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https://www.readbyqxmd.com/read/28922472/mirna-506-promotes-primary-biliary-cholangitis-like-features-in-cholangiocytes-and-immune-activation
#1
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/28919271/-autoimmune-hepatitis-immunological-diagnosis
#2
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28919113/hepatic-tmem30a-deficiency-causes-intrahepatic-cholestasis-by-impairing-expression-and-localization-of-bile-salt-transporters
#3
Leiming Liu, Lingling Zhang, Lin Zhang, Fan Yang, Xudong Zhu, Zhongjie Lu, Yeming Yang, Haiqi Lu, Lifeng Feng, Zhuo Wang, Hui Chen, Sheng Yan, Lin Wang, Zhenyu Ju, Hongchuan Jin, Xianjun Zhu
Mutations in ATP8B1 or ATP11C (members of P4-type ATPases) cause progressive familial intrahepatic cholestasis type 1 (PFIC1) in human or intrahepatic cholestasis in mice. Transmembrane protein 30A (TMEM30A), as a β-subunit, is essential for the function of P4-type ATPases including ATP8B1 and ATP11C, however, its role in the etiology of cholestasis remains poorly understood. To investigate the function of TMEM30A in bile salt homeostasis, we developed Tmem30a liver-specific knockout (LKO) mice. Tmem30a LKO mice suffered from hyperbilirubinaemia, hypercholanaemia, inflammatory infiltration, ductular proliferation and liver fibrosis...
September 14, 2017: American Journal of Pathology
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/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#5
Abdulrahman Al-Hussaini, Kenneth D R Setchell, Bader AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASD) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. METHODS: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis...
September 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28901251/case-report-severe-hematological-muscle-and-liver-toxicity-caused-by-drugs-and-artichoke-infusion-interaction-in-an-elderly-polymedicated-patient
#6
M G Campos, J Machado, M L Costa, S Lino, F Correia, F Maltez
Case report, in a patient with a history of diabetes and hypertension, treated with metformin, gliclazide, enalapril + hydrochlorothiazide, amlodipine, aspirin and diazepam, recently medicated for a gouty crisis with colchicine and clonixin without improvement. Believing it could help in the treatment of gouty crisis symptoms he took about 1.5 L of artichoke infusion (Cynara cardunculus). He felt better and did agriculture work but developed a distal muscle pain, severe anemia, standard biochemical liver cholestasis, increase of alkaline phosphatase and marked increase of inflammatory parameters (hyperleucocytosis) and enters in the emergency department at the hospital...
September 12, 2017: Current Drug Safety
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/28883388/retrospective-clinical-study-of-the-effects-of-t-tube-placement-for-bile-duct-stricture
#8
İbrahim Tayfun Şahiner, Murat Kendirci
BACKGROUND T-tube placement in the common bile duct (CBD) is a surgical alternative to bile duct reconstruction in cholecystectomy for cholecysto-choledocholithiasis, or gallstones. The aim of this retrospective clinical study was to investigate the incidence of late complications of T-tube placement. MATERIAL AND METHODS Retrospective review identified 35 patients who had T-tube placement during cholecystectomy. Clinical data were collected on surgical indications, patient demographics, and clinical symptoms...
September 8, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28882937/granulomatous-hepatitis-in-a-patient-with-crohn-s-disease-and-cholestasis
#9
Bogdan Ioannis Patedakis Litvinov, Amit P Pathak
We describe a case of a 23-year-old woman with a history of Crohn's disease (CD), who initially presented with sepsis-like symptoms, subsequently developed severe cholestasis and following extensive inpatient workup was found to have non-caseating granulomas on her liver biopsy. Infectious aetiologies were excluded and the patient was treated with oral corticosteroids, which ameliorated but did not completely reverse the cholestasis. We review the differential diagnosis of hepatic granulomas and discuss the potential difficulties in establishing their exact aetiology in patients with CD...
September 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28881751/hepatitis-b-virus-surface-proteins-accelerate-cholestatic-injury-and-tumor-progression-in-abcb4-knockout-mice
#10
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/28868180/acute-and-chronic-hepatobiliary-manifestations-of-sickle-cell-disease-a-review
#11
REVIEW
Rushikesh Shah, Cesar Taborda, Saurabh Chawla
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis...
August 15, 2017: World Journal of Gastrointestinal Pathophysiology
https://www.readbyqxmd.com/read/28861314/new-surgical-technique-applied-with-urological-instruments-in-bilobar-multiple-hepatolithiasis-ultra-mini-percutaneous-hepatolithotomy
#12
Ahmet Öztürk, Mehmet Giray Sönmez, Süleyman Bakdık, Yunus Emre Göger, Mehmet Serkan Özkent, Faruk Aksoy, Metin Belviranlı
Intrahepatic bile duct stones may emerge with manifestations as chronic stomach ache, cholestasis, cholangitis, abscess, post-obstructive atrophy and liver cirrhosis presentation may occur. Thus the treatment of symptomatic hepatolithiasis patients should be provided. Different methods such as biliary decompression, endoscopic, percutaneous or open surgery are recommended for the treatment of patients with intrahepatic gallstones. The aim of the treatment is to extract the stones and regain biliary drainage...
September 2017: Turkish Journal of Urology
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/28858213/recreational-exposure-during-algal-bloom-in-carrasco-beach-uruguay-a-liver-failure-case-report
#14
Flavia Vidal, Daniela Sedan, Daniel D'Agostino, María Lorena Cavalieri, Eduardo Mullen, María Macarena Parot Varela, Cintia Flores, Josep Caixach, Dario Andrinolo
In January 2015, a 20-month-old child and her family took part in recreational activities at Carrasco and Malvín beaches (Montevideo, Uruguay). An intense harmful algae bloom (HAB) was developing along the coast at that time. A few hours after the last recreational exposure episode, the family suffered gastrointestinal symptoms which were self-limited except in the child's case, who was admitted to hospital in Uruguay with diarrhea, vomiting, fatigue, and jaundice. The patient had increased serum levels of liver enzymes and bilirubin and five days later presented acute liver failure...
August 31, 2017: Toxins
https://www.readbyqxmd.com/read/28855630/ppar%C3%AE-activation-protects-against-cholestatic-liver-injury
#15
Qi Zhao, Rui Yang, Jing Wang, Dan-Dan Hu, Fei Li
Intrahepatic cholestasis induced by drug toxicity, bile salt export pump (BSEP) deficiency, or pregnancy frequently causes cholestatic liver damage, which ultimately may lead to liver fibrosis and cirrhosis. Here, the preventive and therapeutic effects of peroxisome proliferator-activated receptor α (PPARα) signaling activated by fenofibrate was evaluated on cholestatic liver damage. Metabolomic analysis revealed that alpha-naphthyl isothiocyanate (ANIT)-induced intrahepatic cholestasis resulted in the accumulation of serum long-chain acylcarnitines and triglyceride, and the reduced expression of four fatty acid β-oxidation (β-FAO) relevant genes (Cpt1b, Cpt2, Mcad and Hadha), indicating the disruption of β-FAO...
August 30, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28851649/cholestatic-pregnancy-is-associated-with-reduced-vcam1-expression-in-vascular-endothelial-cell-of-placenta
#16
XueWei Qin, XiaoTian Ni, XiaoYuan Mao, Hao Ying, QiaoLing Du
OBJECTIVE: Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific disease which is closely correlated with abnormal placental vascular formation and deficient vascular maturation. This study intends to explore the role of VCAM-1 in the vascular formation in the placenta of ICP. METHODS: Patients with ICP or healthy puerperant were respectively used as ICP group and control group. The umbilical vein endothelial cell Eahy926 was selected as in vitro cell model...
August 26, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28844960/bile-acid-receptors-in-the-biliary-tree-tgr5-in-physiology-and-disease
#17
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/28844951/cholangiocytes-in-the-pathogenesis-of-primary-sclerosing-cholangitis-and-development-of-cholangiocarcinoma
#18
REVIEW
Brian K Chung, Tom H 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 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28839429/partial-external-biliary-diversion-in-bile-salt-export-pump-deficiency-association-between-outcome-and-mutation
#19
Philipp Ellinger, Jan Stindt, Carola Dröge, Katharina Sattler, Claudia Stross, Stefanie Kluge, Diran Herebian, Sander H J Smits, Martin Burdelski, Sebastian Schulz-Jürgensen, Antje Ballauff, Jan Schulte Am Esch, Ertan Mayatepek, Dieter Häussinger, Ralf Kubitz, Lutz Schmitt
AIM: To investigate the relation of two different mutations to the outcome of partial external biliary diversion (PEBD) in severe bile salt export pump (BSEP) deficiency. METHODS: Mutations in the gene encoding BSEP leading to severe BSEP deficiency in two unrelated patients were identified by genomic sequencing. Native liver biopsies and transiently transfected human embryonic kidney (HEK) 293 cells expressing either wild-type or mutated BSEP were subjected to immunofluorescence analysis to assess BSEP transporter localization...
August 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28838453/cholestasis-after-pediatric-liver-transplantation-recurrence-of-a-progressive-familial-intrahepatic-cholestasis-phenotype-as-a-rare-differential-diagnosis-a-case-report
#20
B Prusinskas, S Kathemann, D Pilic, B Hegen, P Küster, V Keitel, D Häussinger, R Büscher, H A Baba, P F Hoyer, E Lainka
INTRODUCTION: Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe a girl with neonatal cholestasis because of progressive familial intrahepatic cholestasis 2 (PFIC-2) and presence of a homozygous splice site mutation in the ABCB11 gene. Liver transplantation was performed because of end-stage liver disease at the age of 6. Cholestasis with normal gamma-glutamyl transferase (GGT) developed 8 years after liver transplantation...
September 2017: Transplantation Proceedings
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