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Psc OR primary sclerosing cholangitis

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https://www.readbyqxmd.com/read/28815030/a-combined-bile-and-urine-proteomic-test-for-cholangiocarcinoma-diagnosis-in-patients-with-biliary-strictures-of-unknown-origin
#1
Torsten Voigtländer, Jochen Metzger, Bastian Schönemeier, Mark Jäger, Harald Mischak, Michael P Manns, Tim O Lankisch
BACKGROUND: Detection of cholangiocarcinoma (CC) remains a diagnostic challenge particularly in patients with primary sclerosing cholangitis (PSC). We recently established diagnostic peptide marker models in bile and urine to detect CC. Our aim was to combine both models to reach a higher diagnostic accuracy of CC diagnosis. METHODS: Bile (BPA) and urine (UPA) proteome analysis by capillary electrophoresis mass spectrometry was performed in a case-control phase II study on 87 patients (36 CC including 13 with CC on top of PSC, 33 PSC and 18 other benign disorders)...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28807459/rapidly-progressive-atherosclerosis-after-domino-liver-transplantation-from-a-teenage-donor-with-homozygous-familial-hypercholesterolemia
#2
Jessica R Golbus, Linda Farhat, Robert J Fontana, Melvyn Rubenfire
Familial hypercholesterolemia (FH) is an autosomal dominant disorder characterized by impaired clearance of low-density lipoprotein cholesterol. Given limitations in pharmacologic therapy and the significant morbidity and mortality associated with this disease, liver transplantation may be offered to select homozygous FH patients in childhood in an effort to slow progression of atherosclerotic cardiovascular disease. In rare cases, domino liver transplantation can be performed, transplanting the livers of patients with various metabolic disorders into elderly recipients whose projected survival precludes prolonged waiting on the transplant list...
July 22, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28802875/primary-sclerosing-cholangitis-a-comprehensive-review
#3
REVIEW
Tom H Karlsen, Trine Folseraas, Douglas Thorburn, Mette Vesterhus
Primary sclerosing cholangitis (PSC) is a rare disorder characterized by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease (IBD) is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. With no effective medical therapy, most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and lack of proven surveillance strategies, patients experience significant unmet needs...
August 9, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28802066/targeting-senescent-cholangiocytes-and-activated-fibroblasts-with-bcl-xl-inhibitors-ameliorates-fibrosis-in-mdr2-mice
#4
Anja Moncsek, Mohammed S Al-Suraih, Christy E Trussoni, Steven P O'Hara, Patrick L Splinter, Camille Zuber, Eleonora Patsenker, Piero V Valli, Christian D Fingas, Achim Weber, Yi Zhu, Tamar Tchkonia, James L Kirkland, Gregory J Gores, Beat Müllhaupt, Nicholas F LaRusso, Joachim C Mertens
Cholangiocyte senescence has been linked to primary sclerosing cholangitis (PSC). Persistent secretion of growth factors by senescent cholangiocytes leads to the activation of stromal fibroblasts (ASF), which are drivers of fibrosis. The activated phenotype of ASF is characterized by an increased sensitivity to apoptotic stimuli. Here, we examined the mechanisms of apoptotic priming in ASF and explored a combined targeting strategy to deplete senescent cholangiocytes and ASF from fibrotic tissue to ameliorate liver fibrosis...
August 12, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28796833/bile-microbiota-in-primary-sclerosing-cholangitis-impact-on-disease-progression-and-development-of-biliary-dysplasia
#5
Pedro Pereira, Velma Aho, Johanna Arola, Sonja Boyd, Kalle Jokelainen, Lars Paulin, Petri Auvinen, Martti Färkkilä
OBJECTIVE: The etiopathogenesis and risk for development of biliary neoplasia in primary sclerosing cholangitis (PSC) are largely unknown. Microbes or their metabolites have been suggested to play a role. To explore this potential microbial involvement, we evaluated the differences in biliary microbiota in PSC patients at an early disease stage without previous endoscopic retrograde cholangiography (ERC) examinations, advanced disease stage, and with biliary dysplasia or cholangiocarcinoma...
2017: PloS One
https://www.readbyqxmd.com/read/28796371/inflammatory-bowel-disease-with-primary-sclerosing-cholangitis-a-danish-population-based-cohort-study-1977-2011
#6
Jakob Ørskov Sørensen, Ole Haagen Nielsen, Mikael Andersson, Mark Andrew Ainsworth, Henriette Ytting, Erika Bélard, Tine Jess
BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC. METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files. RESULTS: For all PSC-IBD patients diagnosed after 1976 (n=222) and 8,231 IBD controls (i...
August 10, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28794437/genetic-variation-at-the-cd28-locus-and-its-impact-on-expansion-of-pro-inflammatory-cd28-negative-t-cells-in-healthy-individuals
#7
Evaggelia Liaskou, Louisa Jeffery, Dimitrios Chanouzas, Blagoje Soskic, Michael F Seldin, Lorraine Harper, David Sansom, Gideon M Hirschfield
The CD28 locus is associated with susceptibility to a variety of autoimmune and immune-mediated inflammatory diseases including primary sclerosing cholangitis (PSC). Previously, we linked the CD28 pathway in PSC disease pathology and found that vitamin D could maintain CD28 expression. Here, we assessed whether the PSC-associated CD28 risk variant A (rs7426056) affects CD28 expression and T cell function in healthy individuals (n = 14 AA, n = 14 AG, n = 14 GG). Homozygotes for the PSC disease risk allele (AA) showed significantly lower CD28 mRNA expression ex-vivo than either GG or AG (p < 0...
August 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28791317/new-approach-to-decrease-post-ercp-adverse-events-in-patients-with-primary-sclerosing-cholangitis
#8
Udayakumar Navaneethan, Dennisdhilak Lourdusamy, Norma G Gutierrez, Xiang Zhu, John J Vargo, Mansour A Parsi
BACKGROUND AND STUDY AIMS : Endoscopic retrograde cholangiopancreatography (ERCP) is often performed in patients with primary sclerosing cholangitis (PSC). Our aim was to validate a treatment approach with the objective of decreasing ERCP related adverse events (AEs). PATIENTS AND METHODS : All patients who had undergone ERCP for PSC during the period from 2002 - 2012 were identified (group I). This group had traditional ERCP (no bile aspiration prior to contrast injection with balloon dilation and stent placement for treatment of dominant strictures)...
August 2017: Endoscopy International Open
https://www.readbyqxmd.com/read/28783896/liver-fluke-associated-biliary-tract-cancer
#9
REVIEW
Piyapan Prueksapanich, Panida Piyachaturawat, Prapimphan Aumpansub, Wiriyaporn Ridtitid, Roongruedee Chaiteerakij, Rungsun Rerknimitr
Cholangiocarcinoma (CCA) is an aggressive cancer arising from epithelial cells of the bile duct. Most patients with CCA have an unresectable tumor at the time of diagnosis. In Western countries, the risk of CCA increases in patients with primary sclerosing cholangitis, whereas liver fluke infection appears to be the major risk factor for CCA in Asian countries. A diagnosis of liver fluke infection often relies on stool samples, including microscopic examination, polymerase chain reaction-based assays, and fluke antigen detection...
August 9, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28765476/annexin-a11-is-targeted-by-igg4-and-igg1-autoantibodies-in-igg4-related-disease
#10
Lowiek M Hubers, Harmjan Vos, Alex R Schuurman, Robin Erken, Ronald P Oude Elferink, Boudewijn Burgering, Stan F J van de Graaf, Ulrich Beuers
OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan immune-mediated disease that predominantly affects the biliary tract (IgG4-associated cholangitis, IAC) and pancreas (autoimmune pancreatitis, AIP). We recently identified highly expanded IgG4+ B-cell receptor clones in blood and affected tissues of patients with IAC/AIP suggestive of specific (auto)antigenic stimuli involved in initiating and/or maintaining the inflammatory response. This study aimed to identify (auto)antigen(s) that are responsible for the clonal expansion of IgG4+ B cells in IgG4-RD...
August 1, 2017: Gut
https://www.readbyqxmd.com/read/28757171/genetic-determinants-of-cholangiopathies-molecular-and-systems-genetics
#11
REVIEW
Matthias C Reichert, Rabea A Hall, Marcin Krawczyk, Frank Lammert
Familial cholangiopathies are rare but potentially severe diseases. Their spectrum ranges from fairly benign conditions as, for example, benign recurrent intrahepatic cholestasis to low-phospholipid associated cholelithiasis and progressive familial intrahepatic cholestasis (PFIC). Many cholangiopathies such as primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC) affect first the bile ducts ("ascending pathophysiology") but others, such as PFIC, start upstream in hepatocytes and cause progressive damage "descending" down the biliary tree and leading to end-stage liver disease...
July 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28756053/-patients-with-ulcerative-colitis-and-primary-sclerosing-cholangitis-frequently-have-subclinical-inflammation-in-the-proximal-colon
#12
Noa Krugliak Cleveland, David T Rubin, John Hart, Christopher R Weber, Katherine Meckel, Anthony L Tran, Arthur S Aelvoet, Isabella Pan, Alex Gonsalves, John Nick Gaetano, Kelli Williams, Kristen Wroblewski, Bana Jabri, Joel Pekow
BACKGROUND AND AIMS: Primary sclerosing cholangitis (PSC) patients with ulcerative colitis (UC) have a high risk of colonic neoplasia. As histologic inflammation is an independent risk factor for the development of neoplasia, we hypothesized that patients with UC and PSC have more subclinical disease activity than patients with UC alone. METHODS: We performed a retrospective evaluation of patients with ulcerative pancolitis who were in clinical remission and compared endoscopic and histologic activity between those with and without PSC...
July 26, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28754453/pathophysiologic-implications-of-innate-immunity-and-autoinflammation-in-the-biliary-epithelium
#13
REVIEW
Mario Strazzabosco, Romina Fiorotto, Massimiliano Cadamuro, Carlo Spirli, Valeria Mariotti, Eleanna Kaffe, Roberto Scirpo, Luca Fabris
The most studied physiological function of biliary epithelial cells (cholangiocytes) is to regulate bile flow and composition, in particular the hydration and alkalinity of the primary bile secreted by hepatocytes. After almost three decades of studies it is now become clear that cholangiocytes are also involved in epithelial innate immunity, in inflammation, and in the reparative processes in response to liver damage. An increasing number of evidence highlights the ability of cholangiocyte to undergo changes in phenotype and function in response to liver damage...
July 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28754450/pathogenesis-of-biliary-fibrosis
#14
REVIEW
Massimo Pinzani, Tu Vinh Luong
Chronic cholestatic liver diseases such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are associated with active hepatic fibrogenesis, and, ultimately, to the development of cirrhosis. However, the precise relationship between cholestasis, in its broad meaning, and liver tissue fibrosis is still poorly defined. Fibrogenesis is currently viewed as a dynamic process that appears strictly related to the extent and duration of parenchymal injury. This relationship is clearly evident in the presence of reiterative hepatocellular necrosis due to viral infection or alcohol abuse...
July 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28740343/distinct-gut-microbiota-profiles-in-patients-with-primary-sclerosing-cholangitis-and-ulcerative-colitis
#15
Lukas Bajer, Miloslav Kverka, Martin Kostovcik, Peter Macinga, Jiri Dvorak, Zuzana Stehlikova, Jan Brezina, Pavel Wohl, Julius Spicak, Pavel Drastich
AIM: To characterize the gut bacterial microbiota of patients with primary sclerosing cholangitis (PSC) and ulcerative colitis (UC). METHODS: Stool samples were collected and relevant clinical data obtained from 106 study participants, 43 PSC patients with (n = 32) or without (n = 11) concomitant inflammatory bowel disease, 32 UC patients, and 31 healthy controls. The V3 and V4 regions of the 16S ribosomal RNA gene were sequenced on Illumina MiSeq platform to cover low taxonomic levels...
July 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28739581/a-novel-prognostic-model-for-transplant-free-survival-in-primary-sclerosing-cholangitis
#16
Elisabeth M de Vries, Junfeng Wang, Kate D Williamson, Mariska M Leeflang, Kirsten Boonstra, Rinse K Weersma, Ulrich Beuers, Roger W Chapman, Ronald B Geskus, Cyriel Y Ponsioen
OBJECTIVE: Most prognostic models for primary sclerosing cholangitis (PSC) are based on patients referred to tertiary care and may not be applicable for the majority of patients with PSC. The aim of this study was to construct and externally validate a novel, broadly applicable prognostic model for transplant-free survival in PSC, based on a large, predominantly population-based cohort using readily available variables. DESIGN: The derivation cohort consisted of 692 patients with PSC from the Netherlands, the validation cohort of 264 patients with PSC from the UK...
July 24, 2017: Gut
https://www.readbyqxmd.com/read/28733279/cholangiocytes-and-the-environment-in-primary-sclerosing-cholangitis-where-is-the-link
#17
Steven P O'Hara, Tom H Karlsen, Nicholas F LaRusso
No abstract text is available yet for this article.
July 21, 2017: Gut
https://www.readbyqxmd.com/read/28733178/clinical-usefulness-of-serum-antibodies-as-biomarkers-of-gastrointestinal-and-liver-diseases
#18
REVIEW
Antonio Di Sabatino, Federico Biagi, Marco Lenzi, Luca Frulloni, Marco Vincenzo Lenti, Paolo Giuffrida, Gino Roberto Corazza
The progressively growing knowledge of the pathophysiology of a number of immune-mediated gastrointestinal and liver disorders, including autoimmune atrophic gastritis, coeliac disease, autoimmune enteropathy, inflammatory bowel disease, autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cholangitis and autoimmune pancreatitis, together with the improvement of their detection methods have increased the diagnostic power of serum antibodies. In some cases - coeliac disease and autoimmune atrophic gastritis - they have radically changed gastroenterologists' diagnostic ability, while in others - autoimmune hepatitis, inflammatory bowel disease and autoimmune pancreatitis - their diagnostic performance is still inadequate...
June 23, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28724302/efficacy-and-safety-of-vedolizumab-as-a-treatment-option-for-moderate-to-severe-refractory-ulcerative-colitis-in-two-patients-after-liver-transplant-due-to-primary-sclerosing-cholangitis
#19
Raúl Vicente Olmedo Martín, Víctor Amo Trillo, Rocío González Grande, Miguel Jiménez Pérez
Vedolizumab is a humanized IgG1 monoclonal antibody that selectively blocks the lymphocyte integrin α4β7 and prevents its interaction with endothelial adhesion molecules and subsequent transmigration to the gastrointestinal tract. The drug was approved in 2014 for the induction and maintenance treatment of ulcerative colitis and moderate to severe Crohn's disease that is refractory or intolerant to conventional treatment with corticoids and immunosuppressants and/or anti-TNFα drugs. However, inflammatory bowel disease has a variable behavior following liver transplant...
July 14, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28712945/intestinal-and-extraintestinal-cancers-associated-with-inflammatory-bowel-disease
#20
REVIEW
Minna Chang, Liisa Chang, Hanna M Chang, Fuju Chang
Inflammatory bowel disease (IBD) with its 2 most common entities, ulcerative colitis and Crohn's disease, causes an increased risk of developing intestinal cancers. In fact, malignancies are the second most common cause of death after cardiovascular diseases in both sexes of patients with IBD. Risk factors for colorectal cancer in IBD correlate with the duration of the disease, extent of disease, the association with primary sclerosing cholangitis, family history, and early age at onset. Patients with IBD also have an increased risk for developing a variety of extraintestinal malignancies...
June 27, 2017: Clinical Colorectal Cancer
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