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Psc OR primary sclerosing cholangitis

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https://www.readbyqxmd.com/read/29226107/cholangitis-diagnosis-treatment-and-prognosis
#1
REVIEW
Amir Houshang Mohammad Alizadeh
Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC)...
December 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29208940/cea-but-not-ca19-9-is-an-independent-prognostic-factor-in-patients-undergoing-resection-of-cholangiocarcinoma
#2
Sven H Loosen, Christoph Roderburg, Katja L Kauertz, Alexander Koch, Mihael Vucur, Anne T Schneider, Marcel Binnebösel, Tom F Ulmer, Georg Lurje, Wenzel Schoening, Frank Tacke, Christian Trautwein, Thomas Longerich, Cornelis H Dejong, Ulf P Neumann, Tom Luedde
Cholangiocarcinoma (CCA) represents a rare form of primary liver cancer with increasing incidence but dismal prognosis. Surgical treatment has remained the only potentially curative treatment option, but it remains unclear which patients benefit most from liver surgery, highlighting the need for new preoperative stratification strategies. In clinical routine, CA19-9 represents the most widely used tumor marker in CCA patients. However, data on the prognostic value of CA19-9 in CCA patients are limited and often inconclusive, mostly due to small cohort sizes...
December 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29180269/dysregulation-of-antioxidant-responses-in-patients-diagnosed-with-concomitant-primary-sclerosing-cholangitis-inflammatory-bowel-disease
#3
Colin T Shearn, David J Orlicky, Dennis R Petersen
OBJECTIVE: Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by severe peri-biliary tract inflammation and fibrosis, 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). The objective of this study was to determine the impact of end stage PSC/IBD on cellular antioxidant responses and the formation of protein carbonylation...
November 24, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29180000/usefulness-of-biochemical-remission-and-transient-elastography-in-monitoring-disease-course-in-autoimmune-hepatitis
#4
Johannes Hartl, Hanno Ehlken, Marcial Sebode, Moritz Peiseler, Till Krechl, Roman Zenouzi, Johann von Felden, Christina Weiler-Normann, Christoph Schramm, Ansgar W Lohse
BACKGROUND: Liver fibrosis regression but also progression may occur in patients with autoimmune hepatitis (AIH) under treatment. There is a need for non-invasive surrogate markers for fibrosis development in AIH to better guide immunosuppressive treatment. AIMS: The aims of the study were to assess the impact of complete biochemical remission defined as normalisation of transaminases and IgG on histological activity and fibrosis development, and the value of repeat transient elastography (TE) measurement for monitoring disease progression in AIH...
November 24, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29168311/-usefulness-of-serum-wisteria-floribunda-agglutinin-positive-mac-2-binding-protein-in-children-with-primary-sclerosing-cholangitis
#5
Shuichiro Umetsu, Ayano Inui, Tsuyoshi Sogo, Haruki Komatsu, Tomoo Fujisawa
BACKGROUND: Wisteria floribunda agglutinin positive Mac-2 binding protein (WFA+ -M2BP) is a novel serum marker of hepatic fibrosis in adults with chronic hepatitis C. However, it remains unclear whether serum WFA+ -M2BP levels are associated with the progression of liver histology in primary sclerosing cholangitis (PSC) . METHODS: Twenty-eight children and adolescence with pediatric onset-PSC (M/F = 20/8, median age at diagnosis: 9 years) were enrolled in this study...
November 22, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29163969/ercp-in-infants-children-and-adolescents-is-feasible-and-safe-results-from-a-tertiary-care-center
#6
Jasmin Felux, Ekkehard Sturm, Andreas Busch, Emanuel Zerabruck, Florian Graepler, Dietmar Stüker, Andreas Manger, Hans-Joachim Kirschner, Gunnar Blumenstock, Nisar P Malek, Martin Goetz
Background: Indications for endoscopic retrograde cholangiopancreatography (ERCP) in children differ from adults. Paucity of data and concerns about potential lower effectiveness and more side effects limit its use even in high volume centers. We retrospectively analyzed indications, success rates, limitations, and side effects of ERCPs in children <18 years. Methods: From January 2012 to March 2015, 54 ERCPs were performed in 31 children (median age 11 (0-17) years; median weight 22 (3...
November 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#7
Marlen Ivón Castellanos Fernández, Deyanira la Rosa Hernández, Diego Enrique Cabrera Eugenio, Wilson Palanca, Zaily Dorta Guridi, Licet González Fabián
Background: Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean countries. Objectives: The aim of our study was to investigate the clinical patterns, laboratory findings, and immunologic features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. Methods: A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba, from May 2012 to April 2016...
2017: Current Therapeutic Research, Clinical and Experimental
https://www.readbyqxmd.com/read/29152767/development-and-validation-of-a-primary-sclerosing-cholangitis-specific-patient-reported-outcomes-instrument-the-psc-pro
#8
Zobair M Younossi, Arian Afendy, Maria Stepanova, Andrei Racila, Fatema Nader, Rachel Gomel, Ricky Safer, William R Lenderking, Anne Skalicky, Leah Kleinman, Robert P Myers, G Mani Subramanian, John G McHutchison, Cynthia Levy, Christopher L Bowlus, Kris Kowdley, Andrew J Muir
BACKGROUND: PSC is a chronic liver disease associated with inflammation and biliary fibrosis that leads to cholangitis, cirrhosis, and impaired quality of life. Our objective was to develop and validate a PSC-specific PRO instrument. METHODS: We developed a 42-item PSC PRO instrument that contains 2 modules (Symptoms and Impact of Symptoms), and conducted external validation. Reliability and validity were evaluated using clinical data and a battery of other validated instruments...
November 20, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29150966/current-policy-for-allocation-of-donor-livers-in-the-netherlands-advantages-primary-sclerosing-cholangitis-patients-on-the-liver-transplantation-waiting-list-a-retrospective-study
#9
Jorn C Goet, Bettina E Hansen, Madelon Tieleman, Bart van Hoek, Aad P van den Berg, Wojciech G Polak, Jeroen Dubbeld, Robert J Porte, Cynthia Konijn-Janssen, Robert A de Man, Herold J Metselaar, Annemarie C de Vries
Studies from the USA and Nordic countries indicate primary sclerosing cholangitis (PSC) patients have low mortality on the liver transplantation (LTx) waiting list. However, this may vary among geographical areas. Therefore, we compared waiting list mortality and post-transplant survival between laboratory model for end-stage liver disease (LM) and MELD exception (ME)-prioritized PSC and non-PSC candidates in a nationwide study in the Netherlands. A retrospective analysis of patients waitlisted from 2006 to 2013 was conducted...
November 18, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/29149928/rheumatic-manifestations-in-autoimmune-liver-disease
#10
REVIEW
Carlo Selmi, Elena Generali, Merrill Eric Gershwin
Autoimmune liver diseases coexist with rheumatic disorders in approximately 30% of cases and may also share pathogenic mechanisms. Autoimmune liver diseases result from an immune-mediated injury of different tissues, with autoimmune hepatitis (AIH) targeting hepatocytes, and primary biliary cholangitis (PBC) and primary sclerosing cholangitis targeting cholangiocytes. Sjogren syndrome is diagnosed in 7% of AIH cases and serologic autoimmunity profiles are a common laboratory abnormality, particularly in the case of serum antimitochondrial (PBC) or anti-liver kidney microsomal antibodies (AIH)...
February 2018: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29148959/effect-of-measuring-%C3%AE-glutamyl-transpeptidase-and-using-smoflipid-in-a-parenteral-nutrition-infusion-in-a-patient-with-severe-malabsorption-and-overlapping-primary-sclerosing-cholangitis-and-ulcerative-colitis
#11
Merin Kinikini
Studies discussing inflammation and oxidative stress state that these conditions are known contributors in the pathogenesis of cholestatic diseases and ulcerative colitis, and studies examining patients with liver disease have found decreased antioxidant status and significant elevation of lipid peroxides as compared with healthy subjects. One hypothesis in liver disease is that deficient antioxidant defense mechanisms may lead to excess oxygen free radical formation, which promotes deleterious processes in the liver...
November 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29138587/primary-sclerosing-cholangitis-diagnostic-and-management-challenges
#12
REVIEW
Sanjeev Sirpal, Natasha Chandok
Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/29129978/a-case-of-peripheral-ulcerative-keratitis-associated-with-autoimmune-hepatitis
#13
Hamoon Eshraghi, Aria Mahtabfar, Mohammad H Dastjerdi
Purpose: To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis. Case Report: A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determined to be peripheral ulcerative keratitis. The patient was treated with topical and systemic steroids, immunosuppressant drugs (azathioprine and mycophenolate mofetil), a biologic (rituximab), and surgery (conjunctival resection), and the peripheral ulcerative keratitis epithelialized but ultimately led to corneal perforation...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29126693/characterization-of-patients-with-autoimmune-hepatitis-at-an-university-hospital-in-medell%C3%A3-n-colombia-cohort-study
#14
Gabriel Sebastián Díaz-Ramírez, Juan Ignacio Marín-Zuluaga, Jorge Hernando Donado-Gómez, Octavio Muñoz-Maya, Óscar Santos-Sánchez, Juan Carlos Restrepo-Gutiérrez
INTRODUCTION: Autoimmune hepatitis is a chronic liver disease that impacts on morbidity and mortality of patients. Few epidemiological data exist of this in Latin America and Colombia. OBJECTIVES: The aim of this study is to describe the demographic, clinical and laboratory characteristics of the patients; the treatment and the response to it, the evolution and course of the disease, requirement of liver transplantation and mortality. METHODS: Historical cohort study that include patients attended at an University Hospital in Medellin, Colombia between January 2010 and December 2016 with ≥16 years age at the time of diagnosis of autoimmune hepatitis...
November 7, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/29106505/successful-treatment-with-vedolizumab-in-a-patient-with-chronic-refractory-pouchitis-and-primary-sclerosing-cholangitis
#15
M Coletta, M Paroni, F Caprioli
No abstract text is available yet for this article.
July 5, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/29102704/primary-sclerosing-cholangitis-is-not-rare-among-blacks-in-a-multicenter-north-american-consortium
#16
David Goldberg, Cynthia Levy, Kidist Yimam, Stuart Gordon, Lisa Forman, Elizabeth Verna, Lei Yu, Robert Rahimi, Kathleen Schwarz, Bertus Eksteen, Daniel Pratt, Thomas Boyer, David Assis, Christopher Bowlus
No abstract text is available yet for this article.
November 2, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29089545/activation-of-fas-fasl-pathway-and-the-role-of-c-flip-in-primary-culture-of-human-cholangiocarcinoma-cells
#17
Gianluca Carnevale, Guido Carpino, Vincenzo Cardinale, Alessandra Pisciotta, Massimo Riccio, Laura Bertoni, Lara Gibellini, Sara De Biasi, Lorenzo Nevi, Daniele Costantini, Diletta Overi, Andrea Cossarizza, Anto de Pol, Eugenio Gaudio, Domenico Alvaro
Intrahepatic cholangiocarcinoma (iCCA) represents a heterogeneous group of malignancies emerging from the biliary tree, often in the context of chronic bile ducts inflammation. The immunological features of iCCA cells and their capability to control the lymphocytes response have not yet been investigated. The aims of the present study were to evaluate the interaction between iCCA cells and human peripheral blood mononuclear cells (PBMCs) and the role of Fas/FasL in modulating T-cells and NK-cells response after direct co-culture...
October 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29083037/review-article-the-gut-microbiome-as-a-therapeutic-target-in-the-pathogenesis-and-treatment-of-chronic-liver-disease
#18
REVIEW
C A Woodhouse, V C Patel, A Singanayagam, D L Shawcross
BACKGROUND: Mortality from chronic liver disease is rising exponentially. The liver is intimately linked to the gut via the portal vein, and exposure to gut microbiota and their metabolites translocating across the gut lumen may impact upon both the healthy and diseased liver. Modulation of gut microbiota could prove to be a potential therapeutic target. AIM: To characterise the changes in the gut microbiome that occur in chronic liver disease and to assess the impact of manipulation of the microbiome on the liver...
October 30, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29081776/innate-lymphoid-cells-in-intestinal-inflammation
#19
REVIEW
Alessandra Geremia, Carolina V Arancibia-Cárcamo
Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the intestine that encompasses Crohn's disease (CD) and ulcerative colitis. The cause of IBD is unknown, but the evidence suggests that an aberrant immune response toward the commensal bacterial flora is responsible for disease in genetically susceptible individuals. Results from animal models of colitis and human studies indicate a role for innate lymphoid cells (ILC) in the pathogenesis of chronic intestinal inflammation in IBD. ILC are a population of lymphocytes that are enriched at mucosal sites, where they play a protective role against pathogens including extracellular bacteria, helminthes, and viruses...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29069021/relapsing-polychondritis-in-a-liver-transplant-recipient-a-case-report
#20
Anna Mydlak, Dariusz Sołdacki, Bartosz Foroncewicz, Zygmunt Stopa, Agnieszka Powała, Tadeusz Budlewski, Leszek Pączek, Krzysztof Mucha
RATIONALE: Relapsing polychondritis (RP) is a multisystemic, progressive disease of unknown etiology characterized by recurrent inflammation and progressive cartilage destruction. It can involve all types of cartilage including ears and nose, tracheobronchial tree, joints, and any other tissue rich in proteoglycans such as heart, eyes, and blood vessels. Recurrent chondritis can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. To date there is no data in the literature on the post solid organ transplantation RP...
October 2017: Medicine (Baltimore)
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