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Psc OR primary sclerosing cholangitis

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https://www.readbyqxmd.com/read/29452711/primary-sclerosing-cholangitis
#1
REVIEW
Jessica K Dyson, Ulrich Beuers, David E J Jones, Ansgar W Lohse, Mark Hudson
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography...
February 13, 2018: Lancet
https://www.readbyqxmd.com/read/29449577/neutralization-of-il-15-abrogates-experimental-immune-mediated-cholangitis-in-diet-induced-obese-mice
#2
José L Reyes, Danielle T Vannan, Tina Vo, Aliya Gulamhusein, Paul L Beck, Raylene A Reimer, Bertus Eksteen
Obesity is a global epidemic affecting chronic inflammatory diseases. Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that can occur as an extraintestinal manifestation of inflammatory bowel disease (IBD). Previously we reported that patients with PSC who are obese have a higher risk of advanced liver disease. Currently it is unknown how obesity accelerates or worsens PSC. We evaluated the progression of PSC in an antigen-driven cholangitis mouse model of diet-induced obesity. Obesity was induced in our murine model of immune-mediated cholangitis (OVAbil)...
February 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29435321/the-gut-microbiota-bile-acids-and-their-correlation-in-primary-sclerosing-cholangitis-associated-with-inflammatory-bowel-disease
#3
J Torres, C Palmela, H Brito, X Bao, H Ruiqi, P Moura-Santos, J Pereira da Silva, A Oliveira, C Vieira, K Perez, S H Itzkowitz, J F Colombel, L Humbert, D Rainteau, M Cravo, C M Rodrigues, J Hu
Background: Patients with primary sclerosing cholangitis associated with inflammatory bowel disease (PSC-IBD) have a very high risk of developing colorectal neoplasia. Alterations in the gut microbiota and/or gut bile acids could account for the increase in this risk. However, no studies have yet investigated the net result of cholestasis and a potentially altered bile acid pool interacting with a dysbiotic gut flora in the inflamed colon of PSC-IBD. Aim: The aim of this study was to compare the gut microbiota and stool bile acid profiles, as well as and their correlation in patients with PSC-IBD and inflammatory bowel disease alone...
February 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29432923/peribiliary-cysts-presenting-as-primary-sclerosing-cholangitis
#4
Aravind Gokul Tamilarasan, Jessica Yang, Alice Unah Lee
No abstract text is available yet for this article.
February 9, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29430861/clinical-course-of-type-1-autoimmune-pancreatitis-patients-without-steroid-treatment-a-japanese-multicenter-study-of-97-patients
#5
Kensuke Kubota, Terumi Kamisawa, Kenji Hirano, Yoshiki Hirooka, Kazushige Uchida, Tsukasa Ikeura, Hideyuki Shiomi, Hirotaka Ohara, Kyoko Shimizu, Norikazu Arakura, Atsushi Kanno, Junichi Sakagami, Takao Itoi, Tetsuhide Ito, Toshiharu Ueki, Takayoshi Nishino, Kazuo Inui, Nobumasa Mizuno, Hitoshi Yoshida, Masanori Sugiyama, Eisuke Iwasaki, Atsushi Irisawa, Kazuichi Okazaki, Shigeyuki Kawa, Toru Shimosegawa, Yoshifumi Takeyama, Tsutomu Chiba
BACKGROUND: Sporadic autoimmune pancreatitis (AIP) cases showing remission without steroid treatment have been reported, however, the clinical course of these patients has not been clarified. AIM: This study sought to clarify the clinical course in AIP patients with hesitation for steroid treatment. METHODS: We collected clinical data for AIP patients from high-volume centers in Japan. Data for AIP patients with and those without steroid treatment (steroid treatment vs...
February 12, 2018: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/29428100/clinical-analysis-of-liver-transplantation-in-autoimmune-liver-diseases
#6
Cheng-Peng Zhong, Zhi-Feng Xi, Qiang Xia
BACKGROUND: Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation. METHODS: The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively...
February 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29425397/cholangiocyte-derived-exosomal-long-noncoding-rna-h19-promotes-cholestatic-liver-injury-in-mouse-and-human
#7
Xiaojiaoyang Li, Runping Liu, Zhiming Huang, Emily C Gurley, Xuan Wang, Juan Wang, Hongliang He, Hu Yang, Guanhua Lai, Luyong Zhang, Jasmohan S Bajaj, Melanie White, William M Pandak, Phillip B Hylemon, Huiping Zhou
Cholestatic liver injury is an important clinical problem with limited understanding of disease pathologies. Exosomes are small extracellular vesicles released by a variety of cells including cholangiocytes. Exosome-mediated cell-cell communication can modulate various cellular functions by transferring a variety of intracellular components to target cells. Our recent studies indicate that the long non-coding RNA H19 is mainly expressed in cholangiocytes and its aberrant expression is associated with significant down-regulation of small heterodimer partner (SHP) in hepatocytes and cholestatic liver injury in multidrug resistance 2 knockout (Mdr2-/- ) mice...
February 9, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29411404/review-article-the-evidence-that-vancomycin-is-a-therapeutic-option-for-primary-sclerosing-cholangitis
#8
REVIEW
J L Damman, E A Rodriguez, A H Ali, C W Buness, K L Cox, E J Carey, K D Lindor
BACKGROUND AND AIMS: PSC is an autoimmune biliary inflammatory disorder that is often associated with inflammatory bowel disease (IBD), with 50%-75% of patients with PSC having coexisting IBD, most commonly ulcerative colitis. Currently, no medical therapies have been shown to improve the disease course or slow its progression. However, ongoing research has resulted in a growing interest in the use of antibiotics for treatment of PSC, of which vancomycin is the most studied. In this review, we summarise the current evidence on the use of vancomycin in PSC and comment on future research areas of interest...
February 7, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29404503/the-ectonucleotidase-entpd1-cd39-limits-biliary-injury-and-fibrosis-in-mouse-models-of-sclerosing-cholangitis
#9
Zhen-Wei Peng, Sonja Rothweiler, Guangyan Wei, Naoki Ikenaga, Susan B Liu, Deanna Y Sverdlov, Kahini A Vaid, Maria Serena Longhi, Ming Kuang, Simon C Robson, Yury V Popov
The pathogenesis of primary sclerosing cholangitis (PSC) and the mechanistic link to inflammatory bowel disease remain ill-defined. Ectonucleoside triphosphate diphosphohydrolase-1 (ENTPD1)/clusters of differentiation (CD) 39, the dominant purinergic ecto-enzyme, modulates intestinal inflammation. Here, we have explored the role of CD39 in biliary injury and fibrosis. The impact of CD39 deletion on disease severity was studied in multidrug resistance protein 2 (Mdr2)-/- and 3,5-diethoxycarbonyl-1,4-dihydrocollidine mouse models of sclerosing cholangitis and biliary fibrosis...
November 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29378311/high-risk-of-advanced-colorectal-neoplasia-in-patients-with-primary-sclerosing-cholangitis-associated-with-inflammatory-bowel-disease
#10
Shailja C Shah, Joren R Ten Hove, Daniel Castaneda, Carolina Palmela, Erik Mooiweer, Jean-Frédéric Colombel, Noam Harpaz, Thomas A Ullman, Ad A van Bodegraven, Jeroen M Jansen, Nofel Mahmmod, Andrea E van der Meulen-de Jong, Cyriel Y Ponsioen, Christine J van der Woude, Bas Oldenburg, Steven H Itzkowitz, Joana Torres
BACKGROUND & AIMS: Patients with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC, termed PSC-IBD) are at increased risk for colorectal cancer (CRC), but their risk following a diagnosis of low-grade dysplasia (LGD) is not well described. We aimed to determine the rate of advanced colorectal neoplasia (aCRN), defined as high-grade dysplasia and/or colorectal cancer, following a diagnosis of indefinite dysplasia or LGD in this population. METHODS: We performed a retrospective, longitudinal study of 1911 patients with colonic IBD (293 with PSC and 1618 without PSC) who underwent more than 2 surveillance colonoscopies from 2000 through 2015 in the Netherlands or the United States (9265 patient-years of follow up)...
January 25, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29378308/symptoms-do-not-correlate-with-findings-from-colonoscopy-in-children-with-inflammatory-bowel-disease-and-primary-sclerosing-cholangitis
#11
Amanda Ricciuto, Jennifer Fish, Nicholas Carman, Thomas D Walters, Peter C Church, Bettina E Hansen, Eileen Crowley, Iram Siddiqui, Geoffrey C Nguyen, Binita M Kamath, Anne M Griffiths
BACKGROUND & AIMS: Approximately 75% of children with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD). IBD in patients with PSC (PSC-IBD) often has a unique phenotype, including a mild clinical course, yet it is associated with an increased risk of colorectal cancer compared to colonic IBD without PSC. We investigated whether subclinical endoscopic and histologic inflammation could account for the increased risk of colorectal cancer in patients with PSC-IBD, and whether these patients have increased fecal levels of calprotectin, a marker of inflammation...
January 25, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29377876/gender-race-and-disease-etiology-predict-de-novo-malignancy-risk-following-liver-transplantation-insights-for-future-individualized-cancer-screening-guidance
#12
Mamatha Bhat, Kristin Mara, Ross Dierkhising, Kymberly D Watt
BACKGROUND: Malignancy after liver transplant (LT) is a leading cause of mortality, but data is limited. The aim of this study was to identify patients at higher risk for de novo malignancies after LT in a large multicenter database. METHODS: The Scientific Registry of Transplant Recipients database comprising all 108,412 liver transplant recipients across the U.S. between 1987 and March 2015 was analyzed with a median follow-up of 6.95 years. Potential risk factors for malignancies after LT were assessed using Cox regression analysis for the outcome of time to first malignancy...
January 26, 2018: Transplantation
https://www.readbyqxmd.com/read/29377874/successful-sequential-liver-and-haematopoietic-stem-cell-transplantation-in-a-child-with-cd40-ligand-deficiency-and-cryptosporidium-induced-liver-cirrhosis
#13
Paola Quarello, Francesco Tandoi, Francesca Carraro, Elena Vassallo, Michele Pinon, Renato Romagnoli, Ezio David, Dominic Dell Olio, Mauro Salizzoni, Franca Fagioli, Pier Luigi Calvo
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is curative in patients with primary immunodeficiencies. However, pre-HSCT conditioning entails unacceptably high risks if the liver is compromised. The presence of a recurrent opportunistic infection affecting the biliary tree and determining liver cirrhosis with portal hypertension posed particular decisional difficulties in a seven-year-old child with X-linked CD40-ligand deficiency. We aim at adding to the scanty experience available on such rare cases, as successful management with sequential liver transplantation (LT) and HSCT has been reported in detail only in one young adult to date...
January 26, 2018: Transplantation
https://www.readbyqxmd.com/read/29377235/vedolizumab-in-patients-with-concurrent-primary-sclerosing-cholangitis-and-inflammatory-bowel-disease-does-not-improve-liver-biochemistry-but-is-safe-and-effective-for-the-bowel-disease
#14
B Christensen, D Micic, P R Gibson, A Yarur, E Bellaguarda, P Corsello, J N Gaetano, J Kinnucan, V L Rao, S Reddy, S Singh, J Pekow, D T Rubin
BACKGROUND: Blocking of lymphocyte trafficking to bile ducts is a potential mechanism to alter the disease course of patients with primary sclerosing cholangitis (PSC). AIM: To describe the effect of the α4 β7 integrin antibody, vedolizumab, on liver biochemistry and disease activity in patients with PSC and inflammatory bowel disease (IBD). METHODS: This is a retrospective multi-centre study of adult patients with a diagnosis of both IBD and PSC...
January 29, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29370346/reduced-risk-of-inflammatory-bowel-disease-associated-colorectal-neoplasia-with-use-of-thiopurines-a-systematic-review-and-meta-analysis
#15
Zhehui Zhu, Zubing Mei, Yuegui Guo, Guanghui Wang, Tingyu Wu, Ximao Cui, Zhenyu Huang, Yilian Zhu, Dongpeng Wen, Jinlve Song, Hailan He, Weimin Xu, Long Cui, Chenying Liu
Background & Aims: The association between thiopurines and colorectal neoplasia risk remains controversial in inflammatory bowel disease (IBD) patients. We performed a systematic review and meta-analysis examining this association. Methods: A comprehensive search of the PubMed, EMBASE and Cochrane Library databases was performed to identify relevant literature. Random-effects models were applied to calculate the pooled odds ratio (OR) and relative risk (RR) with corresponding 95% confidence intervals (CIs) among case-control and cohort studies, respectively...
January 23, 2018: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/29361103/reviewing-the-risk-of-colorectal-cancer-in-inflammatory-bowel-disease-after-liver-trasnplantation-for-primary-sclerosing-cholangitis
#16
Bhavana Bhagya Rao, Bret Lashner, Kris V Kowdley
The presence of concomitant primary sclerosing cholangitis (PSC) with inflammatory bowel disease (IBD) represents a distinct disease phenotype that carries a higher risk of colorectal cancer (CRC) than the average IBD patient. Given that liver transplantation (LT) is the only treatment that offers a survival benefit in PSC patients with hepatic dysfunction, management decisions in IBD patients' post-LT for PSC are frequently encountered. One such consideration is the risk of CRC in this immunosuppressed cohort...
January 18, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29360691/age-at-disease-onset-of-inflammatory-bowel-disease-is-associated-with-later-extraintestinal-manifestations-and-complications
#17
Denise Herzog, Nicolas Fournier, Patrick Buehr, Vanessa Rueger, Rebekka Koller, Klaas Heyland, Andreas Nydegger, Johannes Spalinger, Susanne Schibli, Laetitia-Marie Petit, Christian P Braegger
INTRODUCTION: A small but increasing number of patients with inflammatory bowel disease are diagnosed during childhood or adolescence, and disease distribution and severity at onset vary according to the age at diagnosis. Clinical factors present at the time of diagnosis can be predictive of the disease course. AIM: The aim of this study was to characterize disease behavior and the cumulative complications and extraintestinal manifestations 10 years after the diagnosis and to assess their association with age at diagnosis...
January 22, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29356767/lack-of-correlation-of-liver-tests-with-fibrosis-stage-at-diagnosis-in-pediatric-primary-sclerosing-cholangitis
#18
Jillian M Cotter, Lorna P Browne, Kelley E Capocelli, Annette McCoy, Cara L Mack
OBJECTIVES: The aims of this study were to characterize pediatric primary sclerosing cholangitis (PSC) at a regional referral-based institution, including scoring of biliary stricturing and liver fibrosis and correlation analyses of scores with serum liver tests, to identify biomarkers of disease severity. METHODS: A retrospective review of 39 PSC subjects was performed, with collection of demographic and outcomes data. Magnetic resonance cholangiopancreaticogram (MRCP) and liver biopsies were re-reviewed and scores of stricturing and fibrosis were correlated with serum liver tests...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29333790/crohn-s-disease-behavior-and-location-is-altered-when-associated-with-primary-sclerosing-cholangitis
#19
Oren Iny, Henit Yanai, Shay Matalon, Erwin Santo, Oren Shibolet, Iris Dotan, Nitsan Maharshak
BACKGROUND: Up to 3.4% of Crohn's disease (CD) patients will be diagnosed with concomitant primary sclerosing cholangitis (PSC). Despite the worldwide increase incidence of CD, data on the clinical characteristics of PSC-CD patients are scarce. OBJECTIVES: To clinically characterize CD in patients who have concomitant PSC. METHODS: A retrospective case-control analysis was conducted with 18 CD patients with concomitant PSC who attended the Inflammatory Bowel Disease Center at the Tel Aviv Sourasky Medical Center between 2011-2014 (PSC-CD patients)...
January 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29324588/both-%C3%AE-1-antitrypsin-z-phenotypes-and-low-caeruloplasmin-levels-are-over-represented-in-alcohol-and-nonalcoholic-fatty-liver-disease-cirrhotic-patients-undergoing-liver-transplant-in-ireland
#20
El-Gaily A El-Rayah, Patrick J Twomey, Eleanor M Wallace, Peter A McCormick
OBJECTIVES: Alcoholic liver disease and nonalcoholic fatty liver disease (NAFLD) are steatotic liver diseases and major causes of cirrhosis. Only a minority of patients with risk factors develop cirrhosis and genetic cofactors may be important in pathogenesis. Mutations in the Wilson's and α-1-antitrypsin genes are not uncommon and we speculated that they may act as cofactors. METHODS: We investigated α-1-antitrypsin phenotyes and caeruloplasmin levels in patients undergoing elective liver transplantation...
January 10, 2018: European Journal of Gastroenterology & Hepatology
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