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Sclerosing cholangitis

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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
#1
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, Robert A de Man, Herold J Metselaar, Annemarie C de Vries
Studies from the United States 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
#2
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
#3
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/29146216/-nisch-syndrome-a-rare-cause-of-neonatal-cholestasis-a-case-report
#4
S Szepetowski, C Lacoste, S Mallet, B Roquelaure, C Badens, A Fabre
NISCH syndrome is a rare autosomal recessive disease. It is characterized by scalp hypotrichosis, scarring alopecia, ichthyosis, and neonatal sclerosing cholangitis. It is caused by mutations in the CLDN1 gene encoding the claudin-1 protein, which is located at tight junctions. Fifteen cases have been reported to date and three different mutations have been identified. We report on the case of a 2-year-old boy from a consanguineous Moroccan family, presenting with NISCH syndrome and carrying the so-called Moroccan homozygous mutation (c...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29138587/primary-sclerosing-cholangitis-diagnostic-and-management-challenges
#5
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
#6
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
#7
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/29112085/diagnosis-and-management-of-paediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#8
Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann, Piotr Czubkowski, Dominique Debray, Antal Dezsofi, Björn Fischler, Girish Gupte, Loreto Hierro, Giuseppe Indolfi, Jörg Jahnel, Françoise Smets, Henkjan J Verkade, Nedim Hadzic
Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2)...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29106505/successful-treatment-with-vedolizumab-in-a-patient-with-chronic-refractory-pouchitis-and-primary-sclerosing-cholangitis
#9
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-multi-center-north-american-consortium
#10
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 1, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29094322/usefulness-of-laparoscopy-and-intraductal-ultrasonography-in-a-patient-with-isolated-immunoglobulin-g4-related-sclerosing-cholangitis
#11
Yoshinori Ohno, Teru Kumagi, Yoshiki Imamura, Taira Kuroda, Mitsuhito Koizumi, Takao Watanabe, Osamu Yoshida, Yoshio Tokumoto, Eiji Takeshita, Masanori Abe, Kenichi Harada, Yoichi Hiasa
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is often associated with type 1 autoimmune pancreatitis, and the frequency of isolated IgG4-SC seems to be quite low, making the diagnosis of isolated IgG4-SC challenging. A 63-year-old male was admitted to our hospital for frequent fever. Abdominal magnetic resonance cholangiopancreatography showed diffuse narrowing of the common bile duct and post-stenotic dilatation of the right posterior bile duct. Laboratory tests showed abnormalities in the levels of hepatobiliary enzymes and serum IgG4 levels...
November 1, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29089545/activation-of-fas-fasl-pathway-and-the-role-of-c-flip-in-primary-culture-of-human-cholangiocarcinoma-cells
#12
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
#13
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
#14
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
#15
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)
https://www.readbyqxmd.com/read/29067151/primary-sclerosing-cholangitis-and-the-management-of-uncertainty-and-complexity
#16
REVIEW
Katherine Arndtz, Gideon M Hirschfield
Primary sclerosing cholangitis (PSC) is a rare chronic autoimmune disease with no effective therapy and a large unmet need for new treatments. Patients require significant healthcare resources over their lifetime with high rates of hospital admission, malignancy, liver transplantation and death. As a rare disease, expertise in management can be limited to large referral liver transplant programmes, and even then there is frequently variation in practice. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing patients with PSC and address related competencies in the 2010 Gastroenterology Curriculum...
October 2017: Frontline Gastroenterology
https://www.readbyqxmd.com/read/29064885/transplantation-versus-resection-for-hilar-cholangiocarcinoma-an-argument-for-shifting-treatment-paradigms-for-resectable-disease
#17
Cecilia G Ethun, Alexandra G Lopez-Aguiar, Douglas J Anderson, Andrew B Adams, Ryan C Fields, Maria B Doyle, William C Chapman, Bradley A Krasnick, Sharon M Weber, Joshua D Mezrich, Ahmed Salem, Timothy M Pawlik, George Poultsides, Thuy B Tran, Kamran Idrees, Chelsea A Isom, Robert C G Martin, Charles R Scoggins, Perry Shen, Harveshp D Mogal, Carl Schmidt, Eliza Beal, Ioannis Hatzaras, Rivfka Shenoy, Kenneth Cardona, Shishir K Maithel
OBJECTIVE: To investigate the influence of type of surgery (transplant vs resection) on overall survival (OS) in patients with hilar cholangiocarcinoma (H-CCA). BACKGROUND: Outcomes after resection for H-CCA are poor, yet transplantation is currently only reserved for well-selected patients with unresectable disease. METHODS: All patients with H-CCA who underwent resection from 2000 to 2015 at 10 institutions were included. Three institutions additionally had active H-CCA transplant protocols with similar selection criteria over similar time periods...
October 23, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/29064110/colectomy-prior-to-diagnosis-of-primary-sclerosing-cholangitis-is-associated-with-improved-prognosis-in-a-nationwide-cohort-study-of-2594-psc-ibd-patients
#18
C Nordenvall, O Olén, P J Nilsson, E von Seth, A Ekbom, M Bottai, P Myrelid, A Bergquist
BACKGROUND: Despite the close relationship between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), the association between colectomy and the prognosis of PSC remains controversial. AIM: To explore whether colectomy prior to PSC-diagnosis is associated with transplant-free survival. METHODS: A nationwide cohort study in Sweden including all patients aged 18 to 69 years in whom both PSC and IBD was diagnosed between 1987 and 2014 was undertaken...
October 24, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29059700/a-favorable-response-to-the-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#19
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
AIM: Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine anti-metabolic, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we performed a preliminary clinical study in children with PSC. METHODS: Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29056396/serology-in-autoimmune-hepatitis-a-clinical-practice-approach
#20
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Serology is key to the diagnosis of autoimmune hepatitis (AIH). Clinicians need to be aware of which tests to request, how to interpret the laboratory reports, and be familiar with the laboratory methodology. If correctly tested, >95% of AIH patients show some serological reactivity. Indirect immunofluorescence on triple rodent tissue is recommended as first screening step, since it allows the detection of all liver-relevant autoantibodies, except for anti-soluble liver antigen (SLA) antibody, which needs to be detected by molecular based assays...
October 19, 2017: European Journal of Internal Medicine
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