have the potential to meet this critically unmet need that will be summarized and discussed herein. RECENT FINDINGS: Although the cause of PSC is unknown, there are a number of novel therapeutics under development. These drugs target presumed pathogenic mechanisms largely extrapolated from ex-vivo and in-vivo preclinical models, as well as translational observations...
March 2, 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28255646/mr-imaging-and-mr-cholangiopancreatography-of-cholangiocarcinoma-developing-in-printing-company-workers
#19
Koichi Koyama, Shoji Kubo, Ai Ueki, Taro Shimono, Shigekazu Takemura, Shogo Tanaka, Masahiko Kinoshita, Genya Hamano, Yukio Miki
PURPOSE: To retrospectively investigate magnetic resonance (MR) imaging findings of occupational cholangiocarcinoma (oCC) occurring among workers in printing companies in Japan, compared to those of non-occupational cholangiocarcinoma (nCC), primary sclerosing cholangitis (PSC), and age-matched normal controls (NORs). MATERIALS AND METHODS: Participants comprised 27 consecutive patients (oCC, n = 5; nCC, n = 8; PSC, n = 6; NOR, n = 8) who underwent MR imaging between May 2009 and October 2012...
March 2, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28255489/newly-diagnosed-colonic-adenocarcinoma-the-presenting-sign-in-a-young-woman-with-undiagnosed-crohn-s-disease-in-the-absence-of-primary-sclerosing-cholangitis-and-a-normal-microsatellite-instability-profile
#20
Brett Matthew Lowenthal, Ann M Ponsford Tipps
Ulcerative colitis has long been linked with an increased risk for colonic adenocarcinoma, whereas Crohn's disease (CD) has recently been reported to pose a similar increased risk. We report a 33-year-old healthy female with no family history who presented with abdominal pain and a colon mass. Histopathology revealed a moderately differentiated adenocarcinoma extending through the muscularis propria with metastatic lymph nodes and intact mismatch repair proteins by immunohistochemical expression and gene sequencing...
2017: Case Reports in Pathology
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