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Psc OR primary sclerosing cholangitis

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https://www.readbyqxmd.com/read/27921168/the-2016-diagnostic-criteria-for-primary-sclerosing-cholangitis
#1
Takahiro Nakazawa, Kenji Notohara, Susumu Tazuma, Atsushi Tanaka, Hiroyuki Isayama, Toshio Tsuyuguchi, Toshiyuki Mori, Hajime Takikawa
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan...
December 5, 2016: Journal of Gastroenterology
https://www.readbyqxmd.com/read/27921051/systemic-mastocytosis-causing-refractory-pruritus-in-a-liver-disease-patient
#2
Naga S Addepally, Jagpal S Klair, Mohit Girotra, Johnny Jones, Farshad Aduli
Systemic mastocytosis (SM) results from clonal, neoplastic proliferation of abnormal mast cells. Patients become susceptible to itching, urticaria, and anaphylactic shock, which occurs due to histamine release from mast cells. SM may coexist alongside other systemic diseases, thus confounding the overall clinical presentation. We discuss a 23-year-old woman with refractory pruritus, which was initially attributed to primary sclerosing cholangitis but had a nonresponse to antihistaminics, ursodiol, and cholestyramine...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27916678/incidence-risk-factors-and-outcomes-of-colorectal-cancer-in-patients-with-ulcerative-colitis-with-low-grade-dysplasia-a-systematic-review-and-meta-analysis
#3
REVIEW
Mathurin Fumery, Parambir S Dulai, Samir Gupta, Larry J Prokop, Sonia Ramamoorthy, William J Sandborn, Siddharth Singh
BACKGROUND & AIMS: Little is known about outcomes of patients with ulcerative colitis with low-grade dysplasia (UC-LGD). We estimated the incidence of and risk factors for progression to colorectal cancer (CRC) in cohorts of patients with UC-LGD who underwent surveillance (surveillance cohort), and the prevalence of dysplasia-related findings among patients who underwent colectomy for UC-LGD (surgical cohort). METHODS: We performed a systematic literature review through June 1, 2016 to identify cohort studies of adults with UC-LGD...
December 1, 2016: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/27912077/nlrp3-activation-induces-il-18-synthesis-and-affects-the-epithelial-barrier-function-in-reactive-cholangiocytes
#4
Luca Maroni, Laura Agostinelli, Stefania Saccomanno, Claudio Pinto, Debora Giordano, Chiara Rychlicki, Samuele De Minicis, Luciano Trozzi, Jesus M Banales, Espen Melum, Tom H Karlsen, Antonio Benedetti, Gianluca Svegliati Baroni, Marco Marzioni
Microbial products are thought to influence the progression of cholangiopathies, in particular primary sclerosing cholangitis (PSC). Inflammasomes are molecular platforms that respond to microbial products through the synthesis of proinflammatory cytokines. We investigated the role of inflammasome activation in cholangiocyte response to injury. Nucleotide-binding oligomerization domain (NOD)-like receptor family, pyrin domain-containing protein 3 (Nlrp3) expression was tested in cholangiocytes of normal and cholestatic livers...
November 29, 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27908204/clinical-implications-of-serial-versus-isolated-biliary-fluorescence-in-situ-hybridization-fish-polysomy-in-primary-sclerosing-cholangitis
#5
Kevin P Quinn, James H Tabibian, Keith D Lindor
BACKGROUND: Cholangiocarcinoma (CCA) is a leading cause of death in patients with primary sclerosing cholangitis (PSC). Biliary polysomy detected by fluorescence in situ hybridization (FISH) helps to identify patients with early CCA, but not all PSC patients with polysomy develop CCA. Here, we examined the features and clinical outcomes of PSC patients with serial versus isolated polysomy. METHODS: All patients with PSC who underwent ≥1 endoscopic retrograde cholangiography (ERC) with FISH testing at Mayo Clinic, Rochester from 2008-2011 were identified...
December 1, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27904625/an-intriguing-case-of-gallstone-ileus-after-hepaticojejunostomy-caused-by-a-stone-on-a-suture
#6
Mahir Gachabayov, Petr Mityushin
Gallstone ileus (GI) is a mechanical obstruction of small or large bowel caused by gallstone passed to the intestinal lumen through spontaneous or postoperative biliodigestive fistula. A 42-year-old female patient was admitted with the clinical presentation of small bowel obstruction. She underwent hepaticojejunostomy 4 years prior to admission for primary sclerosing cholangitis. Barium meal follows through revealed Rigler's triad. The patient underwent laparotomy which revealed GI. A "stone on a suture" was removed through enterotomy...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/27891750/epidemiology-and-outcomes-of-primary-sclerosing-cholangitis-with-and-without-inflammatory-bowel-disease-in-an-australian-cohort
#7
Ken Liu, Ruoxi Wang, Viraj Kariyawasam, Mark Wells, Simone I Strasser, Geoff McCaughan, Crispin Corte, Rupert W Leong
BACKGROUND&AIMS: Epidemiological data on primary sclerosing cholangitis (PSC) outside the Northern hemisphere is limited. Similarly, the impact of inflammatory bowel disease (IBD) on PSC outcomes remains unclear. We aimed to study the epidemiology and outcomes of PSC patients with and without IBD in an Australian cohort. METHODS: We retrospectively studied PSC patients attending two tertiary referral hospitals over 20years. Diagnosis of PSC was made according to international guidelines by positive cholangiography and/or liver biopsy (for small duct PSC) with supporting clinical and laboratory evidence RESULTS: 208 PSC patients (61% male) were studied (2,271patient-years follow-up)...
November 28, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#8
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27880989/validation-of-the-prognostic-value-of-histologic-scoring-systems-in-primary-sclerosing-cholangitis-an-international-cohort-study
#9
Elisabeth M G De Vries, Manon de Krijger, Martti Farkkila, Johanna Arola, Peter Schirmacher, Daniel Gotthardt, Benjamin Goeppert, Palak J Trivedi, Gideon M Hirschfield, Henriette Ytting, Ben Vainer, Henk R van Buuren, Katharina Biermann, Maren H Harms, Olivier Chazouilleres, Dominique Wendum, Astrid Donald Kemgang, Roger W Chapman, Lai Mun Wang, Kate D Williamson, Annette S H Gouw, Valerie Paradis, Christine Sempoux, Ulrich Beuers, Stefan Hubscher, Joanne Verheij, Cyriel Ponsioen
: Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic utility and reproducibility, across a multicenter PSC cohort. Liver biopsies from PSC patients were collected across 7 European centers. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems...
November 23, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27876037/the-efficacy-of-adalimumab-in-psoriatic-arthritis-concomitant-to-overlapping-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis-a-case-report
#10
Teresa Del Ross, Amelia Ruffatti, Annarosa Floreani, Ariela Hoxha, Leonardo Punzi
BACKGROUND: The overlap syndrome of primary biliary cholangitis (formerly called primary biliary cirrhosis) and primary sclerosing cholangitis is an extremely rare condition that has never been described in association with other immune-mediated diseases, including psoriatic arthritis. While treatment with anti-Tumour Necrosis Factor-alpha (TNF-α) agents has proved to be effective in inflammatory arthropathies such as psoriatic arthritis, they have been employed in only a limited number of patients with autoimmune hepatitis, and their effectiveness is unclear...
November 22, 2016: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/27875796/development-of-extensive-inferior-vena-cava-thrombosis-due-to-the-ligation-of-a-large-mesenteric-caval-shunt-during-liver-transplantation-a-case-report
#11
Hideya Kamei, Yasuharu Onishi, Masatoshi Ishigami, Yoji Ishizu, Kojiro Suzuki, Yasuhiro Ogura
INSTRUCTION: Inferior vena cava (IVC) thrombosis can be a life-threatening complication after liver transplantation (LT). Although this complication is usually related to technical problems associated with vascular anastomosis, we report a case of IVC thrombosis which developed from a ligated large mesenteric-caval shunt. PRESENTATION OF CASE: A 35-year-old man underwent LT from a brain-dead donor for primary sclerosing cholangitis. Enhanced computed tomography (CT) before LT showed a huge collateral vessel of the inferior mesenteric vein (IMV) draining into the infra-renal IVC directly...
November 15, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27874998/factors-predictive-of-the-efficacy-of-bezafibrate-therapy-in-patients-with-primary-sclerosing-cholangitis
#12
Suguru Mizuno, Hiroyuki Isayama, Kenji Hirano, Takeo Watanabe, Naminatsu Takahara, Hirofumi Kogure, Saburo Matsubara, Yousuke Nakai, Minoru Tada, Kazuhiko Koike
AIM: Primary sclerosing cholangitis (PSC) is a rare cholestatic disease. We previously reported the effects of bezafibrate on elevated hepatobiliary enzyme levels in patients with this disease both retrospectively and prospectively. In this study, we assessed factors predictive of bezafibrate efficacy. METHODS: Twenty-five patients with PSC, who underwent bezafibrate therapy (400 mg per day) from November 2006 to June 2015, were evaluated. Treatment was judged as being effective if the levels of all of the hepatobiliary enzymes decreased after 12 weeks...
November 22, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/27855457/-cholestatic-liver-disease
#13
Christine Pichler, Tobias Boettler, Robert Thimme
Common cholestatic liver diseases are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both diseases are considered autoimmune disorders; however, the precise pathogenesis remains elusive. Patients usually show no symptoms or present with pruritus and fatigue. High alkaline phosphatase and the presence of antimitochondrial antibodies are sufficient to diagnose PBC. Ursodeoxycholic acid is approved and recommended for the treatment of PBC. The diagnosis of PSC is established by elevated alkaline phosphatase-levels and typical cholangiographic findings...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27852547/prevalence-of-igg-4-associated-cholangiopathy-based-on-serum-igg-4-levels-in-patients-with-primary-sclerosing-cholangitis-and-its-relationship-with-inflammatory-bowel-disease
#14
Seyed Alireza Taghavi, Shaghayegh Kaedi Majd, Mahnoosh Sianati, Masood Sepehrimanesh
BACKGROUND/AIMS: Autoimmune cholangiopathy is part of a fibro-inflammatory immunoglobulin G-4 (IgG-4)-related systemic disease that causes biliary tract strictures. Its clinical presentation is quite similar to that of more common diseases such as primary sclerosing cholangitis (PSC) and pancreatobilliary malignancies. The aims of the present study were to evaluate the prevalence of IgG-4-associated cholangiopathy (IAC) in patients diagnosed with PSC and its relationship with inflammatory bowel disease (IBD)...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27843427/isolated-liver-hilar-infiltration-by-igg4-inflammation-mimicking-cholangiocarcinoma
#15
Laurent Bochatay, Pietro Majno, Emiliano Giostra, Jean Louis Frossard
IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27834858/impact-of-microbes-on-the-pathogenesis-of-primary-biliary-cirrhosis-pbc-and-primary-sclerosing-cholangitis-psc
#16
REVIEW
Jochen Mattner
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host...
November 9, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27834483/autoanti-c-in-a-patient-with-primary-sclerosing-cholangitis-and-autoimmune-hemolytic-anemia-a-rare-presentation
#17
Meenu Bajpai, Ashish Maheshwari, Shruti Gupta, Chhagan Bihari
No abstract text is available yet for this article.
September 2016: Immunohematology
https://www.readbyqxmd.com/read/27824421/-elevated-liver-enzymes
#18
Axel Holstege
Elevated liver enzymes are a frequent finding in both symptomatic and asymptomatic patients necessitating further evaluation to clarify the underlying disease. Three different patterns of increased liver enzymes can be defined to allow for a more precise and rational further diagnostic approach. A predominant increase in transaminase activities reflects a disturbance of hepatocellular integrity which can be found in patients with viral hepatitis, genetic liver diseases like Wilson`s disease or hemochromatosis, and drug-induced liver diseases...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27822365/surveillance-for-cholangiocarcinoma-in-patients-with-primary-sclerosing-cholangitis-effective-and-justified
#19
Nataliya Razumilava, Gregory J Gores
No abstract text is available yet for this article.
August 2016: Clinical Liver Disease
https://www.readbyqxmd.com/read/27806633/surveillance-of-primary-sclerosing-cholangitis-with-erc-and-brush-cytology-risk-factors-for-cholangiocarcinoma
#20
Sonja Boyd, Harri Mustonen, Andrea Tenca, Kalle Jokelainen, Johanna Arola, Martti A Färkkilä
OBJECTIVE: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease leading to bile duct strictures and fibrosis, and predisposing to cholangiocarcinoma (CCA). Biliary dysplasia is a known precursor of CCA. In our unit, PSC patients undergo regular surveillance with ERC and brush cytology (BC), and liver transplantation is an option in case with biliary dysplasia. We evaluated the risk factors for biliary dysplasia and CCA based on ERC imaging, BC and liver function tests...
November 3, 2016: Scandinavian Journal of Gastroenterology
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