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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28529147/norursodeoxycholic-acid-improves-cholestasis-in-primary-sclerosing-cholangitis
#1
Peter Fickert, Gideon M Hirschfield, Gerald Denk, Hanns-Ulrich Marschall, Istvan Altorjay, Martti Färkkilä, Christoph Schramm, Ulrich Spengler, Roger Chapman, Annika Bergquist, Erik Schrumpf, Frederik Nevens, Palak Trivedi, Florian P Reiter, Istvan Tornai, Emina Halilbasic, Roland Greinwald, Markus Pröls, Michael P Manns, Michael Trauner
Primary sclerosing cholangitis (PSC) represents a devastating bile duct disease lacking effective medical therapy. 24-norursodeoxycholic acid (norUDCA) is a side chain-shortened C23 homologue of UDCA and has shown potent anti-cholestatic, anti-inflammatory and anti-fibrotic properties in a preclinical PSC mouse model. AIM: To evaluate the safety and efficacy of 3 doses of oral norUDCA (500mg/d, 1000mg/d or 1500 mg/d) compared with placebo in PSC in a RCT including 38centers from 12European countries...
May 18, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28523321/autoimmune-hepatitis-with-sclerosing-cholangitis-in-a-patient-with-thiopurine-methyltransferase-deficiency-case-presentation
#2
Sorin Claudiu Man, Cristina Nicoleta Schnell, Valentina Sas, Anca Dana Buzoianu, Dan Gheban
The association between two autoimmune diseases is known in the literature as overlap syndrome. We present the case of an 18-year-old boy, diagnosed at the age of 13 with an overlap syndrome between type I autoimmune hepatitis and sclerosing cholangitis. The response to immunosuppressant therapy was hampered by azathioprine-induced toxicity causing severe pancytopenia, as a result of thiopurine methyltransferase enzyme genetic deficiency. Treatment was replaced by mycophenolate mofetil. Although the relapse rate was reduced, the disease progressed to cirrhosis...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28520586/clinical-course-of-ulcerative-colitis-after-liver-transplantation-in-patients-with-concomitant-primary-sclerosing-cholangitis-and-ulcerative-colitis
#3
Mohammad Reza Fattahi, Seyyed Ali Malek-Hosseini, Gholam Reza Sivandzadeh, Ali Reza Safarpour, Kamran Bagheri Lankarani, Ali Reza Taghavi, Fardad Ejtehadi
BACKGROUND: The natural history of ulcerative colitis (UC) after liver transplantation (LT) for primary sclerosing cholangitis (PSC) remains ill defined. This study aimed to evaluate the course of UC after LT for PSC. METHODS: The course of UC, including the clinical colitis severity index, was evaluated in patients with concomitant PSC and UC who received LT for PSC-induced end-stage liver disease. A total of 167 (55.4%) patients with PSC had concurrent inflammatory bowel disease (IBD)...
May 16, 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28509786/igg4-related-disease-of-the-biliary-tract-and-pancreas-clinical-and-experimental-advances
#4
Lowiek M Hubers, Ulrich Beuers
PURPOSE OF REVIEW: IgG4-related disease (IgG4-RD) is an immune-mediated disease of unknown cause. It predominantly affects the biliary tract [IgG4-associated cholangitis (IAC)] and pancreas [autoimmune pancreatitis (AIP)] of mostly elderly men. Accurate diagnostic tests are lacking. Patients benefit from predniso(lo)ne treatment. However, disease relapse is often seen. This review will address pathophysiological aspects and advances in diagnostic and therapeutic strategies. RECENT FINDINGS: The role of IgG1 and IgG4 in the pathophysiology of IgG4-RD was studied in mice which showed more intense organ damage of pancreas and salivary glands when IgG1 rather than IgG4 of patients with IgG4-RD was injected...
May 13, 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28506369/autoimmune-hepatitis-overlap-syndromes-and-liver-pathology
#5
REVIEW
Albert J Czaja, Herschel A Carpenter
Autoimmune hepatitis (AIH) may have an atypical serum alkaline phosphatase elevation, antimitochondrial antibodies, histologic features of bile duct injury/loss, or cholangiographic findings of focal biliary strictures and dilations. These manifestations characterize the overlap syndromes. Patients can be classified as having AIH with features of primary biliary cholangitis, primary sclerosing cholangitis, or a cholestatic syndrome. The gold standard of diagnosis is clinical judgment. Histologic evaluation is a major diagnostic component...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28503557/epidemiology-and-risk-factors-intrahepatic-cholangiocarcinoma
#6
REVIEW
Amar Gupta, Elijah Dixon
Intrahepatic cholangiocarcinoma (ICC) is a rare entity with a distinct clinical course and epidemiology from hilar and extrahepatic cholangiocarcinoma. ICC makes up 8-10% of cholangiocarcinomas and 10-20% of all primary liver tumors. There remains a considerable amount of geographic variation in the incidence of ICC worldwide; however, the overall incidence of this malignancy appears to be rising. Several risk factors have been identified, such as infectious causes (liver flukes, viral hepatitis), biliary tract disease [primary sclerosing cholangitis (PSC), hepaticolithiasis, biliary cystic diseases], metabolic syndrome, lifestyle choices (alcohol abuse, tobacco use), and cirrhosis...
April 2017: Hepatobiliary Surgery and Nutrition
https://www.readbyqxmd.com/read/28502477/knockdown-of-hepatic-gonadotropin-releasing-hormone-by-vivo-morpholino-decreases-liver-fibrosis-in-multidrug-resistance-gene-2-knockout-mice-by-down-regulation-of-mir-200b
#7
Konstantina Kyritsi, Fanyin Meng, Tianhao Zhou, Nan Wu, Julie Venter, Heather Francis, Lindsey Kennedy, Paolo Onori, Antonio Franchitto, Francesca Bernuzzi, Pietro Invernizzi, Kelly McDaniel, Romina Mancinelli, Domenico Alvaro, Eugenio Gaudio, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis occurs during the progression of primary sclerosing cholangitis (PSC) and is characterized by accumulation of extracellular matrix proteins. Proliferating cholangiocytes and activated hepatic stellate cells (HSCs) participate in the promotion of liver fibrosis during cholestasis. Gonadotropin-releasing hormone (GnRH) is a trophic peptide hormone synthesized by hypothalamic neurons and the biliary epithelium and exerts its biological effects on cholangiocytes by interaction with the receptor subtype (GnRHR1) expressed by cholangiocytes and HSCs...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28501828/vedolizumab-a-novel-medical-intervention-in-the-treatment-of-primary-sclerosing-cholangitis
#8
Donevan Westerveld, Joseph Grajo, Lars Beattie, Sarah Glover
No abstract text is available yet for this article.
May 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28497760/the-intestinal-microbiome-and-paediatric-liver-disease
#9
REVIEW
Daniel H Leung, Dean Yimlamai
The intestinal microbiome has been the intense focus of recent study, but how the microbiota affects connected organs, such as the liver, has not been fully elucidated. The microbiome regulates intestinal permeability and helps to metabolise the human diet into small molecules, thus directly affecting liver health. Several studies have linked intestinal dysbiosis to the severity and progression of liver diseases, such as non-alcoholic fatty liver disease, non-alcoholic steatohepatitis, primary sclerosing cholangitis, total parenteral nutrition-associated liver disease, and cystic fibrosis-associated liver disease...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28494557/-clinicopathologic-features-of-drug-induced-vanishing-bile-duct-syndrome
#10
L H Ye, C K Wang, H C Zhang, Z Q Liu, H W Zheng
Vanishing bile duct syndrome (VBDS) manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury (D-VBDS) is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings...
April 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28479483/coffee-and-autoimmunity-more-than-a-mere-hot-beverage
#11
REVIEW
Kassem Sharif, Abdulla Watad, Nicola Luigi Bragazzi, Mohammad Adawi, Howard Amital, Yehuda Shoenfeld
Coffee is one of the world's most consumed beverage. In the last decades, coffee consumption has attracted a huge body of research due to its impact on health. Recent scientific evidences showed that coffee intake could be associated with decreased mortality from cardiovascular and neurological diseases, diabetes type II, as well as from endometrial and liver cancer, among others. In this review, on the basis of available data in the literature, we aimed to investigate the association between coffee intake and its influence on the immune system and the insurgence of the most relevant autoimmune diseases...
May 4, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28468018/the-gut-microbiota-and-the-hepatologist-will-our-bugs-prove-to-be-the-missing-link
#12
Mark J Pallen, Mohammed N Quraishi
The advent of next-generation sequencing has enabled in-depth analysis to study the composition and function of the gut microbiota in a culture-independent manner. Consequently, this has led to rapid interest in understanding the pathogenesis and progression of chronic liver disease in relation to perturbations of the gut microbiota. Animal models and human studies have demonstrated its crucial role in contributing to disease mechanisms in alcoholic and non-alcoholic liver disease and more recently in primary sclerosing cholangitis...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28467297/successful-hepatoatrial-anastomosis-during-a-consecutive-liver-retransplant-in-the-same-patient-shows-good-long-term-results-case-report-and-2-year-follow-up
#13
Konrad Kobryń, Paweł Nyckowski, Piotr Milkiewicz, Grzegorz Niewiński, Jolanta Piwowarska, Wojciech Figiel, Piotr Smoter, Michał Wasilewicz, Waldemar Patkowski, Marek Krawczyk
Liver retransplant is the last and only treatment for patients with irreversible graft failure. It is recognized as a high-risk procedure; thus surgical difficulties are multiplied with every successive liver transplant. Liver retransplant is a demanding technical procedure for the surgeon, with no guarantee of postoperative and long-term survival. Here, we report a 29-year-old male patient who underwent a liver transplant in April 2009 due to primary sclerosing cholangitis with overlapping autoimmune hepatitis...
May 3, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28465630/pathogenesis-and-clinical-spectrum-of-primary-sclerosing-cholangitis
#14
REVIEW
Neelam G Gidwaney, Swati Pawa, Kiron M Das
Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has been documented in the literature since 1867. This disease has a strong predilection for affecting men and can be seen in individuals as young as 2 years of age. PSC has a strong associated with inflammatory bowel disease, more commonly with ulcerative colitis, and is also part of the clinical spectrum of IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association with inflammatory bowel disease. The exact pathogenesis of PSC is not well understood at present, however, is likely a combination of a genetic predisposition with alteration of the molecular structure of the gut...
April 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28461191/predictors-of-the-clinical-course-of-primary-sclerosing%C3%A2-cholangitis
#15
EDITORIAL
Evangelos Kalaitzakis, Einar S Björnsson
No abstract text is available yet for this article.
April 28, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28457384/liver-retransplantation-for-hepatic-abscess-due-to-hepatic-artery-thrombosis-a-case-report
#16
G Zanus, M Romano, M Finotti, E Dalla Bona, D Sgarabotto, D Bassi, C Mescoli, P Angeli, P Burra, E Gringeri, A Vitale, F D'Amico, P Feltracco, U Cillo
INTRODUCTION: Hepatic artery thrombosis (HAT) is a well-recognized complication of liver transplantation (LT). HAT is an important risk factor for infectious, in particular hepatic abscess, which can cause graft loss and increasing morbidity and mortality. CASE REPORT: We present a case report of complicated LT in a 52-year-old Caucasian man with primary sclerosing cholangitis. In 2007 the patient was included on the waiting list in Padua for LT. In 2012 the patient underwent percutaneous transhepatic biliary drainage for bile duct stricture, complicated with acute pancreatitis...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28453918/value-of-brush-cytology-for-optimal-timing-of-liver-transplantation-in-primary-sclerosing-cholangitis
#17
Marko J Vannas, Sonja Boyd, Martti A Färkkilä, Johanna Arola, Helena Isoniemi
BACKGROUND AND AIMS: Primary sclerosing cholangitis is associated with a high risk of cholangiocarcinoma. Here, we investigated the value of surveillance for dysplasia using brush cytology, to determine the optimal timing of liver transplantation in primary sclerosing cholangitis. We compared our preoperative findings, with the final explanted liver histopathology. METHODS: 126 consecutive patients were transplanted for primary sclerosing cholangitis from 1984 to 2012...
May 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28450276/pregnancy-and-bile-acid-disorders
#18
Vanessa Pataia, Peter H Dixon, Catherine Williamson
During pregnancy, extensive adaptations in maternal metabolic and immunological physiology occur. Consequently, pre-existing disease may be exacerbated or attenuated and new disease susceptibility may be unmasked. Cholestatic diseases, characterized by a supraphysiological raise in bile acid levels, require careful monitoring during pregnancy. This review describes the latest advances in the knowledge of intrahepatic cholestasis of pregnancy (ICP), the commonest bile acid disorder specific to pregnancy, with a focus on the disease etiology and potential mechanisms of ICP-associated adverse pregnancy outcomes, including fetal demise...
April 27, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28441268/lack-of-efficacy-of-infliximab-in-the-treatment-of-primary-sclerosing-cholangitis-in-inflammatory-bowel-diseases-in-childhood
#19
Sabrina Cardile, Manila Candusso, Bronislava Papadatou, Fiammetta Bracci, Daniela Knafelz, Giuliano Torre
No abstract text is available yet for this article.
June 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28432731/association-between-progressive-hepatic-morphology-changes-on-serial-mr-imaging-and-clinical-outcome-in-primary-sclerosing-cholangitis
#20
Yu Xuan Kitzing, Siobhan A Whitley, Sara S Upponi, Brijesh Srivastava, Graeme Jm Alexander, David J Lomas
INTRODUCTION: Hepatic morphology changes are well described in Primary Sclerosing Cholangitis and characterised by a combination of atrophy and hypertrophy changes. This study investigates the relationship between progression of these changes over time and clinical outcome in patients with PSC. METHODS: Fifty-three patients with PSC (mean age 44, 28 males and 25 females) who underwent serial MRI liver studies at least one year apart were identified. The first and the last MRI studies were selected for the retrospective analysis...
April 22, 2017: Journal of Medical Imaging and Radiation Oncology
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