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https://www.readbyqxmd.com/read/28447402/is-extra-glandular-organ-damage-in-primary-sj%C3%A3-gren-s-syndrome-related-to-the-presence-of-systemic-auto-antibodies-and-or-hypergammaglobulinemia-a-long-term-cohort-study-with-110-patients-from-the-netherlands
#1
Evert-Jan Ter Borg, Johannes Cornelis Kelder
AIM: To test the hypothesis that systemic auto-antibodies or hypergammaglobulinemia are related to the prevalence of extra-glandular tissue organ damage (EGOD) in primary Sjögren's syndrome (SS). METHODS: A real practice-based investigation of a relatively large (n = 110) Dutch cohort of primary SS patients systematically followed up in a large non-academic hospital. RESULTS: After a follow up of mean 8.2 years a significant correlation was found between disease duration and the prevalence of EGOD...
April 26, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28444987/platelet-count-spleen-length-and-platelet-count-to-spleen-length-ratio-for-the-diagnosis-of-oesophageal-varices-in-people-with-chronic-liver-disease-or-portal-vein-thrombosis
#2
REVIEW
Agostino Colli, Juan Cristóbal Gana, Jason Yap, Thomasin Adams-Webber, Natalie Rashkovan, Simon C Ling, Giovanni Casazza
BACKGROUND: Current guidelines recommend screening of people with oesophageal varices via oesophago-gastro-duodenoscopy at the time of diagnosis of hepatic cirrhosis. This requires that people repeatedly undergo unpleasant invasive procedures with their attendant risks, although half of these people have no identifiable oesophageal varices 10 years after the initial diagnosis of cirrhosis. Platelet count, spleen length, and platelet count-to-spleen length ratio are non-invasive tests proposed as triage tests for the diagnosis of oesophageal varices...
April 26, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28433114/hepatic-features-of-wilson-disease
#3
Salih Boga, Aftab Ala, Michael L Schilsky
In Wilson disease (WD) defective AT7B function leads to biliary copper excretion and pathologic copper accumulation, particularly in liver and brain, where it induces cellular damage. Liver disease most often precedes neurologic or psychiatric manifestations. In most patients with neurologic or psychiatric symptoms there is some degree of liver disease at the time of disease presentation. Hepatic manifestations of WD can be extremely variable. Patients with clinically asymptomatic WD are often found by family screening or identified on routine laboratory testing...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#4
Gideon M Hirschfield, Ulrich Beuers, Christophe Corpechot, Pietro Invernizzi, David Jones, Marco Marzioni, Christoph Schramm
Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in end-stage biliary cirrhosis. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse and risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the amelioration of associated symptoms...
April 17, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28416166/two-year-outcomes-of-balloon-occluded-retrograde-transvenous-obliteration-of-gastric-varices-in-liver-transplant-recipients-a-multi-institutional-study
#5
W E Saad, J F B Chick, R N Srinivasa, N Saad, S Kim, A Fischman, G Frey, A Al-Osaimi, S Caldwell
PURPOSE: To assess the 2-year effectiveness and safety of balloon-occluded retrograde transvenous obliteration (BRTO) for gastric varices (GVs) in liver transplant recipients. MATERIALS AND METHODS: Eleven liver transplant recipients underwent consecutive BRTO for GVs at four institutions. Patients included eight (73%) men and three (27%) women with mean age of 56 years±12 (SD) (range: 26-67 years). Underlying cause of liver transplantation was hepatitis C virus (HCV)-related cirrhosis in five (45%), alcohol- and HCV-related cirrhosis in three (27%), primary biliary cirrhosis in two (18%), and alcoholic cirrhosis in one (9%)...
April 13, 2017: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/28413649/hazardous-factors-besides-infection-in-hypoglycemia
#6
Yu-Jang Su, Yen-Chun Lai, Chia-Jung Liao
Hypoglycemia is one of the most common issues encountered in daily emergency practice. In addition to the treatment of hypoglycemia, certain other situations concomitant with hypoglycemia require further treatment. The aim of the present study was to compare demographic and clinical characteristics of infected [urinary tract infection (UTI), pneumonia or biliary tract infection (BTI)] vs. non-infected hypoglycemic patients to establish which hypoglycemic patients required further observation or hospitalization...
April 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28407375/h19-promotes-cholestatic-liver-fibrosis-by-preventing-zeb1-mediated-inhibition-of-epcam
#7
Yongfeng Song, Chune Liu, Xia Liu, Jocelyn Trottier, Michele Beaudoin, Li Zhang, Chad Pope, Guangyong Peng, Olivier Barbier, Xiaobo Zhong, Linheng Li, Li Wang
Based on our recent finding that disruption of bile acid (BA) homeostasis in mice results in the induction of hepatic lncRNA H19 expression, we sought to elucidate the role of H19 in cholestatic liver fibrosis. Hepatic overexpression of H19RNA augmented bile duct ligation (BDL)-induced liver fibrosis, which was accompanied by the elevation of serum ALT, AST, bilirubin, and BA levels. Multiple genes related to liver fibrosis, inflammation, and biliary hyperplasia were increased in H19-BDL vs Null-BDL mice, whereas genes in BA synthesis were decreased...
April 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28407283/acoustic-radiation-force-impulse-imaging-for-assessing-liver-fibrosis-preoperatively-in-infants-with-biliary-atresia-comparison-with-liver-fibrosis-biopsy-pathology
#8
Fen Gao, Ya-Qing Chen, Jing Fang, Sheng-Li Gu, Luan Li, Xiao-Ying Wang
OBJECTIVES: The purpose of this study was to investigate the diagnostic performance of acoustic radiation force impulse (ARFI) in assessing liver fibrosis preoperatively in infants with biliary atresia (BA). METHODS: A total of 50 consecutive infants with BA and 50 healthy infants who underwent ARFI examination were recruited. Siemens Acuson S2000 in Virtual Touch Quantification mode (Siemens Medical Solutions, Mountain View, CA) was used to measure shear wave speeds (SWSs)...
April 13, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28405682/apamin-suppresses-biliary-fibrosis-and-activation-of-hepatic-stellate-cells
#9
Jung-Yeon Kim, Hyun-Jin An, Woon-Hae Kim, Yoon-Yub Park, Kyung Duck Park, Kwan-Kyu Park
Cholestatic liver disease is characterized by the progressive destruction of biliary epithelial cells (BECs) followed by fibrosis, cirrhosis and liver failure. Activated hepatic stellate cells (HSCs) and portal fibroblasts are the major cellular effectors of enhanced collagen deposition in biliary fibrosis. Apamin, an 18 amino acid peptide neurotoxin found in apitoxin (bee venom), is known to block Ca2+-activated K+ channels and prevent carbon tetrachloride-induced liver fibrosis. In the present study, we aimed to ascertain whether apamin inhibits biliary fibrosis and the proliferation of HSCs...
May 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28400566/serum-metabolomics-analysis-reveals-a-distinct-metabolic-profile-of-patients-with-primary-biliary-cholangitis
#10
Juan Hao, Tao Yang, Yang Zhou, Guo-Yuan Gao, Feng Xing, Yuan Peng, Yan-Yan Tao, Cheng-Hai Liu
Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease associated with profound metabolic changes. The purpose of this study was to identify a distinctive metabolic signature from the training set with 29 PBC patients, 30 hepatitis B virus (HBV)-caused cirrhosis (HBC) and 41 healthy controls, and to validate the applicability and stability of the distinctive model from the validation set with 21 PBC patients, 7 autoimmune hepatitis (AIH) and 9 HBC. The sera were investigated using high resolution nuclear magnetic resonance (NMR) and the datasets were analyzed pairwise using pattern recognition methods...
April 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28395724/-single-nucleotide-polymorphism-of-stat4-rs7574865-is-associated-with-the-susceptibility-of-primary-biliary-cirrhosis-in-han-population-of-partial-regions-of-jiangsu-province
#11
Liming Zheng, Hong Zhou
Objective To investigate the correlation of single nucleotide polymorphism (SNP) of signal transducer and activator of transcription 4 (STAT4) rs7574865 gene with primary biliary cirrhosis (PBC) in Han population of Jiangsu province. Methods The peripheral blood samples were collected from 138 inpatients with PBC and 116 unrelated healthy donors in the Third People's Hospital of Changzhou City in Jiangsu province. The STAT4 rs7574865 SNP was determined by polymerase chain reaction-sequence specific primer (PCR-SSP) assay...
April 2017: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
https://www.readbyqxmd.com/read/28393104/safety-of-ercp-in-patients-with-liver-cirrhosis-a-national-database-study
#12
Udayakumar Navaneethan, Basile Njei, Xiang Zhu, Kiran Kommaraju, Mansour A Parsi, Shyam Varadarajulu
Background and aims Given the limited data on the safety of endoscopic retrograde cholangiopancreatography (ERCP) in patients with liver cirrhosis, we attempted to evaluate this question using a large national database. Methods We conducted a matched case - control study using the 2010 National Inpatient Sample database in which four non-cirrhotic controls were matched randomly for every cirrhotic patient from the same 10-year age group. We compared adverse events and safety of inpatient ERCP between patients with (n = 3228) and without liver cirrhosis (controls, n = 12 912)...
April 2017: Endoscopy International Open
https://www.readbyqxmd.com/read/28388943/time-dependent-roc-curve-analysis-in-medical-research-current-methods-and-applications
#13
Adina Najwa Kamarudin, Trevor Cox, Ruwanthi Kolamunnage-Dona
BACKGROUND: ROC (receiver operating characteristic) curve analysis is well established for assessing how well a marker is capable of discriminating between individuals who experience disease onset and individuals who do not. The classical (standard) approach of ROC curve analysis considers event (disease) status and marker value for an individual as fixed over time, however in practice, both the disease status and marker value change over time. Individuals who are disease-free earlier may develop the disease later due to longer study follow-up, and also their marker value may change from baseline during follow-up...
April 7, 2017: BMC Medical Research Methodology
https://www.readbyqxmd.com/read/28373632/primary-biliary-cholangitis-its-pathological-characteristics-and-immunopathological-mechanisms
#14
Koichi Tsuneyama, Hayato Baba, Yuki Morimoto, Takaaki Tsunematsu, Hirohisa Ogawa
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease that predominantly affects middle-aged women and is characterized by the chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and, ultimately, fibrosis. The serological hallmark of PBC is the presence of anti-mitochondrial autoantibodies (AMA). Several mechanisms have been proposed for immune-mediated bile duct damage in PBC, including the roles of T cells, B cells, other cell phenotypes, and AMA...
2017: Journal of Medical Investigation: JMI
https://www.readbyqxmd.com/read/28372450/a-bronchobiliary-fistula-treated-with-cyanoacrylate-glue-in-a-patient-with-liver-cirrhosis
#15
Raúl Honrubia López, Ana Barbado Cano, Gloria Ruiz Fernández, Katherine Yelenia Bustamante Robles
A 60-year-old male was operated upon in 2002 for liver hydatidosis, which included partial right hepatectomy with cholecystectomy and bilioduodenal anastomosis. He then developed liver cirrhosis secondary to left hepatic duct stricture. He presents at the emergency room with dry cough, which he had for a month and then became associated with yellowish, bitter-tasting "fluid" expectoration. A chest-abdominal CT scan revealed a 6 x 5-cm collection roughly located somewhere between the middle pulmonary lobe and subphrenic area ...
April 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28371104/old-and-new-treatments-for-primary-biliary-cholangitis
#16
David Chascsa, Elizabeth J Carey, Keith D Lindor
Primary biliary cholangitis (formerly primary biliary cirrhosis) is a rare progressive cholestatic liver disease, whose hallmark features include a persistently elevated alkaline phosphatase level, presence of anti-mitochondrial antibodies and characteristic histology. Since 1998, ursodeoxycholic acid (UDCA), a bile acid, has been the only available therapeutic agent. Primary biliary cholangitis is associated with the development of end-stage liver disease, increased morbidity and mortality. UDCA has been shown to improve serum biochemistries, histology and delay the need for liver transplantation...
April 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28365879/geoepidemiology-and-changing-mortality-in-primary-biliary-cholangitis
#17
REVIEW
Annarosa Floreani, Atsushi Tanaka, Christopher Bowlus, Merrill Eric Gershwin
Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic cholestatic disease characterized by an autoimmune-mediated destruction of small and medium-sized intrahepatic bile ducts. Originally PBC was considered to be rare and almost invariably fatal, mainly because the diagnosis was made in patients presenting with advanced symptomatic disease (jaundice and decompensated cirrhosis). However, the development of a reproducible indirect immunofluorescence assay for antimitochondrial antibody made it possible to diagnose the disease at an earlier stage, and introduction of ursodeoxycholic acid therapy as the first-line therapy for PBC drastically changed PBC-related mortality...
April 1, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/28363639/increased-expression-of-ctla4-by-t-cells-induced-by-b7-in-sera-reduces-adaptive-immunity-in-patients-with-acute-liver-failure
#18
Wafa Khamri, Robin D Abeles, Tie Zheng Hou, Amy E Anderson, Ahmed El-Masry, Evangelos Triantafyllou, Christine Bernsmeier, Fin S Larsen, Arjuna Singanayagam, Nobuaki Kudo, Lucia A Possamai, Fanny Lebosse, Georg Auzinger, William Bernal, Christopher Willars, Christopher J Weston, Giovanna Lombardi, Julia Wendon, Mark Thursz, Charalambos G Antoniades
BACKGROUND & AIMS: Patients with acute liver failure (ALF) have defects in innate immune responses to microbes (immuneparesis) and are susceptible to sepsis. Cytotoxic T-lymphocyte associated protein 4 (CTLA4), which interacts with the membrane receptor B7 (also called CD80 and CD86), is a negative regulator of T-cell activation. We collected T cells from patients with ALF and investigated whether inhibitory signals downregulate adaptive immune responses in patients with ALF. METHODS: We collected peripheral blood mononuclear cells from patients with ALF and controls from September 2013 through September 2015 (45 patients with ALF, 20 patients with acute-on-chronic liver failure, 15 patients with cirrhosis with no evidence of acute decompensation, 20 patients with septic shock but no cirrhosis or liver disease, and 20 healthy individuals)...
March 28, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28359183/changes-in-drug-transport-and-metabolism-and-their-clinical-implications-in-non-alcoholic-fatty-liver-disease
#19
Christoph G Dietrich, Monika Rau, Daniel Jahn, Andreas Geier
The incidence of non-alcoholic fatty liver disease (NAFLD) is rising, especially in Western countries. Drug treatment in patients with NAFLD is common since it is linked to other conditions like diabetes, obesity, and cardiovascular disease. Consequently, changes in drug metabolism may have serious clinical implications. Areas covered: A literature search for studies in animal models or patients with obesity, fatty liver, non-alcoholic steatohepatitis (NASH) or NASH cirrhosis published before November 2016 was performed...
March 31, 2017: Expert Opinion on Drug Metabolism & Toxicology
https://www.readbyqxmd.com/read/28358366/forkhead-box-a3-attenuated-the-progression-of-fibrosis-in-a-rat-model-of-biliary-atresia
#20
Rui Dong, Yifan Yang, Zhen Shen, Chao Zheng, Zhu Jin, Yanlei Huang, Zhien Zhang, Shan Zheng, Gong Chen
Biliary atresia is a rare, devastating disease of infants where a fibroinflammatory process destroys the bile ducts, leading to fibrosis and biliary cirrhosis, and death if untreated. The cause and pathogenesis remain largely unknown. We tried to investigate factors involved in biliary atresia, especially forkhead box A3 (Foxa3), which might exert a role in the treatment of liver disease. We used RNA sequencing to sequence the whole transcriptomes of livers from six biliary atresia and six choledochal cysts patients...
March 30, 2017: Cell Death & Disease
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