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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/28926118/chop-induced-loss-of-intestinal-epithelial-stemness-contributes-to-bile-duct-ligation-induced-cholestatic-liver-injury
#1
Runping Liu, Xiaojiaoyang Li, Zhiming Huang, Derrick Zhao, Bhagyalaxmi Sukka Ganesh, Guanhua Lai, William M Pandak, Phillip B Hylemon, Jasmohan S Bajaj, Arun J Sanyal, Huiping Zhou
Impaired intestinal barrier function promotes the progression of various liver diseases including cholestatic liver disease. The close association of primary sclerosing cholangitis (PSC) with inflammatory bowel disease highlights the importance of the gut-liver axis. It has been reported that bile duct ligation (BDL)-induced liver fibrosis is significantly reduced in C/EBP homologous protein knock out (CHOP(-/-) ) mice. However, the underlying mechanisms remain unclear. In the current study, we demonstrate that BDL induces striking and acute hepatic ER stress responses after 1 day, which return to normal after 3 days...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28922472/mirna-506-promotes-primary-biliary-cholangitis-like-features-in-cholangiocytes-and-immune-activation
#2
Oihane Erice, Patricia Munoz-Garrido, Javier Vaquero, Maria J Perugorria, Maite G Fernandez-Barrena, Elena Saez, Alvaro Santos-Laso, Ander Arbelaiz, Raul Jimenez-Agüero, Joaquin Fernandez-Irigoyen, Enrique Santamaria, Verónica Torrano, Arkaitz Carracedo, Meenakshisundaram Ananthanarayanan, Marco Marzioni, Jesus Prieto, Ulrich Beuers, Ronald P Oude Elferink, Nicholas F LaRusso, Luis Bujanda, Jose J G Marin, Jesus M Banales
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with autoimmune phenomena targeting intrahepatic bile duct cells (cholangiocytes). Although PBC etiopathogenesis still remains obscure, development of anti-mitochondrial auto-antibodies against pyruvate dehydrogenase complex-E2 (PDC-E2) is a common feature. MicroRNA (miR) dysregulation occurs in liver and immune cells of PBC patients, but their functional relevance is largely unknown. We previously reported that miR-506 is overexpressed in PBC cholangiocytes and directly targets both Cl(-) /HCO3(-) anion exchanger 2 (AE2) and type III inositol 1,4,5-trisphosphate receptor (InsP3R3), leading to cholestasis...
September 18, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28921801/complications-symptoms-quality-of-life-and-pregnancy-in-cholestatic-liver-disease
#3
Kais Zakharia, Anilga Tabibian, Keith D Lindor, James H Tabibian
Cholestatic liver diseases (CLDs) encompass a variety of disorders of bile formation and/or flow which generally result in progressive hepatobiliary injury and ultimately end-stage liver disease. Many patients with CLD are diagnosed between the ages of 20-50 years, a particularly productive period of life professionally, biologically, and in other respects; it is not surprising, thus, that CLD is often associated with impaired health-related quality of life (HRQOL) and uncertainty regarding implications for and outcomes of pregnancy...
September 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28910492/p53-mediated-regulation-of-bile-acid-disposition-attenuates-cholic-acid-induced-cholestasis-in-mice
#4
Pan Chen, Dongshun Li, Yixin Chen, Jiahong Sun, Kaili Fu, Lihuan Guan, Huizhen Zhang, Yiming Jiang, Xi Li, Xuezhen Zeng, Xiao Chen, Min Huang, Huichang Bi
BACKGROUND AND PURPOSE: Tumor suppressor p53 is traditionally recognized as a surveillance molecule to preserve genome integrity. Recent studies have emerged on discovering its functions in metabolic diseases. Here we investigated the role of p53 in the regulation bile acid disposition and cholestasis. EXPERIMENTAL APPROACH: Bile acid disposition related gene expression profile altered by p53 activation was assessed in mouse primary hepatocytes with p53 depletion and in Trp53-null mice...
September 14, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28902430/reduced-serum-igf-1-associated-with-hepatic-osteodystrophy-is-a-main-determinant-of-low-cortical-but-not-trabecular-bone-mass
#5
Zhongbo Liu, Tianzhen Han, Haim Werner, Clifford J Rosen, Mitchell B Schaffler, Shoshana Yakar
Hepatic osteodystrophy is multifactorial in its pathogenesis. Numerous studies have shown that impairments of the hepatic growth hormone/insulin-like growth factor-1 axis (GH/IGF-1) are common in patients with non-alcoholic fatty liver disease, chronic viral hepatitis, liver cirrhosis, and chronic cholestatic liver disease. Moreover these conditions are also associated with low bone mineral density (BMD) and greater fracture risk, particularly in cortical bone sites. Hence, we addressed whether disruptions in the GH/IGF-1 axis were causally related to the low bone mass in states of chronic liver disease using a mouse model of liver-specific GH-receptor (GHR) gene deletion (Li-GHRKO)...
September 13, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28899465/-clinical-features-and-myo5b-mutations-of-a-family-affected-by-microvillus-inclusion-disease
#6
Ying Cheng, Hong Liang, Na-Li Cai, Li Guo, Yu-Ge Huang, Yuan-Zong Song
Microvillus inclusion disease (MVID) is an autosomal recessive disorder caused by biallelic mutations in the MYO5B or STX3 gene. Refractory diarrhea and malabsorption are the main clinical manifestations. The aim of this study was to investigate the clinical features and MYO5B gene mutations of an infant with MVID. A 21-day-old female infant was referred to the hospital with the complaint of diarrhea for 20 days. On physical examination, growth retardation of the body weight and length was found along with moderately jaundiced skin and sclera...
September 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28898457/organic-solute-transporter-beta-slc51b-deficiency-in-two-brothers-with-congenital-diarrhea-and-features-of-cholestasis
#7
Mutaz Sultan, Anuradha Rao, Orly Elpeleg, Frédéric M Vaz, Bassam Y Abu Libdeh, Saul J Karpen, Paul A Dawson
Primary bile acid malabsorption (PBAM) is associated with congenital diarrhea, steatorrhea, and a block in the intestinal return of bile acids in the enterohepatic circulation. Mutations in the ileal Na(+) -dependent bile acid transporter (ASBT; SLC10A2) can cause PBAM, but do not appear to account for most familial cases. Another major transporter involved in the intestinal reclamation of bile acids is the heteromeric Organic Solute Transporter alpha-beta (OSTα-OSTβ; SLC51A-SLC51B), which exports bile acid across the basolateral membrane...
September 12, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28898276/liver-myofibroblasts-of-murine-origins-express-mesothelin-identification-of-novel-rat-mesothelin-splice-variants
#8
Michel Fausther, Elise G Lavoie, Jonathan A Dranoff
Liver myofibroblasts are specialized effector cells that drive hepatic fibrosis, a hallmark process of chronic liver diseases, leading to progressive scar formation and organ failure. Liver myofibroblasts are increasingly recognized as heterogeneous with regards to their origin, phenotype, and functions. For instance, liver myofibroblasts express cell markers that are universally represented such as, ItgαV and Pdgfrβ, or restricted to a given subpopulation such as, Lrat exclusively expressed in hepatic stellate cells, and Gpm6a in mesothelial cells...
2017: PloS One
https://www.readbyqxmd.com/read/28889972/the-emerging-role-of-amp-activated-protein-kinase-in-cholestatic-liver-diseases
#9
REVIEW
Xiaojiaoyang Li, Runping Liu, Luyong Zhang, Zhenzhou Jiang
AMP-activated protein kinase (AMPK), recognized as an energy sensor with three heterotrimeric subunits (α, β and γ), not only maintains basal intracellular adenosine triphosphate levels but also regulates energy-intensive pathological responses, such as neurodegenerative and metabolic diseases, through multiple signaling pathways. Recent studies open a new direction for AMPK research and demonstrate that AMPK is a critical player in the pathogenesis of cholestatic liver injury and plays paradoxical roles in the regulation of different pathological processes, including the disruption of bile acid homeostasis and the regulation of hepatic polarity, inflammation and fibrosis...
September 7, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28882739/autoimmune-hepatitis%C3%A2-%C3%A2-update-on-clinical-management-in-2017
#10
REVIEW
Timur Liwinski, Christoph Schramm
Autoimmune hepatitis (AIH) is a progressive immune mediated liver disease of unknown origin. Key diagnostic features include hypergammaglobulinemia/elevated serum-IgG, characteristic circulating autoantibodies, periportal hepatitis with interface activity on liver biopsy and the exclusion of hepatotropic viruses. However, the diagnosis is challenging in cholestatic and severe presentations. It can be difficult to differentiate AIH from drug-induced liver injury. Although many patients initially respond to standard immunosuppressive therapy, a significant proportion experiences intolerable side effects or insufficient treatment response...
September 4, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28881751/hepatitis-b-virus-surface-proteins-accelerate-cholestatic-injury-and-tumor-progression-in-abcb4-knockout-mice
#11
Daniel Zahner, Hannah Glimm, Tomomitsu Matono, Yuri Churin, Diran Herebian, Ertan Mayatepek, Kernt Köhler, Stefan Gattenlöhner, Anne Stinn, Annette Tschuschner, Martin Roderfeld, Elke Roeb
Understanding of the pathophysiology of cholestasis associated carcinogenesis could challenge the development of new personalized therapeutic approaches and thus improve prognosis. Simultaneous damage might aggravate hepatic injury, induce chronic liver disease and even promote carcinogenesis. We aimed to study the effect of Hepatitis B virus surface protein (HBsAg) on cholestatic liver disease and associated carcinogenesis in a mouse model combining both impairments. Hybrids of Abcb4(-/-) and HBsAg transgenic mice were bred on fibrosis susceptible background BALB/c...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28868484/acute-hepatitis-in-the-dress-syndrome
#12
Ana Maria Oliveira, Rita Carvalho, Alexandra Martins, Jorge Reis
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, idiosyncratic reaction characterized by diffuse maculopapular rash, facial edema, lymphadenopathy, fever, eosinophilia and/or other leukocyte abnormalities, and involvement of internal organs as liver, kidney, heart and lung. Diagnosing this entity is specifically complicated due to the multiplicity of organs involved. DRESS syndrome must be recognized promptly and the causative drug withdrawn in order to improve patient outcomes...
November 2016: GE Port J Gastroenterol
https://www.readbyqxmd.com/read/28860789/new-developments-in-the-treatment-of-primary-biliary-cholangitis-role-of-obeticholic-acid
#13
REVIEW
Manan A Jhaveri, Kris V Kowdley
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease that predominantly affects women in early to middle age. It is typically associated with autoantibodies to mitochondrial antigens and results in immune-mediated destruction of small and medium-sized intrahepatic bile ducts leading to cholestasis, hepatic fibrosis and may progress to cirrhosis or hepatic failure and, in some cases, hepatocellular carcinoma. The clinical presentation and the natural history of PBC have improved over the years due to recognition of earlier widespread use of ursodeoxycholic acid (UDCA); about one-third of patients show suboptimal biochemical response to UDCA with poor prognosis...
2017: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/28860489/prognostic-significance-of-elevated-cholestatic-enzymes-for-fibrosis-and-hepatocellular-carcinoma-in-hospital-discharged-chronic-viral-hepatitis-patients
#14
Wen Xie, Yang Cao, Min Xu, Jiangbin Wang, Changyu Zhou, Xingxiang Yang, Xiaoxia Geng, Wenhong Zhang, Ning Li, Jun Cheng
Chronic viral hepatitis (CVH) is the root cause of liver fibrosis and subsequent hepatocellular carcinoma (HCC). We conducted a cross-sectional, observational study based on medical records and primary data collection from patients with CVH who were admitted in five hospitals across China between February and September 2013 to determine the prevalence of elevated cholestatic enzymes (ALP and/or GGT above ULN) in discharged Chinese patients with CVH as a primary outcome and secondarily evaluated the relationship of these enzymes with fibrosis and disease severity...
August 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28844960/bile-acid-receptors-in-the-biliary-tree-tgr5-in-physiology-and-disease
#15
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 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28836457/investigational-drugs-in-phase-ii-clinical-trials-for-primary-biliary-cholangitis
#16
Marina G Silveira, Keith D Lindor
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease that may lead to biliary fibrosis, and eventually cirrhosis. The primary treatment for PBC is ursodeoxycholic acid (UDCA), which has favorably altered its natural history. However, up to 40% of patients have an inadequate response to UDCA, and are therefore at high risk of liver-related complications. Obeticholic acid has recently been approved for use in patients with PBC with inadequate response or who are intolerant to UDCA, but improvement in long-term outcomes has not yet been demonstrated...
August 31, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28806642/circulating-follicular-helper-t-cells-presented-distinctively-different-responses-toward-bacterial-antigens-in-primary-biliary-cholangitis
#17
Zun-Qiang Zhou, Da-Nian Tong, Jiao Guan, Mei-Fang Li, Qi-Ming Feng, Min-Jie Zhou, Zheng-Yun Zhang
Primary biliary cholangitis (PBC) is a chronic and progressive cholestatic liver disease with unknown causes. The initiation of PBC is associated with bacterial infections and abnormal immune correlates, such as the presence of self-reactive anti-mitochondrial antibodies and shifted balance of T cell subsets. In particular, the CD4(+)CXCR5(+) follicular helper T (Tfh) cells are highly activated in PBC patients and are significantly associated with PBC severity, but the underlying reasons are unknown. In this study, we found that the circulating CD4(+)CXCR5(+) T cells were enriched with the interferon (IFN)-γ-secreting Th1-subtype and the interleukin (IL)-17-secreting Th17-subtype, but not the IL-4-secreting Th2 subtype...
August 11, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28804737/studies-on-the-mechanisms-of-bile-acid-initiated-hepatic-inflammation-in-cholestatic-liver-injury
#18
Shi-Ying Cai, James L Boyer
The mechanism of bile acid induced cholestatic liver injury remains controversial, thus hindering the development of new therapies for these diseases. In this research highlight, we briefly review the evolution of our understanding of the pathogenesis of bile acid induced liver injury, and summarize our recent findings on this topic. Our data suggests that under pathophysiological conditions bile acid induced liver injury is mediated by inflammatory responses that are initiated from stressed hepatocytes. We conclude by mentioning potential new therapeutic approaches for treating cholestatic liver injury based on these pathophysiologic concepts...
2017: Inflammation and Cell Signaling
https://www.readbyqxmd.com/read/28802564/interactions-between-platelets-and-inflammatory-monocytes-affect-sickness-behavior-in-mice-with-liver-inflammation
#19
Charlotte D'Mello, Wagdi Almishri, Hongqun Liu, Mark Gordon Swain
BACKGROUND & AIMS: Patients with inflammatory liver disease commonly develop debilitating symptoms, called sickness behaviors, which arise via changes in brain function. Monocytes that produce tumor necrosis factor (TNF) interact with cerebral endothelial cells to activate microglial cells and promote sickness behavior. Platelets regulate inflammation, and aggregates of monocytes and platelets are increased in the circulation of patients with liver disease. We investigated the role of platelets in inducing inflammatory features of circulating monocytes and promoting sickness behaviors in mice with cholestatic liver injury...
August 9, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28802084/the-parameters-that-help-to-differentiate-biliary-atresia-from-other-diseases
#20
Makoto Hayashida, Toshiharu Matsuura, Yoshiaki Kinoshita, Genshiro Esumi, Koichiro Yoshimaru, Yusuke Yanagi, Yoshiaki Takahashi, Tomoaki Taguchi
BACKGROUND: The diagnosis of biliary atresia (BA) is still challenging. The aim of this study was to analyze the clinical features and parameters that contribute to a diagnosis of BA. METHODS: From January 2000 to December 2013, 37 patients who underwent operative cholangiography were evaluated retrospectively. The patients were divided into two groups: a BA group, and a group with other cholestatic diseases (non-BA group). The demographic data and preoperative liver function test results were analyzed...
August 12, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
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