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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/28539583/transient-receptor-potential-canonical-5-channels-plays-an-essential-role-in-hepatic-dyslipidemia-associated-with-cholestasis
#1
Khadija M Alawi, David Tandio, Jin Xu, Pratish Thakore, Georgia Papacleovoulou, Elizabeth S Fernandes, Cristina Legido-Quigley, Catherine Williamson, Susan D Brain
Transient receptor potential canonical 5 (TRPC5), a calcium-permeable, non-selective cation channel is expressed in the periphery, but there is limited knowledge of its regulatory roles in vivo. Endogenous modulators of TRPC5 include a range of phospholipids that have an established role in liver disease, including lysophosphatidylcholine (LPC). Cholestasis is characterized by impairment of excretion of bile acids, leading to elevation of hepatic bile acids. We investigated the contribution of TRPC5 in a murine model of cholestasis...
May 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28535810/fibrates-for-the-treatment-of-cholestatic-itch-fitch-study-protocol-for-a-randomized-controlled-trial
#2
Ruth Bolier, Elsemieke S de Vries, Albert Parés, Jeltje Helder, E Marleen Kemper, Koos Zwinderman, Ronald P Oude Elferink, Ulrich Beuers
BACKGROUND: Pruritus (itch) is a frequent, burdensome and difficult-to-treat symptom in patients with cholestasis. Fibrates are currently under investigation for the treatment of primary biliary cholangitis in patients with a suboptimal response to ursodeoxycholic acid. Moreover, there is empirical evidence for a possible antipruritic effect. We aim to prove this in a randomized controlled trial, including patients with cholestatic liver diseases other than primary biliary cholangitis that are accompanied by pruritus...
May 23, 2017: Trials
https://www.readbyqxmd.com/read/28526812/soluble-axl-is-an-accurate-biomarker-of-cirrhosis-and-hepatocellular-carcinoma-development-results-from-a-large-scale-multicenter-analysis
#3
Mirko Dengler, Katharina Staufer, Heidemarie Huber, Rudolf Stauber, Heike Bantel, Karl Heinz Weiss, Patrick Starlinger, Hannelore Pock, Petra Klöters-Plachky, Daniel N Gotthardt, Peter Rauch, Carolin Lackner, Judith Stift, Christine Brostjan, Thomas Gruenberger, Takashi Kumada, Hidenori Toyoda, Toshifumi Tada, Thomas S Weiss, Michael Trauner, Wolfgang Mikulits
Patients with chronic liver disease (CLD) and cirrhosis are at high risk for hepatocellular carcinoma (HCC). Current diagnostic tools for HCC detection include imaging techniques and serum biomarkers such as α-fetoprotein (AFP). Yet, these methods are limited in sensitivity and specificity to accurately detect early HCC. Here we focused on the potential of soluble Axl (sAxl) as a biomarker in CLD patients by analyzing serum samples of 1067 patients and healthy controls from centers in Europe and Asia. We show that serum concentrations of sAxl were significantly increased at early (82...
May 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28526488/-targeting-the-gut-liver-axis-in-liver-disease
#4
REVIEW
Reiner Wiest, Agustin Albillos, Michael Trauner, Jashmohan Bajaj, Rajiv Jalan
The gut is open to the outer environment, harbours the microbiome containing several fold more genetic material than the human genome and produces a myriad of metabolites as well as hormones/peptides. The liver is at the nexus between this vast source of nutrients, toxins and hormones and the remaining human body. Not surprisingly, this liver-gut-axis has hence, been demonstrated in experimental models and in-vitro systems to contribute to the pathogenesis of most liver diseases such as alcoholic and non-alcoholic fatty liver disease (NAFLD), -steatohepatitis (NASH), cholestatic liver diseases, hepatocellular carcinoma, acute-on-chronic liver failure, progression to fibrosis/cirrhosis and complications of cirrhosis...
May 16, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28517369/treatment-of-primary-biliary-cholangitis-non-responders-a-systematic-review
#5
Duminda Suraweera, Harman Rahal, Melissa Jimenez, Matthew Viramontes, Gina Choi, Sammy Saab
BACKGROUND: Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic cholestatic liver disease characterized by an immune mediated destruction of intrahepatic bile ducts. Ursodeoxycholic acid (UDCA) has been the primary medication for the treatment of PBC, resulting in improved liver tests, resolution of symptoms and increased transplant free survival. However, not all patients respond to UDCA. The aim of this systematic review is to provide an evidence based assessment of the medications that have been studied in patients who are refractory to UDCA...
May 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28506907/validity-criteria-for-the-diagnosis-of-fatty-liver-by-m-probe-based-controlled-attenuation-parameter
#6
Vincent Wai-Sun Wong, Salvatore Petta, Jean-Baptiste Hiriart, Calogero Cammà, Grace Lai-Hung Wong, Fabio Marra, Julien Vergniol, Anthony Wing-Hung Chan, Antonino Tuttolomondo, Wassil Merrouche, Henry Lik-Yuen Chan, Brigitte Le Bail, Umberto Arena, Antonio Craxì, Victor de Lédinghen
BACKGROUND & AIMS: Controlled attenuation parameter (CAP) can be performed together with liver stiffness measurement (LSM) by transient elastography and is often used to diagnose fatty liver. We aimed to define the validity criteria of CAP. METHODS: CAP was measured by the M probe prior to liver biopsy in 754 consecutive patients with different liver diseases at 3 centres in Europe and Hong Kong (derivation cohort, n=340; validation cohort, n=414; 101 chronic hepatitis B, 154 chronic hepatitis C, 349 non-alcoholic fatty liver disease, 37 autoimmune hepatitis, 49 cholestatic liver disease, 64 others; 277 F3-4; age 52±14; body mass index 27...
May 12, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28506373/heart-disease-and-the-liver-pathologic-evaluation
#7
REVIEW
Anne Knoll Koehne de Gonzalez, Jay H Lefkowitch
Liver injury due to acute and chronic heart failure has long been recognized. This article discusses the concepts of acute cardiogenic liver injury (ACLI) and cardiac or congestive hepatopathy (CH) along with their clinical manifestations and sequelae. Histologically, ACLI manifests as centrilobular hepatocellular necrosis, whereas CH is associated with centrilobular hepatocyte atrophy, dilated sinusoids, and perisinusoidal fibrosis, progressing to bridging fibrosis and ultimately cirrhosis. ACLI is associated with marked increases in aminotransferase levels, whereas CH is associated with a cholestatic pattern of laboratory tests...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28506369/autoimmune-hepatitis-overlap-syndromes-and-liver-pathology
#8
REVIEW
Albert J Czaja, Herschel A Carpenter
Autoimmune hepatitis (AIH) may have an atypical serum alkaline phosphatase elevation, antimitochondrial antibodies, histologic features of bile duct injury/loss, or cholangiographic findings of focal biliary strictures and dilations. These manifestations characterize the overlap syndromes. Patients can be classified as having AIH with features of primary biliary cholangitis, primary sclerosing cholangitis, or a cholestatic syndrome. The gold standard of diagnosis is clinical judgment. Histologic evaluation is a major diagnostic component...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28506343/-current-status-of-research-on-infantile-cholestatic-liver-disease-in-china-a-visualization-analysis
#9
Qiong Liao, Chao-Min Wan, Yu Zhu, Xiao-Yan Yang, Min Shu
OBJECTIVE: To investigate the current status of research on infantile cholestatic liver disease in China and future research trends. METHODS: A co-word analysis was performed in October 2016. Document retrieval and screening were performed in the Chinese databases CNKI and Wanfang Data using "cholestasis" and "infant" as key words. Excel 2010 was used to establish a co-occurrence matrix of high-frequency key words, and Ucinet 6.0 and Netdraw were used to develop a visualized network of these high-frequency key words...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28501004/adipose-derived-mesenchymal-stem-cells-slow-disease-progression-of-acute-on-chronic-liver-failure
#10
Carlos Gilsanz, Maria-Angeles Aller, Sherezade Fuentes-Julian, Isabel Prieto, Alejandro Blázquez-Martinez, Salvador Argudo, Jorge Fernández-Delgado, Jose Beleña, Jaime Arias, María P De Miguel
A serious complication of chronic hepatic insufficiency is acute-on-chronic liver failure, a recognized syndrome characterized by acute decompensation of cirrhosis and organ/system failure. We investigated the use of adipose-derived mesenchymal stem cells (AD-MSCs) in an experimental model of acute-on-chronic liver failure, developed by microsurgical extrahepatic cholestasis in rats. Rats undergoing microsurgical extrahepatic cholestasis were treated by intraparenchymal liver injection of human or rat AD-MSCs, undifferentiated or previously differentiated in vitro toward the hepatocyte lineage...
May 10, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28494557/-clinicopathologic-features-of-drug-induced-vanishing-bile-duct-syndrome
#11
L H Ye, C K Wang, H C Zhang, Z Q Liu, H W Zheng
Vanishing bile duct syndrome (VBDS) manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury (D-VBDS) is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings...
April 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28493820/deoxyguanosine-kinase-deficiency-a-report-of-four-patients
#12
Özlem Ünal, Burcu Hişmi, Mustafa Kılıç, Hayriye Hızarcıoğlu Gülşen, Turgay Coşkun, Serap Hatice Sivri, Ali Dursun, Aysel Yüce, Ayşegül Tokatlı
BACKGROUND: Hepatic involvement is a common feature in childhood mitochondrial disorders. Deoxyguanosine kinase (DGUOK) deficiency is one of the mitochondrial DNA depletion syndromes associated with hepatocerebral syndrome. Hepatic disease and neurologic dysfunction occurs within weeks after birth. Low birth weight is one of the common features. This study aims to describe the clinical and laboratory features of four infants carrying four different pathogenic variants in the DGUOK gene...
May 11, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28489679/ipilimumab-associated-cholestatic-hepatitis-a-case-report-and-literature-review
#13
Sümeyra Yildirim, Kemal Deniz, Ender Doğan, Mevlüt Başkol, Şebnem Gürsoy, Metin Özkan
Ipilimumab is a monoclonal antibody that exerts its effects by inhibiting the cytotoxic T-lymphocyte-associated protein 4 receptor on cytotoxic T lymphocytes. It is frequently used for the treatment of unresectable or metastatic melanoma. Ipilimumab may lead to several immune-related disease including colitis, thyroiditis, pneumonia, hepatitis, or pancreatitis as a side effect. Limited number of cases with hepatic damage as an ipilimumab-related adverse event has been reported in the literature. This agent has been implicated in causing acute hepatitis-like liver injury...
May 5, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28476903/endoscopic-nasobiliary-drainage-an-effective-treatment-option-for-benign-recurrent-intrahepatic-cholestasis-bric
#14
Ashok Choudhury, Anand V Kulkarni, Bishnupriya Sahoo, Chhagan Bihari
Benign recurrent intrahepatic cholestasis (BRIC) is characterised by recurrent episodes of jaundice, severe pruritus and low or normal serum γ-glutamyltransferase activity lasting from several weeks to months. BRIC is an autosomal recessive disorder caused by the mutation in either of the two hepatic transporter genes-ATP8B1 or ABCB11 gene. The disease is very well known for episodic flare of jaundice with cholestatic symptoms that are spontaneous or perpetuated by acute insults, followed by self-recovery...
May 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28473062/coffee-consumption-prevents-fibrosis-in-a-rat-model-that-mimics-secondary-biliary-cirrhosis-in-humans
#15
Jonathan Arauz, Natanael Zarco, Erika Hernández-Aquino, Marina Galicia-Moreno, Liliana Favari, José Segovia, Pablo Muriel
Investigations demonstrated that oxidative stress plays an important role in injury promotion in cholestatic liver disease. We hypothesized that coffee attenuates cholestasis-induced hepatic necrosis and fibrosis via its antioxidant, anti-inflammatory, and antifibrotic properties. The major aim of this study was to evaluate the hepatoprotective properties of coffee and caffeine in a model of chronic bile duct ligation (BDL) in male Wistar rats. Liver injury was induced by 28-day BDL, and conventional coffee, decaffeinated coffee, or caffeine was administered daily...
April 2017: Nutrition Research
https://www.readbyqxmd.com/read/28473040/individualized-medicine-in-gastroenterology-and-hepatology
#16
REVIEW
Michael C Stephens, Lisa A Boardman, Konstantinos N Lazaridis
After the completion of the Human Genome Project, there has been an acceleration in methodologies on sequencing nucleic acids (DNA and RNA) at a high precision and with ever-decreasing turnaround time and cost. Collectively, these approaches are termed next-generation sequencing and are already affecting the transformation of medical practice. In this symposium article, we highlight the current knowledge of the genetics of selected gastrointestinal tract and liver diseases, namely, inflammatory bowel disease, hereditary cholestatic liver disease, and familial colon cancer syndromes...
May 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28468009/what-comes-after-ursodeoxycholic-acid-in-primary-biliary-cholangitis
#17
Lin Lee Wong, Vinod S Hegade, David E J Jones
Primary biliary cholangitis (PBC) is a rare autoimmune liver disease characterized by chronic cholestasis. Treatment with the accepted primary therapy ursodeoxycholic acid (UDCA) has been shown to be associated with delayed disease progression probably through reduced impact of cholestatic injury on the target biliary epithelial cells. Patients with inadequate response to UDCA (which can be identified through validated biochemical criteria) are at increased risk of disease progression, need for liver transplantation, and death...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28441661/defective-platelet-activation-and-bleeding-complications-upon-cholestasis-in-mice
#18
Nina Sarah Gowert, Meike Klier, Maria Reich, Friedrich Reusswig, Lili Donner, Verena Keitel, Dieter Häussinger, Margitta Elvers
BACKGROUND/AIMS: Platelets are essential mediators of hemostasis to avoid excessive blood loss. Cirrhosis and chronic liver diseases are characterized by alterations in hemostasis. Alterations in the secondary hemostasis have been well studied, while defects in primary hemostasis, especially the consequences of cholestatic liver disease on platelet function are not well defined. METHODS: After bile duct ligation (BDL) platelet activation and thrombus formation were analyzed in mice...
April 20, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#19
(no author information available yet)
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 18, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28425415/drug-induced-liver-injury-at-a-tertiary-hospital-in-india-etiology-clinical-features-and-predictors-of-mortality
#20
Chetan Rathi, Nirav Pipaliya, Ruchir Patel, Meghraj Ingle, Aniruddha Phadke, Prabha Sawant
INTRODUCTION AND AIMS: Drug-induced liver injury (DILI) is rare; however, it is one of the important causes of acute liver failure which results in significant morbidity or mortality. MATERIAL AND METHODS: Patients with suspected DILI were enrolled based on predefined criteria and followed up for at least 6 months or until normalization of liver tests. Causality assessment was done by applying the Roussel Uclaf Causality Assessment Method model. RESULTS: We collected data from 82 individuals diagnosed with DILI at our hospital from 2014 through 2015 (41 men; median age, 38 years)...
May 2017: Annals of Hepatology
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