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Psc OR primary sclerosing cholangitis

Kilian Friedrich, Carina Baumann, Andreas Wannhoff, Christian Rupp, Arianeb Mehrabi, Karl Heinz Weiss, Daniel N Gotthardt
BACKGROUND AND AIMS: The disease course of primary sclerosing cholangitis (PSC) is variable and difficult to predict. MicroRNA-122 (miR-122) is associated with various liver diseases. We investigated the value of miR-122 as a biomarker for the disease course of PSC. METHODS: We determined serum miR-122 levels in a long-term, prospective cohort of 114 PSC patients and a second validation cohort. RESULTS: Based on miR-122 levels, PSC patients were assigned to low or high level miR-122 groups...
June 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
Juan E Corral, Omar Y Mousa, Murli Krishna, Iris J M Levink, Khela R Pursell, Mohammad Afsh, Paul T Kröner, Denise M Harnois, Herbert C Wolfsen, Michael B Wallace, Frank J Lukens
BACKGROUND:  Volumetric laser endomicroscopy (VLE) provides circumferential images 3 mm into the biliary and pancreatic ducts. We aimed to correlate VLE images with the normal and abnormal microstructure of these ducts. METHODS: Samples from patients undergoing hepatic or pancreatic resection were evaluated. VLE images were collected using a low-profile VLE catheter inserted manually into the biliary and pancreatic ducts ex vivo. Histological correlation was assessed by two unblinded investigators...
June 18, 2018: Endoscopy
Daisuke Imai, Tomoharu Yoshizumi, Kazuhito Sakata, Toru Ikegami, Shinji Itoh, Noboru Harada, Takashi Motomura, Takeo Toshima, Yohei Mano, Yuji Soejima, Yoshihiko Maehara
BACKGROUND: Although risk factors for the long-term mortality of liver transplantation are well described, there is a lack of detailed study regarding these factors for adult living donor liver transplantation (LDLT). METHODS: We retrospectively analyzed 528 adult LDLT recipients in our hospital. The risk factors were analyzed for overall deaths >5 years post-LDLT. RESULTS: Over the 20-year follow-up, 137 patients died. Patient survival at 1, 3, 5, and 10 years post-LDLT was 87...
June 13, 2018: Transplantation
Andrea Tenca, Harri Mustonen, Kati Lind, Eila Lantto, Kaija-Leena Kolho, Sonja Boyd, Johanna Arola, Kalle Jokelainen, Martti Färkkilä
BACKGROUND AND AIMS: Endoscopic retrograde cholangiography (ERCP) has been considered the gold standard for the diagnosis and follow-up of primary sclerosing cholangitis, but it has been replaced by less invasive magnetic resonance imaging and cholangio-pancreatography (MRI-MRCP). However, the role of these two techniques in the evaluation of disease activity and severity needs to be elucidated. METHODS: Patients with primary sclerosing cholangitis (n: 48, male 31, median age: 35...
June 14, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Takashi Hashimoto, Jordan D Rosen, Kristen M Sanders, Gil Yosipovitch
Basophils are blood granulocytes and normally constitute less than 1% of blood peripheral leukocytes. Basophils share some morphological and functional similarities with mast cells, and basophils were once regarded as redundant and negligible circulating mast cells. However, recent studies reveal the indispensable roles of basophils in various diseases, including allergic and pruritic diseases. Basophils may be involved in itch through the mediation of a Th2 immune response, interaction with other cells in the skin, and secretion of a wide variety of itch-related mediators, e...
June 12, 2018: Experimental Dermatology
Mai Sedki, Cynthia Levy
PURPOSE OF REVIEW: Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease for which specific medical therapy is not available. The goals of treatment are primarily early detection and management of complications. In this review, we discuss novel therapies under evaluation and provide the foundation for surveillance strategies. RECENT FINDINGS: Drugs under investigation include norursodeoxycholic acid, nuclear receptor agonists, anti-fibrotics, antibiotics, and anti-inflammatory drugs...
June 9, 2018: Current Gastroenterology Reports
James H Tabibian, Todd H Baron
Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by intra- and/or extra-hepatic bile duct inflammation and fibro-obliteration, which ultimately leads to biliary cirrhosis and related sequelae, including development of hepatobiliary and colorectal carcinomata. PSC can be diagnosed at essentially any age and carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma...
June 8, 2018: Expert Review of Gastroenterology & Hepatology
P J Trivedi, J Reece, R W Laing, E Slaney, R Cooney, B K Gunson, S K Kamarajah, T Pinkney, F Thompson, P Muiesan, A Schlegel, G M Hirschfield, T Iqbal, J Ferguson
BACKGROUND: Liver transplantation is the only life-extending intervention for primary sclerosing cholangitis (PSC). Given the co-existence with colitis, patients may also require colectomy; a factor potentially conferring improved post-transplant outcomes. AIM: To determine the impact of restorative surgery via ileal pouch-anal anastomosis (IPAA) vs retaining an end ileostomy on liver-related outcomes post-transplantation. METHODS: Graft survival was evaluated across a prospectively accrued transplant database, stratified according to colectomy status and type...
June 8, 2018: Alimentary Pharmacology & Therapeutics
Karn Wijarnpreecha, Panadeekarn Panjawatanan, Omar Y Mousa, Wisit Cheungpasitporn, Surakit Pungpapong, Patompong Ungprasert
Background/Objectives: Studies have suggested that smokers may have a lower risk of primary sclerosing cholangitis (PSC) although the results have been inconsistent. This systematic review and meta-analysis was conducted to summarize all available data to better characterize this association. Methods: A comprehensive literature review was conducted using Medline and Embase databases through January 2018 to identify all studies that compared the risk of PSC among current/former smokers versus nonsmokers...
May 2018: United European Gastroenterology Journal
Sumera Rizvi, John Eaton, Ju Dong Yang, Vinay Chandrasekhara, Gregory J Gores
The diagnosis of malignant biliary strictures remains problematic, especially in the perihilar region and in primary sclerosing cholangitis (PSC). Conventional cytology obtained during endoscopic retrograde cholangiography (ERC)-guided brushings of biliary strictures is suboptimal due to limited sensitivity, albeit it remains the gold standard with a high specificity. Emerging technologies are being developed and validated to address this pressing unmet patient need. Such technologies include enhanced visualization of the biliary tree by cholangioscopy, intraductal ultrasound, and confocal laser endomicroscopy...
May 2018: Seminars in Liver Disease
Ravish Parekh, Ahmed Abdulhamid, Sheri Trudeau, Nirmal Kaur
Background: Outcome data regarding the use of tumor necrosis factor alpha inhibitors (anti-TNF α ) in patients with inflammatory bowel disease (IBD) after liver transplant (LT) for primary sclerosing cholangitis (PSC) are scant. Methods: We performed a retrospective chart review to investigate outcomes among a series of post-liver-transplant PSC/IBD patients receiving anti-TNF α therapy at Henry Ford Health System ((HFHS), Detroit, MI). Results: A total of five patients were treated with anti-TNF α agents for IBD after LT for PSC from 1993 through 2015...
2018: Case Reports in Gastrointestinal Medicine
Dennis R Petersen, David J Orlicky, James R Roede, Colin T Shearn
OBJECTIVE: Primary Sclerosing Cholangitis (PSC) is a severe cholestatic liver disease characterized by progressive peri-biliary tract inflammation, elevated oxidative stress and hepatocellular injury. A hallmark of PSC patients is the concurrent diagnosis of Inflammatory Bowel Disease occurring in approximately 70%-80% of PSC patients (PSC/IBD). We previously reported dysregulation of key anti-oxidant pathways in PSC/IBD. The objective of this study was to expand previous data by examining the abundance of thioredoxins (Trx) in PSC/IBD...
May 29, 2018: Experimental and Molecular Pathology
M J Nielsen, D Thorburn, D J Leeming, J R Hov, S Nygård, B Moum, F Saffioti, O H Gilja, K M Boberg, G Mazza, H Røsjø, M Pinzani, T H Karlsen, M A Karsdal, M Vesterhus
BACKGROUND: Primary sclerosing cholangitis is a progressive liver disease with a remarkably variable course. Biomarkers of disease activity or prognostic models predicting outcome at an individual level are currently not established. AIM: To evaluate the prognostic utility of four biomarkers of basement membrane and interstitial extracellular matrix remodeling in patients with primary sclerosing cholangitis. METHODS: Serum samples were available from 138 large-duct primary sclerosing cholangitis patients (of which 102 [74%] with IBD) recruited 2008-2012 and 52 ulcerative colitis patients (controls)...
May 30, 2018: Alimentary Pharmacology & Therapeutics
Timon E Adolph, Christoph Grander, Alexander R Moschen, Herbert Tilg
The intestinal and hepatobiliary tract exhibits host-specific commensal colonization. The resident microbiota has emerged as a key player in intestinal and hepatic diseases. Alcoholic and nonalcoholic fatty liver diseases (ALD/NAFLD), primary sclerosing cholangitis (PSC), liver cirrhosis, and some of their clinical complications, such as hepatic encephalopathy (HE), have been linked to a microbial signature, as also observed for severe liver inflammation in alcoholic hepatitis. In turn, the liver impacts, and communicates with, the microbiota through hepatic mediators, such as bile acids or inflammatory signals...
May 26, 2018: Trends in Immunology
C S Tse, E V Loftus, L E Raffals, A A Gossard, A L Lightner
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic biliary disease associated with inflammatory bowel disease (IBD) with no known cure. AIM: To evaluate the effect of biological therapies on PSC progression in IBD patients. METHODS: We performed a retrospective cohort study of 88 cases (75 unique patients with 12 patients treated >1 biologics) of IBD (48 ulcerative colitis, 24 Crohn's disease and 3 indeterminate colitis) with concomitant PSC who received biological therapy (42 infliximab, 19 adalimumab, 27 vedolizumab) between June 2002 and October 2017...
May 28, 2018: Alimentary Pharmacology & Therapeutics
Steven Werlin, Virginie Scotet, Kevin Uguen, Marie-Pierre Audrezet, Michael Cohen, Yasmin Yaakov, Rifaat Safadi, Yaron Ilan, Fred Konikoff, Eitan Galun, Meir Mizrahi, Mordechai Slae, Shirley Sayag, Malena Cohen-Cymberknoh, Michael Wilschanski, Claude Ferec
BACKGROUND: The etiology of primary sclerosing cholangitis (PSC) is unknown. PSC and Cystic Fibrosis related liver disease have common features: chronic inflammation, biliary damage and similar cholangiographic findings. It is unknown whether or not PSC is related to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. We hypothesize that a sub-group of PSC patients may be a "single-organ" presentation of CF. METHODS: Patients with PSC underwent nasal potential difference (NPD) measurement, sweat chloride measurement and complete CFTR sequencing by new generation sequencing...
May 25, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Wei Liu, Qiaofei Liu, Wenqin Wang, Penghui Wang, Jieming Chen, Tao Hong, Ning Zhang, Binglu Li, Qiang Qu, Xiaodong He
Background: Obstructive jaundice is one of the most common symptoms which can be caused by both malignant and benign hepato-biliary-pancreatic diseases. The differences and the differential diagnostic roles of the serum CA19-9, total bilirubin (TBIL) and especially, the ratio of CA19-9 to TBIL in these patients have not been well elucidated. This study compared the differences and the differential diagnostic roles of the increase-folds of the serum CA19-9, TBIL and the ratio of increase-folds of CA19-9 to increase-folds of TBIL in 508 cases of malignant (MOJ) and benign (BOJ) obstructive jaundice patients...
2018: Journal of Cancer
Cyriel Y Ponsioen, Urban Arnelo, Annika Bergquist, Erik A Rauws, Vemund Paulsen, Paolo Cantú, Ilaria Parzanese, Elisabeth M De Vries, Kim N Van Munster, Karouk Said, Olivier Chazouillères, Benoit Desaint, Astrid Kemgang, Martti Färkkilä, Schalk Van der Merwe, Werner Van Steenbergen, Hanns-Ulrich Marschall, Per-Ove Stotzer, Douglas Thorburn, Stephen P Pereira, Lars Aabakken
BACKGROUND & AIMS: Dominant strictures occur in approximately 50% of patients with primary sclerosing cholangitis (PSC). Short-term stents have been reported to produce longer resolution of dominant strictures than single-balloon dilatation. We performed a prospective study to compare the efficacy and safety of balloon dilatation vs short-term stents in patients with non-end stage PSC. METHODS: We performed an open-label trial of patients with PSC undergoing therapeutic endoscopic retrograde cholangiopancreatography (ERCP) at 9 tertiary-care centers in Europe, from July 2011 through April 2016...
May 24, 2018: Gastroenterology
Maria Eugenia Guicciardi, Christy E Trussoni, Anuradha Krishnan, Steven F Bronk, Maria J Lorenzo Pisarello, Steven P O'Hara, Patrick L Splinter, Yandong Gao, Pamela Vig, Alexander Revzin, Nicholas F LaRusso, Gregory J Gores
BACKGROUND & AIMS: Macrophages contribute to liver disease, but their role in cholestatic liver injury, including primary sclerosing cholangitis (PSC), is unclear. We tested the hypothesis that macrophages contribute to the pathogenesis of, and are therapeutic targets for, PSC. METHODS: Immune cell profile, hepatic macrophage number, localization and polarization, fibrosis, and serum markers of liver injury and cholestasis were measured in an acute (intrabiliary injection of the IAP antagonist BV6) and chronic (Mdr2-/- mice) mouse model of sclerosing cholangitis (SC)...
May 23, 2018: Journal of Hepatology
Vanessa Pataia, Georgia Papacleovoulou, Vanya Nikolova, Anne-Maj Samuelsson, Stephanie Chambers, Eugene Jansen, Paul D Taylor, Lucilla Poston, Catherine Williamson
BACKGROUND: Obesity is a heterogeneous phenotype and risk associations to non-communicable diseases such as cardiovascular disease and type 2 diabetes are influenced by several factors. The paternal metabolic status at the time of conception influences offspring susceptibility to developing obesity and adiposity-associated cardiometabolic disease. Cholestatic liver diseases are characterized by raised circulating serum bile acid levels and dyslipidemia, and are commonly treated with ursodeoxycholic acid (UDCA)...
May 24, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
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