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https://www.readbyqxmd.com/read/28739581/a-novel-prognostic-model-for-transplant-free-survival-in-primary-sclerosing-cholangitis
#1
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
#2
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
#3
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/28731850/the-diagnostic-accuracy-of-biomarkers-for-diagnosis-of-primary-biliary-cholangitis-pbc-in-anti-mitochondrial-antibody-ama-negative-pbc-patients-a-review-of-literature
#4
Federica de Liso, Caterina Matinato, Mariangela Ronchi, Rita Maiavacca
Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is an autoimmune disease of the liver characterized by anti-mitochondrial antibodies (AMA) in 90%-95% of patients. The aim of this study was to evaluate the diagnostic value of several serum biomarkers in patients with PBC but negative for AMA. Some antinuclear antibodies (ANA) pattern, detected by indirect immunofluorescence (IIF), such as multiple nuclear dot (MND) and rim-like patterns are well-known to be specific for PBC. The corresponding nuclear antigens are the components of the nuclear pore complex (Gp210 for rim-like pattern) and Sp100, PML proteins (for MND pattern) detectable by immunoblotting and ELISA methods...
July 21, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28711597/micrornas-and-extracellular-vesicles-in-cholangiopathies
#7
REVIEW
P Olaizola, P Y Lee-Law, A Arbelaiz, A Lapitz, M J Perugorria, L Bujanda, J M Banales
Cholangiopathies encompass a heterogeneous group of disorders affecting biliary epithelial cells (i.e. cholangiocytes). Early diagnosis, prognosis and treatment still remain clinically challenging for most of these diseases and are critical for adequate patient care. In the past decade, extensive research has emphasized microRNAs (miRs) as potential non-invasive biomarkers and tools to accurately identify, predict and treat cholangiopathies. MiRs can be released extracellularly conjugated with lipoproteins or encapsulated in extracellular vesicles (EVs)...
July 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28706981/outcomes-of-liver-transplant-recipients-with-autoimmune-liver-disease-using-long-term-dual-immunosuppression-regimen-without-corticosteroid
#8
Sanjaya K Satapathy, Ollie D Jones, Jason M Vanatta, Faisal Kamal, Satish K Kedia, Yu Jiang, Satheesh P Nair, James D Eason
BACKGROUND: Liver transplant (LT) recipients with autoimmune liver disease (primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis) are at increased risk of developing acute cellular rejection (ACR), and in many cases graft failure due to recurrent disease. We describe our experience with dual immunosuppression without steroid maintenance and analyze its effect on disease recurrence; ACR; patient and graft survivals; and complications, such as sepsis and de novo malignancy...
July 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28706774/are-dominant-strictures-in-primary-sclerosing-cholangitis-a-risk-factor-for-cholangiocarcinoma
#9
REVIEW
Roger W Chapman, Kate D Williamson
PURPOSE OF REVIEW: Cholangiocarcinoma is a devastating, unpredictable complication of large duct primary sclerosing cholangitis (PSC), which occurs in 5-15% of patients. The aim of this review is to discuss whether dominant strictures (DS) occurring in the larger bile ducts in PSC are a risk factor for the development of cholangiocarcinoma. RECENT FINDINGS: The development of DS is related to specific genetic polymorphisms affecting the innate immune system and the microbiome...
2017: Current Hepatology Reports
https://www.readbyqxmd.com/read/28706773/why-doesn-t-primary-biliary-cholangitis-respond-to-immunosuppressive-medications
#10
REVIEW
Antonio Molinaro, Hanns-Ulrich Marschall
PURPOSE OF REVIEW: The purpose of this review is to discuss reasons why immunosuppressive therapy so far failed in Primary Biliry Cholangitis. RECENT FINDINGS: Even targeted immunosuppressive therapy seems ineffective or potentially harmful. SUMMARY: Bile acid-mediated cholangiocyte damage, facilitated by insufficient bicarbonate secretion, seems to attenuate the anti-inflammatory and anti-fibrotic actions of immunosuppressant and immunomodulatory drugs in a clinically significant way...
2017: Current Hepatology Reports
https://www.readbyqxmd.com/read/28706093/does-neutrophil-to-lymphocyte-ratio-predict-1-year-mortality-in-patients-with-primary-biliary-cholangitis-results-from-a-retrospective-study-with-validation-cohort
#11
Lin Lin, Meiyu Piao, Xihui Jiang, Houning Lv, Ningning Zhao, Fang Yang, Chao Sun
OBJECTIVES: Neutrophil-to-lymphocyte ratio (NLR) has been used to predict prognosis in various liver diseases, but its role in primary biliary cholangitis (PBC) is not clarified. We aimed to investigate the prognostic usefulness of NLR for 1-year mortality in PBC. METHODS: The study recruited a retrospective cohort with 88 patients with PBC and a prospective validation cohort with 63 participants who were followed-up for 1 year. NLR and other laboratory measurements were analysed by multivariate regression model for identifying independent factors for early mortality...
July 12, 2017: BMJ Open
https://www.readbyqxmd.com/read/28703133/association-of-autoimmune-hepatitis-with-src-homology-2-adaptor-protein-3-gene-polymorphisms-in-japanese-patients
#12
Takeji Umemura, Satoru Joshita, Hideaki Hamano, Kaname Yoshizawa, Shigeyuki Kawa, Eiji Tanaka, Masao Ota
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease characterized by an autoimmune reaction to hepatocytes. The Src homology 2 adaptor protein 3 (SH2B3) gene is a member of the SH2B family of adaptor proteins that has been implicated in the integration and regulation of multiple signaling events. SH2B3 is involved in cytokine signaling pathways and serves as a negative mediator of T-cell receptor signaling. Genome-wide association analyses in Caucasians have linked a missense mutation at rs3184504 in SH2B3 with AIH...
July 13, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28698093/bile-acids-and-intestinal-microbiota-in-autoimmune-cholestatic-liver-diseases
#13
REVIEW
You Li, Ruqi Tang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
Autoimmune cholestatic liver diseases, including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), are manifested as an impairment of normal bile flow and excessive accumulation of potentially toxic bile acids. Endogenous bile acids are involved in the pathogenesis and progression of cholestasis. Consequently, chronic cholestasis affects the expression of bile acid transporters and nuclear receptors, and results in liver injury. Several lines of evidence suggest that intestinal microbiota plays an important role in the etiopathogenesis of cholestatic liver diseases by regulating metabolism and immune responses...
July 8, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28695657/hla-haplotypes-in-primary-sclerosing-cholangitis-patients-of-admixed-and-non-european-ancestry
#14
E K K Henriksen, M K Viken, M Wittig, K Holm, T Folseraas, S Mucha, E Melum, J R Hov, K N Lazaridis, B D Juran, O Chazouillères, M Färkkilä, D N Gotthardt, P Invernizzi, M Carbone, G M Hirschfield, S M Rushbrook, E Goode, C Y Ponsioen, R K Weersma, B Eksteen, K K Yimam, S C Gordon, D Goldberg, L Yu, C L Bowlus, A Franke, B A Lie, T H Karlsen
Primary sclerosing cholangitis (PSC) is strongly associated with several human leukocyte antigen (HLA) haplotypes. Due to extensive linkage disequilibrium and multiple polymorphic candidate genes in the HLA complex, identifying the alleles responsible for these associations has proven difficult. We aimed to evaluate whether studying populations of admixed or non-European descent could help in defining the causative HLA alleles. When assessing haplotypes carrying HLA-DRB1*13:01 (hypothesized to specifically increase the susceptibility to chronic cholangitis), we observed that every haplotype in the Scandinavian PSC population carried HLA-DQB1*06:03...
July 11, 2017: HLA
https://www.readbyqxmd.com/read/28693186/cholangiocarcinoma-following-external-beam-radiotherapy-a-report-of-two-cases
#15
Anisha Kulkarni, Justin S Gundara, Anthony J Gill, Thomas J Hugh, Jaswinder S Samra
Cholangiocarcinoma (CCA) is a relatively rare primary malignancy, with established risk factors that include primary sclerosing cholangitis, choledochal cysts and hepatolithiasis. In the present study, two cases of CCA, which occurred following abdominal external beam radiotherapy (EBRT) for non-Hodgkin's lymphoma, are reported. Case 1 and 2 were diagnosed with cholangiocarcinoma 30 and 4 years following treatment with combined chemotherapy and abdominal radiotherapy treatment, respectively. The patients received chemotherapy as treatment of cholangiocarcinoma; however, whilst their symptoms improved, they succumbed within 12 and 2 months respectively following the diagnosis with cholangiocarcinoma...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28690845/diagnostic-autoantibodies-for-autoimmune-liver-diseases
#16
REVIEW
Ban-Hock Toh
Autoimmune liver diseases are conditions of low prevalence that comprise the triad of autoimmune hepatitis, primary biliary cholangitis (cirrhosis) and primary sclerosing cholangitis and their poorly characterised overlapping syndromes. Diagnostic autoantibodies are associated with autoimmune hepatitis and primary biliary cholangitis but not with primary sclerosing cholangitis. Autoantibodies are useful disease markers that facilitate early diagnosis of autoimmune hepatitis and primary biliary cholangitis and allow for therapeutic intervention to prevent progression to liver cirrhosis and associated complications...
May 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/28690452/advances-in-the-diagnosis-and-management-of-colonic-dysplasia-in-patients-with-inflammatory-bowel-disease
#17
Shirley Cohen-Mekelburg, Yecheskel Schneider, Stephanie Gold, Ellen Scherl, Adam Steinlauf
The prevalence of colorectal cancer (CRC) in inflammatory bowel disease (IBD) is estimated at 3.7%. Risk factors for CRC include more severe disease (as reflected by the extent of disease and the duration of poorly controlled disease), family history of CRC, pseudopolyps, primary sclerosing cholangitis, and male sex. In addition, both early and late onset of IBD have been shown to be risk factors in different studies. Most societal guidelines recommend initiation of surveillance colonoscopy at 8 to 10 years after IBD symptom onset, followed by subsequent surveillance in 1- to 2-year intervals...
June 2017: Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28690174/clinical-outcomes-of-donation-after-circulatory-death-liver-transplantation-in-primary-sclerosing-cholangitis
#18
Palak J Trivedi, Irene Scalera, Emma Slaney, Richard W Laing, Bridget Gunson, Gideon M Hirschfield, Andrea Schlegel, James Ferguson, Paolo Muiesan
BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a progressive fibro-inflammatory cholangiopathy for which liver transplantation is the only life-extending intervention. These patients may benefit from accepting liver donation after circulatory death (DCD), however their subsequent outcome is unknown. METHODS: Clinical outcomes were prospectively evaluated in PSC patients undergoing transplantation from 2006 to 2016 stratified by donor type (DCD, n=35 vs...
July 6, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28684726/hepatic-artery-pseudoaneurysm-following-orthotopic-liver-transplantation-increasing-clinical-suspicion-for-a-rare-but-lethal-pathology
#19
Jon Harrison, Meredith Harrison, Cataldo Doria
BACKGROUND Despite an incidence of between 1% and 2%, the mortality rate in ruptured hepatic artery pseudoaneurysm after orthotopic liver transplantation approaches 69%. Our aim is to report operative and outcomes data for 7 patients with pseudoaneurysm following transplant at 1 institution, with emphasis on suspected risk factors for aneurysm formation. From these risk factors, we performed a systematic review to assess their clinical saliency. MATERIAL AND METHODS Using PRISMA guidelines, we completed a PubMed and online database review to gather studies addressing risk factors for pseudoaneurysm following transplant...
July 7, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28676324/norursodeoxycholic-acid-in-patients-with-primary-sclerosing-cholangitis-a-new-urso-saga-in-the-horizon
#20
EDITORIAL
Olivier Chazouillères
No abstract text is available yet for this article.
July 1, 2017: Journal of Hepatology
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