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Primary biliary cirrhosis

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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/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#3
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
#4
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/28407375/h19-promotes-cholestatic-liver-fibrosis-by-preventing-zeb1-mediated-inhibition-of-epcam
#5
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/28400566/serum-metabolomics-analysis-reveals-a-distinct-metabolic-profile-of-patients-with-primary-biliary-cholangitis
#6
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
#7
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/28388943/time-dependent-roc-curve-analysis-in-medical-research-current-methods-and-applications
#8
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
#9
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/28371104/old-and-new-treatments-for-primary-biliary-cholangitis
#10
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
#11
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
#12
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/28352320/fungal-infection-involvement-in-primary-biliary-cirrhosis-a-review-of-2-cases
#13
Yanyan Wang, Zheng Zhao, Hui Lu, Jianglin Zhang, Feng Huang
The present study aimed to analyze the imaging, clinical and pathological features of fungal infection involvement in primary biliary cirrhosis (PBC) by retrospectively analyzing and reviewing the features of two patients with fungal infection involvement in PBC. Both patients were female. One patient had a confirmed diagnosis of PBC. The other patient had confirmed Sjogren syndrome and PBC. The two cases of PBC were infected with fungal infection after treatment with hormonal and immunosuppressive agents. RCR of sputum confirmed Pneumocystis spp...
February 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28350426/pharmacological-interventions-for-primary-biliary-cholangitis-an-attempted-network-meta-analysis
#14
REVIEW
Francesca Saffioti, Kurinchi Selvan Gurusamy, Leonardo Henry Eusebi, Emmanuel Tsochatzis, Brian R Davidson, Douglas Thorburn
BACKGROUND: Primary biliary cholangitis (previously primary biliary cirrhosis) is a chronic liver disease caused by the destruction of small intra-hepatic bile ducts resulting in stasis of bile (cholestasis), liver fibrosis, and liver cirrhosis. The optimal pharmacological treatment of primary biliary cholangitis remains uncertain. OBJECTIVES: To assess the comparative benefits and harms of different pharmacological interventions in the treatment of primary biliary cholangitis through a network meta-analysis and to generate rankings of the available pharmacological interventions according to their safety and efficacy...
March 28, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28333129/protection-against-oxidative-stress-mediated-by-the-nrf2-keap1-axis-is-impaired-in-primary-biliary-cholangitis
#15
Urszula Wasik, Małgorzata Milkiewicz, Agnieszka Kempinska-Podhorodecka, Piotr Milkiewicz
In response to oxidative stress, nuclear factor (erythroid-derived 2)-like2 (Nrf2) induces expression of cytoprotective genes. The Nrf2 pathway is controlled by microRNAs and Kelch-like ECH-associated protein1 (Keap1). Nrf2 is stabilized when Keap1 is degraded through the autophagy pathway in a p62-dependent manner. The inhibition of autophagy causes protein accumulation, and Keap1 is inactivated by binding to p62. We investigated the role of the Nrf2/Keap1 axis in the amelioration of oxidative stress in primary biliary cholangitis (PBC)...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28299343/polymorphisms-of-il12rb2-may-affect-the-natural-history-of-primary-biliary-cholangitis-a-single-centre-study
#16
Urszula Wasik, Ewa Wunsch, Gary L Norman, Eirini I Rigopoulou, Dimitrios P Bogdanos, Piotr Milkiewicz, Małgorzata Milkiewicz
Background. Recent GWAS in primary biliary cholangitis (PBC) showed strong associations with SNPs located within interleukin-12 receptor (IL12R) beta-2 (IL12RB2) gene. Aims. We assessed whether genetic variation of IL12RB2 is associated with laboratory and clinical features of PBC. Methods. Genomic DNA was isolated from 306 patients with PBC and 258 age/gender-matched controls. PBC-specific anti-mitochondrial antibodies (AMA) were tested in all subjects by ELISA. Two SNPs, rs3790567 and rs6679356, of IL12RB2 were genotyped using the MGB-TaqMan SNP assay...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28275240/polygenic-risk-assessment-reveals-pleiotropy-between-sarcoidosis-and-inflammatory-disorders-in-the-context-of-genetic-ancestry
#17
C A Lareau, C F DeWeese, I Adrianto, C J Lessard, P M Gaffney, M C Iannuzzi, B A Rybicki, A M Levin, C G Montgomery
Sarcoidosis is a complex disease of unknown etiology characterized by the presence of granulomatous inflammation. Though various immune system pathways have been implicated in disease, the relationship between the genetic determinants of sarcoidosis and other inflammatory disorders has not been characterized. Herein, we examined the degree of genetic pleiotropy common to sarcoidosis and other inflammatory disorders to identify shared pathways and disease systems pertinent to sarcoidosis onset. To achieve this, we quantify the association of common variant polygenic risk scores from nine complex inflammatory disorders with sarcoidosis risk...
March 2017: Genes and Immunity
https://www.readbyqxmd.com/read/28263464/assessment-of-hepatic-impairment-and-implications-for-pharmacokinetics-of-substance-use-treatment
#18
Andrew H Talal, Charles S Venuto, Islam Younis
Although the liver is the primary site of metabolism and biliary excretion for many medications, data are limited on the liver's pharmacokinetic abilities in cirrhosis. Cirrhosis develops through collagen deposition, eventually culminating in end-stage liver disease that compromises hepatic drug metabolism. Consequently, the US Food and Drug Administration (FDA) recommends evaluating the pharmacokinetics of medications in subjects with hepatic impairment if hepatic metabolism constitutes more than 20% of their elimination or if they have a narrow therapeutic range...
March 2017: Clinical Pharmacology in Drug Development
https://www.readbyqxmd.com/read/28263100/decreased-serum-dnase1-activity-in-patients-with-autoimmune-liver-diseases
#19
Nikolaos K Gatselis, Aigli G Vakrakou, Kalliopi Zachou, Theodoros Androutsakos, Kalliopi Azariadis, Gregorios Hatzis, Menelaos N Manoussakis, George N Dalekos
Deoxyribonuclease1 (DNase1) is involved in chromatin degradation of apoptotic cells. Its deficiency results in accumulation of self-DNA, which in turn may induce inflammation and autoimmunity. We assessed for the first time serum DNase1-activity in a large consecutive cohort of treatment-naïve patients with autoimmune liver diseases (ALD). DNase1-activity was determined by single radial enzyme-diffusion (SRED) at diagnosis of 224 patients with autoimmune hepatitis (AIH), 249 with primary biliary cirrhosis (PBC) and 36 with primary sclerosing cholangitis (PSC)...
March 2017: Autoimmunity
https://www.readbyqxmd.com/read/28261290/prediction-of-oesophageal-varices-in-patients-with-primary-biliary-cirrhosis-by-non-invasive-markers
#20
Lili Gao, Fanping Meng, Jun Cheng, Hanwei Li, Jun Han, Weihui Zhang
INTRODUCTION: Preliminary data suggested that non-invasive methods could be useful to assess presence of oesophageal varices (OV) in liver cirrhosis. The primary objectives were to investigate non-invasive markers for diagnosing and grading OV in patients with primary biliary cirrhosis. MATERIAL AND METHODS: This study included a total of 106 consecutive treatment-naive patients with primary biliary cirrhosis (PBC). Results of physical examination, blood tests, and abdominal ultrasound scan (USS) were measured...
March 1, 2017: Archives of Medical Science: AMS
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