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https://www.readbyqxmd.com/read/29039048/sclerosing-and-obstructive-cholangiopathy-in-biliary-atresia-mechanisms-and-association-with-biliary-innate-immunity
#1
REVIEW
Kenichi Harada
Biliary atresia (BA) is histologically characterized by a progressive, sclerosing cholangitis and the obstruction of extrahepatic bile ducts. In terms of the etiology and pathogenesis of BA, several viral infections consisting of dsRNA, including Reoviridae, have been implicated. Human biliary epithelial cells (BECs) possess an innate immune system consisting of Toll-like receptors (TLRs). BECs have negative regulatory mechanisms of TLR tolerance to avoid an excessive inflammatory response to lipopolysaccharide (LPS), a TLR4 ligand; however, they lack the tolerance to poly(I:C) (a synthetic analog of viral dsRNA), a TLR3 ligand...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29026064/the-effect-of-recipient-body-mass-index-and-its-extremes-on-survival-and-graft-vascular-and-biliary-complications-after-liver-transplantation-a-single-center-retrospective-study
#2
Emmanouil Giorgakis, Michele Tedeschi, Eliano Bonaccorsi-Riani, Shirin Elizabeth Khorsandi, Krishna Menon, Hector Vilca-Melendez, Wayel Jassem, Parthi Srinivasan, Andreas Prachalias, Nigel Heaton
BACKGROUND This is the largest UK-based study on the effect of recipient body mass index (BMI) and its extremes (BMI <18.5 and BMI ≥35 kg/m²) on liver transplant (LT) outcomes. Its purpose was to analyze the BMI effect on post-LT mortality, graft loss, primary non-function (PNF), and graft vascular and biliary complications. MATERIAL AND METHODS Data were retrieved from a single-center LT database of 2,115 consecutive patients receiving first LT during period February 2004 to September 2015. Survivals were compared across the BMI groups; the effects of recipient BMI on survival, PNF, and graft vascular and biliary complications were analyzed via regression...
October 13, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/29023905/cholangiopathy-in-critically-ill-patients-surviving-beyond-the-intensive-care-period-a-multicentre-survey-in-liver-units
#3
L Laurent, C Lemaitre, A Minello, A Plessier, G Lamblin, A Poujol-Robert, A Gervais-Hasenknopf, E-A Pariente, P Belenotti, N Mostefa-Kara, P Sogni, M Legrand, J-M Cournac, F Tamion, G Savoye, P Bedossa, D-C Valla, V Vilgrain, O Goria
BACKGROUND: The outcome of cholangiopathy developing in intensive care unit (ICU) is not known in patients surviving their ICU stay. AIM: To perform a survey in liver units, in order to clarify the course of cholangiopathy after surviving ICU stay. METHODS: The files of the liver units affiliated to the French network for vascular liver disease were screened for cases of ICU cholangiopathy developing in patients with normal liver function tests on ICU admission, and no prior history of liver disease...
October 11, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29022092/unique-manifestations-of-biliary-atresia-provide-new-immunological-insight-into-its-etiopathogenesis
#4
REVIEW
Toshihiro Muraji, Haruo Ohtani, Satoshi Ieiri
Biliary atresia (BA) is a unique cholestatic disease of newborns with a background of exaggerated immune response in the liver of unknown mechanism. Three hypotheses have been proposed; autoimmune type of cholangiopathy triggered by virus infection, graft-versus-host disease type of immune-mediated disease associated with maternal microchimerism and ductal plate malformation theory. Researchers on virus infection theory have experimentally explored immune process causing cholangiopathy on murine models of this disease, while in maternal microchimerism hypothesis were detected maternal cells in the BA patients' liver, of which roles are yet to be determined...
October 11, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983725/liver-transplantation-for-biliary-atresia-a-systematic-review
#5
REVIEW
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shinji Uemoto
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28962898/role-of-the-bicarbonate-responsive-soluble-adenylyl-cyclase-in-cholangiocyte-apoptosis-in-primary-biliary-cholangitis-a-new-hypothesis
#6
REVIEW
Jung-Chin Chang, Simei Go, Arthur J Verhoeven, Ulrich Beuers, Ronald P J Oude Elferink
Primary biliary cholangitis (PBC) is a chronic fibrosing cholangiopathy characterized by an autoimmune stereotype and defective biliary bicarbonate secretion due to down-regulation of anion exchanger 2 (AE2). Despite the autoimmune features, immunosuppressants are ineffective while two bile acid-based therapies (ursodeoxycholic acid and obeticholic acid) have been shown to improve biochemical and histological features of cholestasis and long-term prognosis. However, the etiology and pathogenesis of PBC is largely unknown...
September 26, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28940041/a-review-of-long-term-outcome-and-quality-of-life-of-patients-after-kasai-operation-surviving-with-native-livers
#7
REVIEW
Kenneth K Y Wong, Carol W Y Wong
Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy...
September 22, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#8
Kyle Geary, Cemal Yazici, Anita Seibold, Grace Guzman
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy...
September 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28885771/impact-of-donor-age-in-donation-after-cardiac-death-liver-transplantation-is-the-cut-off-60-still-of-relevance
#9
Andrea Schlegel, Irene Scalera, M Thamara, P R Perera, Marit Kalisvaart, Hynek Mergental, Darius F Mirza, John Isaac, Paolo Muiesan
Background Advanced donor age has been identified as risk factor, when combined with donor warm ischemia, e.g. in donation after circulatory death (DCD). In several countries DCD livers, older than 60 years are not considered suitable due to concerns related to poor graft function and development of ischaemic cholangiopathy. We evaluate in this study outcome after DCD liver transplantation using grafts from donors older than 60 years. Methodology We analysed outcome after DCD liver transplantation (n=315), comparing donors >60 years (n=93) and donors ≤60 years (n=222) from our centre between 2005 and 2015...
September 8, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28865156/gene-disease-associations-identify-a-connectome-with-shared-molecular-pathways-in-human-cholangiopathies
#10
Zhenhua Luo, Anil G Jegga, Jorge A Bezerra
Cholangiopathies are a diverse group of progressive diseases whose primary cell targets are cholangiocytes. To identify shared pathogenesis and molecular connectivity among the three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC] and primary sclerosing cholangitis [PSC]), we built a comprehensive platform of published data on gene variants, gene expression and functional studies, and applied network-based analytics in search for shared molecular circuits. Mining the data platform with largest connected component and interactome analyses, we validated previously reported associations and identified essential- and hub-genes...
September 2, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28859949/probability-management-and-long-term-outcomes-of-biliary-complications-after-hepatic-artery-thrombosis-in-liver-transplant-recipients
#11
Masato Fujiki, Koji Hashimoto, Emmanouil Palaios, Cristiano Quintini, Federico N Aucejo, Teresa Diago Uso, Bijan Eghtesad, Charles M Miller
BACKGROUND: Hepatic artery thrombosis after liver transplantation is a devastating complication associated with ischemic cholangiopathy that can occur even after successful revascularization. This study explores long-term outcomes after hepatic artery thrombosis in adult liver transplantation recipients, focusing on the probability, risk factors, and resolution of ischemic cholangiopathy. METHODS: A retrospective chart review of 1,783 consecutive adult liver transplantations performed between 1995 and 2014 identified 44 cases of hepatic artery thrombosis (2...
August 28, 2017: Surgery
https://www.readbyqxmd.com/read/28844960/bile-acid-receptors-in-the-biliary-tree-tgr5-in-physiology-and-disease
#12
REVIEW
Kathleen Deutschmann, Maria Reich, Caroline Klindt, Carola Dröge, Lina Spomer, Dieter Häussinger, Verena Keitel
Bile salts represent signalling molecules with a variety of endocrine functions. Bile salt effects are mediated by different receptor molecules, comprising ligand-activated nuclear transcription factors as well as G protein-coupled membrane-bound receptors. The farnesoid X receptor (FXR) and the plasma membrane-bound G protein-coupled receptor TGR5 (Gpbar-1) are prototypic bile salt receptors of both classes and are highly expressed in the liver including the biliary tree as well as in the intestine. In liver, TGR5 is localized in different non-parenchymal cells such as sinusoidal endothelial cells, Kupffer cells, hepatic stellate cells and small and large cholangiocytes...
August 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28844953/how-the-biliary-tree-maintains-immune-tolerance
#13
REVIEW
Haiyan Zhang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
The liver is a vital organ with distinctive anatomy, histology and heterogeneous cell populations. These characteristics are of particular importance in maintaining immune homeostasis within the liver microenvironments, notably the biliary tree. Cholangiocytes are the first line of defense of the biliary tree against foreign substances, and are equipped to participate through various immunological pathways. Indeed, cholangiocytes protect against pathogens by TLRs-related signaling; maintain tolerance by expression of IRAK-M and PPARγ; limit immune response by inducing apoptosis of leukocytes; present antigen by expressing human leukocyte antigen molecules and costimulatory molecules; recruit leukocytes to the target site by expressing cytokines and chemokines...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28844951/cholangiocytes-in-the-pathogenesis-of-primary-sclerosing-cholangitis-and-development-of-cholangiocarcinoma
#14
REVIEW
Brian K Chung, Tom Hemming Karlsen, Trine Folseraas
Primary sclerosing cholangitis (PSC) is an idiopathic cholangiopathy strongly associated with inflammatory bowel disease (IBD) and characterized by cholestasis, chronic immune infiltration and progressive fibrosis of the intrahepatic and extrahepatic bile ducts. PSC confers a high risk of cholangiocarcinoma (CCA) with PSC-CCA representing the leading cause of PSC-associated mortality. PSC-CCA is derived from cholangiocytes and associated progenitor cells - a heterogeneous group of dynamic epithelial cells lining the biliary tree that modulate the composition and volume of bile production by the liver...
August 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28842167/direct-visualization-of-igg4-related-cholangiopathy
#15
Chung Yao Yu, Suraj Patel, James Buxbaum
No abstract text is available yet for this article.
August 24, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28836688/src-kinase-inhibition-reduces-inflammatory-and-cytoskeletal-changes-in-%C3%AE-f508-human-cholangiocytes-and-improves-cftr-correctors-efficacy
#16
Romina Fiorotto, Mariangela Amenduni, Valeria Mariotti, Luca Fabris, Carlo Spirli, Mario Strazzabosco
CFTR, the channel mutated in cystic fibrosis (CF), is expressed by the biliary epithelium (i.e cholangiocytes) of the liver. Progressive clinical liver disease (CFLD) occurs in about 10% of CF patients and represents the third leading cause of death. Impaired secretion and inflammation contribute to CFLD, however the lack of human-derived experimental models has hampered the understanding of CFLD pathophysiology and the search for a cure. We have investigated the cellular mechanisms altered in human CF cholangiocytes using induced pluripotent stem cells (iPSC) derived from healthy controls and a ΔF508 CFTR patient...
July 24, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28834180/attitudes-and-barriers-to-the-use-of-dcd-livers-comparison-of-a-u-s-transplant-center-survey-to-the-united-network-for-organ-sharing-data
#17
Linda Sher, Cristiano Quintini, Sameh Adel Fayek, Peter Abt, Mary Lo, Pui Yan, Lingyun Ji, Susan Groshen, Jamie Case, Christopher Lee Marsh
Transplantation of liver grafts from donation after circulatory death (DCD) is limited. To identify barriers of DCD livers utilization, all active United States (U.S.) liver transplant centers (n=138) were surveyed and responses compared to the United Network for Organ Sharing (UNOS) data. Seventy-four centers (54%) responded, and diversity in attitudes was observed, with many not using organ and/or recipient prognostic variables defined in prior studies and UNOS data analysis. Most centers (74%) believed lack of a system allowing a timely re-transplant is a barrier to utilization...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28805978/obeticholic-acid-a-selective-farnesoid-x-receptor-agonist-regulates-bile-acid-homeostasis-in-sandwich-cultured-human-hepatocytes
#18
Yuanyuan Zhang, Jonathan P Jackson, Robert L St Claire, Kimberly Freeman, Kenneth R Brouwer, Jeffrey E Edwards
Farnesoid X receptor (FXR) is a master regulator of bile acid homeostasis through transcriptional regulation of genes involved in bile acid synthesis and cellular membrane transport. Impairment of bile acid efflux due to cholangiopathies results in chronic cholestasis leading to abnormal elevation of intrahepatic and systemic bile acid levels. Obeticholic acid (OCA) is a potent and selective FXR agonist that is 100-fold more potent than the endogenous ligand chenodeoxycholic acid (CDCA). The effects of OCA on genes involved in bile acid homeostasis were investigated using sandwich-cultured human hepatocytes...
August 2017: Pharmacology Research & Perspectives
https://www.readbyqxmd.com/read/28772351/the-influence-of-functional-warm-ischemia-time-on-dcd-liver-transplant-recipients-outcomes
#19
Jessica C Coffey, Kerollos N Wanis, Diethard Monbaliu, Nicholas Gilbo, Markus Selzner, Neeta Vachharajani, Mark A Levstik, Max Marquez, Maria B Majella Doyle, Jacques Pirenne, David Grant, Julie K Heimbach, William Chapman, Kelly Vogt, Roberto Hernandez-Alejandro
BACKGROUND: Duration of functional warm ischemia (f-WIT) is thought to have a causal effect on outcomes in controlled donation after circulatory death (DCD) liver transplantation (LT). METHODS: A retrospective cohort study was conducted at five centers. Data were extracted on donor and recipient characteristics, with attention to parameters recorded during withdrawal of life support to in situ cold perfusion. F-WIT was the time elapsed from any of the hemodynamic and oxygenation parameters to the start of in situ cold perfusion...
August 3, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28763485/cxcr2-signaling-and-the-microbiome-suppress-inflammation-bile-duct-injury-and-the-phenotype-of-experimental-biliary-atresia
#20
Junbae Jee, Reena Mourya, Pranavkumar Shivakumar, Lin Fei, Michael Wagner, Jorge A Bezerra
Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dependent bile duct injury and obstruction. Adult wild-type (WT) and Cxcr2-/- mice were fed a diet supplemented with sulfamethoxazole/trimethoprim (SMZ/TMP) during pregnancy and lactation, and their pups were injected intraperitoneally with rhesus rotavirus (RRV) within 24 hours of life to induce experimental biliary atresia...
2017: PloS One
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