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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/29333790/crohn-s-disease-behavior-and-location-is-altered-when-associated-with-primary-sclerosing-cholangitis
#1
Oren Iny, Henit Yanai, Shay Matalon, Erwin Santo, Oren Shibolet, Iris Dotan, Nitsan Maharshak
BACKGROUND: Up to 3.4% of Crohn's disease (CD) patients will be diagnosed with concomitant primary sclerosing cholangitis (PSC). Despite the worldwide increase incidence of CD, data on the clinical characteristics of PSC-CD patients are scarce. OBJECTIVES: To clinically characterize CD in patients who have concomitant PSC. METHODS: A retrospective case-control analysis was conducted with 18 CD patients with concomitant PSC who attended the Inflammatory Bowel Disease Center at the Tel Aviv Sourasky Medical Center between 2011-2014 (PSC-CD patients)...
January 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29331005/pregnant-lady-with-undiagnosed-hodgkin-s-disease-presenting-as-secondary-sclerosing-cholangitis
#2
Taif Khattak, Syed Hashim Ali Shah, Waqas Khan, Syed Shahmeer Raza, Uzair Ahmad, Farhan Ullah
Hodgkin's lymphoma. A 25 years old lady, 34 weeks primigravida was referred from the Emergency Department to the Medical Unit Khyber Teaching Hospital-MTI, Peshawar with four weeks of fever, progressive jaundice, pruritus, night sweats and weight loss. LFTs showed cholestatic picture, ERCP showed scanty intrahepatic giving beading and autumn tree appearance typical of sclerosing cholangitis. CBD was normal. Doppler U/S of hepatic and portal vein reported normal. She was started on steroids, ursodeoxycholic acid and antibiotics 3rd generation cephalosporins to which she did not respond well...
October 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29324588/both-%C3%AE-1-antitrypsin-z-phenotypes-and-low-caeruloplasmin-levels-are-over-represented-in-alcohol-and-nonalcoholic-fatty-liver-disease-cirrhotic-patients-undergoing-liver-transplant-in-ireland
#3
El-Gaily A El-Rayah, Patrick J Twomey, Eleanor M Wallace, Peter A McCormick
OBJECTIVES: Alcoholic liver disease and nonalcoholic fatty liver disease (NAFLD) are steatotic liver diseases and major causes of cirrhosis. Only a minority of patients with risk factors develop cirrhosis and genetic cofactors may be important in pathogenesis. Mutations in the Wilson's and α-1-antitrypsin genes are not uncommon and we speculated that they may act as cofactors. METHODS: We investigated α-1-antitrypsin phenotyes and caeruloplasmin levels in patients undergoing elective liver transplantation...
January 10, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29321484/loss-of-tolerance-to-gut-immunity-protein-glycoprotein-2-gp2-is-associated-with-progressive-disease-course-in-primary-sclerosing-cholangitis
#4
Tamas Tornai, David Tornai, Nora Sipeki, Istvan Tornai, Rayan Alsulaimani, Kai Fechner, Dirk Roggenbuck, Gary L Norman, Gabor Veres, Gabriella Par, Alajos Par, Ferenc Szalay, Peter Laszlo Lakatos, Peter Antal-Szalmas, Maria Papp
Glycoprotein 2[GP2] is a specific target of pancreatic autoantibodies[PAbs] in Crohn's disease(CD) and is involved in gut innate immunity processes. Our aim was to evaluate the prevalence and prognostic potential of PAbs in primary sclerosing cholangitis(PSC). Sixty-five PSC patients were tested for PAbs by indirect immunofluorescence and compared with healthy (n = 100) and chronic liver disease controls(CLD, n = 488). Additionally, a panel of anti-microbial antibodies and secretory (s)IgA levels were measured, as markers of bacterial translocation and immune dysregulation...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29317337/heart-and-bile-acids-clinical-consequences-of-altered-bile-acid-metabolism
#5
REVIEW
Tharni Vasavan, Elisa Ferraro, Effendi Ibrahim, Peter Dixon, Julia Gorelik, Catherine Williamson
Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart...
January 6, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#6
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age: 10...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29315689/quality-of-life-and-primary-sclerosing-cholangitis-the-business-of-defining-what-counts
#7
EDITORIAL
K Arndtz, G M Hirschfield
No abstract text is available yet for this article.
January 9, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29314129/editorial-the-role-of-colonic-inflammation-in-the-progression-of-liver-disease-in-primary-sclerosing-cholangitis-authors-reply-and-letter-the-effects-of-colectomy-prior-to-the-diagnosis-of-primary-sclerosing-cholangitis-on-prognosis-may-have-been-overestimated
#8
EDITORIAL
https://www.readbyqxmd.com/read/29314124/editorial-the-role-of-colonic-inflammation-in-the-progression-of-liver-disease-in-patients-with-primary-sclerosing-cholangitis
#9
EDITORIAL
J Sabino, J Torres
No abstract text is available yet for this article.
February 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29313399/localized-intrahepatic-igg4-related-sclerosing-cholangitis-igg4-sc-as-an-additional-type-of-igg4-sc-a-systematic-analysis-of-12-cases
#10
Zhichao Mo, Kuvaneshan Ramen, Yunfeng Shan, Mengtao Zhou, Xiangwu Zheng, Xiuling Wu, Zhengping Yu, Xiaoke Ji, Qiyu Zhang, Qiqiang Zeng
OBJECTIVES: IgG4-related sclerosing cholangitis (IgG4-SC), a recently defined disease entity, has been classified into four types based on the stricture regions revealed by cholangiography. However, localized intrahepatic IgG4-SC is not included into the classification. This study aimed to analyze and characterize localized intrahepatic IgG4-SC and justify the inclusion of this type into the classification. METHODS: PubMed and Embase were searched for studies published from March 2001 to June 2017 reporting localized intrahepatic IgG4-SC...
January 9, 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29312864/risk-factors-and-clinical-indicators-for-the-development-of-biliary-strictures-post-liver-transplant-significance-of-bilirubin
#11
Elizabeth Ann Forrest, Janske Reiling, Geraldine Lipka, Jonathan Fawcett
AIM: To identify risk factors associated with the formation of biliary strictures post liver transplantation over a period of 10-year in Queensland. METHODS: Data on liver donors and recipients in Queensland between 2005 and 2014 was obtained from an electronic patient data system. In addition, intra-operative and post-operative characteristics were collected and a logistical regression analysis was performed to evaluate their association with the development of biliary strictures...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29311406/the-changing-face-of-the-diagnosis-of-chronic-and-malignant-liver-diseases-potential-new-biomarkers
#12
Vania Cruz-Ramón, Paulina Chinchilla-López, Oscar Ramírez-Pérez, Xingshun Qi, Nahum Méndez-Sánchez
The early diagnosis of primary sclerosing cholangitis, hepatocellular carcinoma, and cholangiocarcinoma is often challenging. In a recent study in 134 patients (Arbelaiz, Hepatology 2017; 66:1125-1143), it was reported that specific proteins found in serum extracellular vesicles of patients with primary sclerosing cholangitis, hepatocellular carcinoma,orcholangiocarcinomamay be useful as noninvasive diagnostic and prognostic tools. This current article critically appraises this study.
December 27, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/29310470/new-therapies-target-the-toxic-consequences-of-cholestatic-liver-disease
#13
Peter L M Jansen
Introduction In most cholestatic liver diseases the primary cholestasis-causing lesions are located in the biliary tree and may be of (auto)immune origin. Bile salts are responsible for the secondary toxic consequences. Bile salt and nuclear hormone directed therapies primarily aim at improving this secondary toxic injury. In primary biliary cholangitis, trials show statistically significant responses on biochemical endpoints. Preclinical studies suggest that FXR- and PPAR-agonists, inhibitors of the apical sodium-dependent bile salt transporter (ASBT-inhibitors) and the C23 UDCA derivative nor-UDCA are promising agents for the treatment of primary sclerosing cholangitis (PSC)...
January 9, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29301479/risk-factors-and-prognosis-for-recurrent-primary-sclerosing-cholangitis-after-liver-transplantation-a-nordic-multicentre-study
#14
Lina Lindström, Kristin K Jørgensen, Kirsten M Boberg, Maria Castedal, Allan Rasmussen, Andreas Arendtsen Rostved, Helena Isoniemi, Matteo Bottai, Annika Bergquist
OBJECTIVES: The risk for recurrent primary sclerosing cholangitis (rPSC) after liver transplantation is associated with inflammatory bowel disease (IBD). We assessed the frequency of rPSC and studied risk factors for recurrent disease with special focus on IBD. We also evaluated the importance of rPSC for prognosis. MATERIALS AND METHODS: All liver transplanted PSC patients in the Nordic countries between 1984 and 2007 (n = 440), identified by the Nordic Liver Transplant Registry, were studied...
January 4, 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29282390/autoimmune-hepatitis-a-risk-factor-for-cholangiocarcinoma
#15
Rajat Garg, Uqba Khan, Ahmed AlRajjal, Zyad Kafri
Cholangiocarcinoma (CCA) is a very aggressive and lethal tumor, which arises from the epithelial cells of bile ducts. CCA comprises about 3% of all gastrointestinal malignancies and its incidence is on the rise in the recent years. Anatomically, it is classified into intrahepatic, perihilar, or extrahepatic (distal) CCA. There are a number of risk factors associated with CCA including primary sclerosing cholangitis, fibropolycystic liver disease, parasitic infection, viral hepatitis, chronic liver disease, and genetic disorders like Lynch syndrome...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29278002/cholestasis-after-tips-placement-in-a-patient-with-primary-sclerosing-cholangitis-an-uncommon-complication
#16
Alejandro Salagre García, Carolina Muñoz Codoceo, Elena Gómez Domínguez, Inmaculada Fernández Vázquez, Gregorio Castellano Tortajada
We present the case of a patient with primary sclerosing cholangitis who presented a rapidly evolving cholestatic syndrome, with severe hyperbilirubinemia after placing a TIPS. It was resolved with a biliary prosthesis inserted by ERCP. To date, no similar cases have been described in the literature, as it is a rare complication and, on the other hand, the experience with TIPS in patients with PSC is limited. The causes of hyperbilirubinemia after TIPS placement are very varied and it is crucial to perform a differential diagnosis to establish an early treatment...
December 26, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29272982/the-significance-of-serum-igg4-and-ca19-9-autoantibodies-in-diagnosis-and-differential-diagnosis-of-igg4-related-sclerosing-cholangitis
#17
Liming Tan, Xiaolin Guan, Tingting Zeng, Sifan Wu, Wei Zheng, Huiying Fu, Tingting Long, Qiaohua Wang, Yimei Meng, Yongjian Tian, Jianlin Yu, Juanjuan Chen, Hua Li, Liping Cao
OBJECTIVE: To investigate the value of serum levels of IgG4 and CA19-9, and autoantibodies in the diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC). METHODS: We detected the serum IgG4 and CA19-9 of 45 IgG4-SC patients, 173 non-IgG4-SC patients and 48 healthy controls by immunoassay and chemiluminescence, respectively, with antinuclear antibody (ANA), anti-neutrophil antibody (ANCA), anti-smooth muscle antibody (SMA) and anti-mitochondrial antibody (AMA) level detected by indirect immunofluorescence...
December 22, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29248458/hepatic-stem-progenitor-cell-activation-differs-between-primary-sclerosing-and-primary-biliary-cholangitis
#18
Guido Carpino, Vincenzo Cardinale, Trine Folseraas, Diletta Overi, Annarosa Floreani, Antonio Franchitto, Paolo Onori, Nora Cazzagon, Pasquale Bartolomeo Berloco, Tom Hemming Karlsen, Domenico Alvaro, Eugenio Gaudio
Primary sclerosing cholangitis and primary biliary cholangitis are human primary cholangiopathies; these diseases are characterized by the damage of mature cholangiocytes and by the appearance of ductular reaction as the results of hepatic progenitor cell activation. The aims of this study were to evaluate differences in progenitor cell niche activation between these two human cholangiopathies. Human liver tissue was obtained from normal liver donors (n=5), primary sclerosing (n=20), and primary biliary cholangitis (n=20)...
December 14, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/29244227/surveillance-for-hepatobiliary-cancers-in-patients-with-primary-sclerosing-cholangitis
#19
Ahmad Hassan Ali, James H Tabibian, Navine Naser-Ghodsi, Ryan J Lennon, Thomas DeLeon, Mitesh J Borad, Moira Hilscher, Marina G Silveira, Elizabeth J Carey, Keith D Lindor
BACKGROUND AND AIMS: Primary sclerosing cholangitis (PSC) is a risk factor for cholangiocarcinoma (CCA) and gallbladder carcinoma (GBCa). Surveillance for GBCa is recommended, but the clinical utility of surveillance for other hepatobiliary cancers (HBCa) in PSC, namely CCA and hepatocellular carcinoma (HCC), is unclear. We aimed to determine whether surveillance is associated with better survival after diagnosis of HBCa in patients with PSC. METHODS: Medical records of PSC patients seen at Mayo Clinic Rochester from 1995-2015 were reviewed...
December 15, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29234196/glycochenodeoxycholic-acid-does-not-increase-transforming-growth-factor-beta-expression-in-bile-duct-epithelial-cells-or-collagen-synthesis-in-myofibroblasts
#20
Anna Wang, Dorothy Yu, Yuewen Gong, Jessie Garber, Gerald Y Minuk
Background/Aims: Primary sclerosing cholangitis (PSC) is a chronic, progressive hepatobiliary disorder characterized by extensive fibrosis and stricturing of the intra- and/or extra-hepatic bile ducts: Previous studies have documented low phosphatylcholine (PC) concentrations in PSC bile. The aim of this study was to determine whether low PC levels in bile facilitate toxic bile acid induced injury of biliary tract epithelial cells resulting in enhanced transforming growth factor-beta (TGF-β) expression and increased collagen synthesis by myofibroblasts...
December 2017: Journal of Clinical and Experimental Hepatology
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