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https://www.readbyqxmd.com/read/28425419/a-rare-bsep-mutation-associated-with-a-mild-form-of-progressive-familial-intrahepatic-cholestasis-type-2
#1
Orith Waisbourd-Zinman, Lea F Surrey, Anna E Schwartz, Pierre A Russo, Jessica Wen
Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425413/ursodeoxycholic-acid-therapy-in-patients-with-primary-biliary-cholangitis-with-limited-liver-transplantation-availability
#2
Yazmín Karel Melchor-Mendoza, Braulio Martínez-Benítez, Aline Mina-Hawat, Gustavo Rodríguez-Leal, Ximena Duque, Segundo Moran-Villota
INTRODUCTION: There is little information on survival rates of patients with primary biliary cholangtis (PBC) in developing countries. This is particularly true in Latin America, where the number of liver transplants performed remains extremely low for patients with advanced liver disease who fulfill criteria for liver transplantation. The goal of this study was to compare survival rate of patients with PBC in developing countries who were treated with ursodeoxycholic acid (UDCA) versus survival of patients who received other treatments (OT) without UDCA, prescribed before the UDCA era...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28420650/changes-in-lxr-signaling-influence-early-pregnancy-lipogenesis-and-protect-against-dysregulated-fetoplacental-lipid-homeostasis
#3
Vanya Nikolova, Georgia Papacleovoulou, Elena Bellafante, Luiza Borges Manna, Eugene Jansen, Silvère Baron, Shadi Abu-Hayyeh, Malcolm G Parker, Catherine Williamson
Human pregnancy is associated with enhanced de novo lipogenesis in the early stages followed by hyperlipidemia during advanced gestation. Liver X receptors (LXRs) are oxysterol-activated nuclear receptors which stimulate de novo lipogenesis and also promote the efflux of cholesterol from extrahepatic tissues followed by its transport back to the liver for biliary excretion. Although LXR is recognized as a master regulator of triglyceride and cholesterol homeostasis it is unknown whether it facilitates the gestational adaptations in lipid metabolism...
April 18, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28409358/added-value-of-gadoxetic-acid-enhanced-t1-weighted-magnetic-resonance-cholangiography-for-the-diagnosis-of-post-transplant-biliary-complications
#4
Sonja Kinner, Tilman B Schubert, Adnan Said, Joshua D Mezrich, Scott B Reeder
OBJECTIVES: Biliary complications after liver transplantation (LT) are common. This study aimed to ascertain the value of gadoxetic acid-enhanced T1-weighted (T1w) magnetic resonance cholangiography (MRC) to evaluate anastomotic strictures (AS), non-anastomotic strictures (NAS) and biliary casts (BC). METHODS: Sixty liver-transplanted patients with suspicion of biliary complications and T2w-MRCP and T1w-MRC followed by endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC) were analysed...
April 13, 2017: European Radiology
https://www.readbyqxmd.com/read/28408243/erlotinib-and-bevacizumab-in-patients-with-advanced-non-small-cell-lung-cancer-and-activating-egfr-mutations-belief-an-international-multicentre-single-arm-phase-2-trial
#5
Rafael Rosell, Urania Dafni, Enriqueta Felip, Alessandra Curioni-Fontecedro, Oliver Gautschi, Solange Peters, Bartomeu Massutí, Ramon Palmero, Santiago Ponce Aix, Enric Carcereny, Martin Früh, Miklos Pless, Sanjay Popat, Athanasios Kotsakis, Sinead Cuffe, Paolo Bidoli, Adolfo Favaretto, Patrizia Froesch, Noemí Reguart, Javier Puente, Linda Coate, Fabrice Barlesi, Daniel Rauch, Michael Thomas, Carlos Camps, Jose Gómez-Codina, Margarita Majem, Rut Porta, Riyaz Shah, Emer Hanrahan, Roswitha Kammler, Barbara Ruepp, Manuela Rabaglio, Marie Kassapian, Niki Karachaliou, Rachel Tam, David S Shames, Miguel A Molina-Vila, Rolf A Stahel
BACKGROUND: The tyrosine kinase inhibitor erlotinib improves the outcomes of patients with advanced non-small-cell lung carcinoma (NSCLC) harbouring epidermal growth factor receptor (EGFR) mutations. The coexistence of the T790M resistance mutation with another EGFR mutation in treatment-naive patients has been associated with a shorter progression-free survival to EGFR inhibition than in the absence of the T790M mutation. To test this hypothesis clinically, we developed a proof-of-concept study, in which patients with EGFR-mutant NSCLC were treated with the combination of erlotinib and bevacizumab, stratified by the presence of the pretreatment T790M mutation...
April 10, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28407375/h19-promotes-cholestatic-liver-fibrosis-by-preventing-zeb1-mediated-inhibition-of-epcam
#6
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/28405682/apamin-suppresses-biliary-fibrosis-and-activation-of-hepatic-stellate-cells
#7
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
#8
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/28398710/additional-values-of-high-resolution-gadoxetic-acid-enhanced-mr-cholangiography-for-evaluating-the-biliary-anatomy-of-living-liver-donors-comparison-with-t2-weighted-mr-cholangiography-and-conventional-gadoxetic-acid-enhanced-mr-cholangiography
#9
Hyo-Jin Kang, Jeong Min Lee, Jeong Hee Yoon, Ijin Joo, Won Chang, Kyung-Suk Suh, Kwang-Woong Lee, Nam-Joon Yi, Joon Koo Han
PURPOSE: To determine the incremental value of small field of view (sFOV) high-resolution (HR) gadoxetic acid-enhanced 3D T1 -weighted (W) magnetic resonance cholangiography (MRC) for evaluating the biliary anatomy of potential living donors by comparing it to T2 W-MRC. MATERIALS AND METHODS: In all, 73 living donor candidates underwent gadoxetic acid-enhanced MRI (3.0T) including three kinds of MRCs: 3D multislice T2 W-MRC, regular FOV (rFOV) (380 × 380mm, resolution 1...
April 11, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28382295/acetic-acid-sclerotherapy-for-treatment-of-biliary-leak-from-an-isolated-right-posterior-sectoral-duct-after-cholecystectomy
#10
Jeong-Ik Park, Young-Kil Choi, Bo-Hyun Jung
Bile duct injury is one of the most serious complications of both laparoscopic and open cholecystectomy. Isolated bile duct injury can occur from the misidentification of aberrant right hepatic ducts, and it is troublesome because the early diagnosis is easy to miss and the definite treatment is controversial. We report a case of an isolated right posterior sectoral duct injury following cholecystectomy managed successfully with acetic acid sclerotherapy combined with coil embolization for a fistula tract.
April 2017: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/28381385/hida-scan-for-functional-gallbladder-disorder-ensure-that-you-know-how-the-scan-was-done
#11
Naeem Goussous, Hadia Maqsood, Ethan Spiegler, Gopal C Kowdley, Steven C Cunningham
BACKGROUND: Despite the increasing use of fatty meal (FM) as a substitute for cholecystokinin (CCK) in pain reproduction during hepato-imino-diacetic acid (HIDA) scan in functional gallbladder disorder, there are no studies comparing the differences between CCK and FM. The present study was to compare the efficacy of FM in comparison of CCK in FGBD application. METHODS: Patients undergoing HIDA scans from August 2013 to May 2014 were divided into two groups: those undergoing CCK-stimulated HIDA scan versus FM-stimulated HIDA scan...
April 2017: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/28380440/monoglyceride-lipase-deficiency-affects-hepatic-cholesterol-metabolism-and-lipid-dependent-gut-transit-in-apoe-mice
#12
Nemanja Vujic, Melanie Korbelius, Christina Leopold, Madalina Duta-Mare, Silvia Rainer, Stefanie Schlager, Madeleine Goeritzer, Dagmar Kolb, Thomas O Eichmann, Clemens Diwoky, Andreas Zimmer, Robert Zimmermann, Achim Lass, Branislav Radovic, Dagmar Kratky
Monoglyceride lipase (MGL) hydrolyzes monoglycerides (MGs) to glycerol and fatty acids. Among various MG species MGL also degrades 2-arachidonoylglycerol (2-AG), the most abundant endocannabinoid and potent activator of cannabinoid receptors (CBR) 1 and 2. MGL-knockout (-/-) mice exhibit pronounced 2-AG accumulation, but lack central cannabimimetic effects due to CB1R desensitization. We have previously shown that MGL affects plaque stability in apolipoprotein E (ApoE)-/- mice, an established animal model for dyslipidemia and atherosclerosis...
March 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28376623/the-clinical-extremes-of-autoimmune-cholangitis
#13
Sara Campos, Dário Gomes, Maria Augusta Cipriano, Carlos Sofia
Autoimmune cholangitis (AIC) was first described in 1987 as immunocholangitis in three women who presented with signs and symptoms of primary biliary cholangitis (PBC), but who were antimitochondrial (AMA) negative and antinuclear antibodies (ANA) positive, and responded to immunosuppressive therapy with azathioprine and prednisolone (1). AIC is a rare chronic cholestatic inflammatory disease characterized by the presence of high ANA or smooth muscle antibodies (SMA) but AMA seronegativity. Histologically, AIC exhibits bile duct injury (2)...
April 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28371104/old-and-new-treatments-for-primary-biliary-cholangitis
#14
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/28371099/finding-the-cure-for-primary-biliary-cholangitis-still-waiting
#15
Atsushi Tanaka, M Eric Gershwin
The introduction of ursodeoxycholic acid (UDCA)may well have contributed to some of the improvements in morbidity and mortality of primary biliary cholangitis (PBC). Yet nearly 40% of PBC patients are unresponsive to UDCA. Further the data on UDCA is confounded by the changes in the goepidemiology and particularly the earlier diagnosis of PBC. In this regard we welcome the addition of obeticholic acid (OCA) as an alternative therapeutic option forthe treatment of PBC in those patients refractory to UDCA. However, OCA is intellectually disappointing...
April 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28370258/polyphenic-trait-promotes-liver-cancer-in-a-model-of-epigenetic-instability
#16
Marco Cassano, Sandra Offner, Evarist Planet, Alessandra Piersigilli, Suk Min Jang, Hugues Henry, Markus B Geuking, Catherine Mooser, Kathy D McCoy, Andrew J Macpherson, Didier Trono
Hepatocellular carcinoma (HCC) represents the fifth most common form of cancer worldwide and carries a high mortality rate due to lack of effective treatment. Males are eight times more likely to develop HCC than females, an effect largely driven by sex hormones, albeit through still poorly understood mechanisms. We previously identified TRIM28 (tripartite protein 28), a scaffold protein capable of recruiting a number of chromatin modifiers, as a crucial mediator of sexual dimorphism in the liver. Trim28(hep-/-) mice display sex-specific transcriptional deregulation of a wide range of bile and steroid metabolism genes and development of liver adenomas in males...
March 30, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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/28365715/gall-bladder-agenesis-a-rare-embryonic-cause-of-recurrent-biliary-colic
#18
Aarthi Rajkumar, Albina Piya
BACKGROUND Gallbladder agenesis (GA) is an extremely rare anatomic anomaly with a reported incidence of less than 0.5%. It is usually asymptomatic, but can present with features of biliary colic and cholecystitis. We present here a case of GA in a patient with recurrent biliary colic.  CASE REPORT A 24-year-old African American woman presented with recurrent episodes of right upper-quadrant abdominal pain. During her first episode, she was found to have elevated transaminases and clinical features of cholecystitis, but ultrasound did not visualize a gallbladder and she was discharged with a diagnosis of biliary colic...
April 2, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28350426/pharmacological-interventions-for-primary-biliary-cholangitis-an-attempted-network-meta-analysis
#19
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/28349866/bolstering-cholesteryl-ester-hydrolysis-in-liver-a-hepatocyte-targeting-gene-delivery-strategy-for-potential-alleviation-of-atherosclerosis
#20
Hongliang He, Michael G Lancina, Jing Wang, William J Korzun, Hu Yang, Shobha Ghosh
Current atherosclerosis treatment strategies primarily focus on limiting further cholesteryl esters (CE) accumulation by reducing endogenous synthesis of cholesterol in the liver. No therapy is currently available to enhance the removal of CE, a crucial step to reduce the burden of the existing disease. Given the central role of hepatic cholesteryl ester hydrolase (CEH) in the intrahepatic hydrolysis of CE and subsequent removal of the resulting free cholesterol (FC), in this work, we applied galactose-functionalized polyamidoamine (PAMAM) dendrimer generation 5 (Gal-G5) for hepatocyte-specific delivery of CEH expression vector...
June 2017: Biomaterials
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