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https://www.readbyqxmd.com/read/29792896/exploring-new-treatment-paradigms-for-alcoholic-hepatitis-by-extrapolating-from-nash-and-cholestasis
#1
EDITORIAL
Thoetchai Bee Peeraphatdit, Douglas A Simonetto, Vijay H Shah
No abstract text is available yet for this article.
May 21, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29787821/slc25a13-c-1610_1612delinsat-mutation-in-an-indian-patient-and-literature-review-of-79-cases-of-citrin-deficiency-for-genotype-phenotype-associations
#2
A Radha Rama Devi, Shaik Mohammad Naushad
Here, we report SLC25A13 c.1610_1612delinsAT mutation from India in a 13-year old boy who presented with recurrent episodes of delirium and hyperammonemia. This is the second case with this mutation; the first case was of Pakistani origin. The boy responded to diet modification, sodium benzoate and arginine supplementation. Furthermore, we have aimed to establish genotype-phenotype correlation of 79 cases of citrin deficiency (46 males and 33 females) reported in 24 studies from all over the world. Inverse association was observed between age of onset and jaundice (r = -0...
May 19, 2018: Gene
https://www.readbyqxmd.com/read/29787781/regulation-of-bile-secretion-by-calcium-signaling-in-health-and-disease
#3
REVIEW
David C Trampert, Michael H Nathanson
Calcium (Ca2+ ) signaling controls secretion in many types of cells and tissues. In the liver, Ca2+ regulates secretion in both hepatocytes, which are responsible for primary formation of bile, and cholangiocytes, which line the biliary tree and further condition the bile before it is secreted. Cholestatic liver diseases, which are characterized by impaired bile secretion, may result from impaired Ca2+ signaling mechanisms in either hepatocytes or cholangiocytes. This review will discuss the Ca2+ signaling machinery and mechanisms responsible for regulation of secretion in both hepatocytes and cholangiocytes, and the pathophysiological changes in Ca2+ signaling that can occur in each of these cell types to result in cholestasis...
May 19, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29786568/nutritive-support-for-newborns-in-critical-conditions-semi-elemental-formilas-as-a-means-of-enteral-nutrition
#4
Dmytro Shkurupii
OBJECTIVE: Introduction: The goal is to increase the efficiency of nutritional support in intensive care of newborns by substantiating the choice of the enteral blend. PATIENTS AND METHODS: Materials and methods: A prospective cohort study was conducted, which included 173 newborns. The main group consisted of 143 patients of intensive care units. The comparison group - 30 virtually healthy newborns. Physical parameters, changes in protein metabolism, serum immunoglobulins, functioning of the gastrointestinal tract and hepatobiliary system in the development of critical conditions and depending on the type of enteric urine formula were determined...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29783833/-non-transplant-surgical-intervention-in-progressive-familial-intrahepatic-cholestasis
#5
X F Li, J Y Gong, J S Wang
No abstract text is available yet for this article.
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29783821/-clinical-and-genetic-analysis-of-eleven-pediatric-patients-with-alagille-syndrome
#6
L Guo, S T Zhao, Y Cheng, M Deng, H Li, Y Z Song, X R Cai, Q Zhou
Objective: To explore the clinical and molecular genetic features of patients with Alagille syndrome (AS). Methods: The clinical data of eleven pediatric patients, who were suspected to have AS at the Department of Pediatrics in the First Affiliated Hospital of Jinan University from August 2010 to March 2017, were collected and analyzed. Genomic DNA was extracted from peripheral blood leukocytes of the patients and their parents. For 5 patients collected before March 2006, all JAG1 exons and their flanking sequences were directly sequenced...
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29778510/assessment-of-intestinal-failure-associated-liver-disease-according-to-different-diagnostic-criteria
#7
Anna Simona Sasdelli, Federica Agostini, Caterina Pazzeschi, Mariacristina Guidetti, Simon Lal, Loris Pironi
BACKGROUND & AIMS: Intestinal failure associated liver disease (IFALD) has been defined using numerous criteria; however the clinical relevance of these criteria has never been compared. We therefore aimed to evaluate the prevalence, incidence, evolution of IFALD diagnosed by different criteria and to assess any clinical features that may be associated with its occurrence. METHODS: A cross sectional (CS) and retrospective study were carried out on adults on home parenteral nutrition (HPN) for chronic intestinal failure (CIF) managed at a single center...
May 8, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29774570/high-mobility-group-box-1-drives-fibrosis-progression-signaling-via-the-receptor-for-advanced-glycation-end-products-in-mice
#8
Xiaodong Ge, Elena Arriazu, Fernando Magdaleno, Daniel J Antoine, Rouchelle Dela Cruz, Neil Theise, Natalia Nieto
BACKGROUND & RATIONALE: High-mobility group box-1 (HMGB1) is a damage-associated molecular pattern (DAMP) increased in response to liver injury. Since HMGB1 is a ligand for the receptor for advanced glycation end-products (RAGE), we hypothesized that induction of HMGB1 could participate in the pathogenesis of liver fibrosis via RAGE cell-specific signaling mechanisms. RESULTS: liver HMGB1 protein expression correlated with fibrosis stage in patients with chronic Hepatitis C virus (HCV) infection, primary biliary cirrhosis (PBC) and alcoholic steatohepatitis (ASH)...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29772655/antioxidant-activity-and-hepatoprotective-potential-of-quercetin-7-rhamnoside-in-vitro-and-in-vivo
#9
Zhi-Qiang Huang, Pan Chen, Wei-Wei Su, Yong-Gang Wang, Hao Wu, Wei Peng, Pei-Bo Li
Hypericum japonicum is traditionally used as a folk medicine to treat cholestasis and hepatitis. Quercetin 7-rhamnoside (Q7R) is one of the main flavonoid components of Hypericum japonicum and has been rarely studied. The aim of the present study was to evaluate the antioxidant activity and hepatoprotective potential of Q7R. In the in vitro experiments, DPPH, ABTS and ferric reducing antioxidant power (FRAP) assays were first performed to assess the antioxidant properties of Q7R, and then a H₂O₂-induced oxidative damage cellular model was used to determine the cytoprotective and antioxidant properties of Q7R in human liver L-02 cells...
May 16, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29771932/a-multi-center-preclinical-study-of-gadoxetate-dce-mri-in-rats-as-a-biomarker-of-drug-induced-inhibition-of-liver-transporter-function
#10
Anastassia Karageorgis, Stephen C Lenhard, Brittany Yerby, Mikael F Forsgren, Serguei Liachenko, Edvin Johansson, Mark A Pilling, Richard A Peterson, Xi Yang, Dominic P Williams, Sharon E Ungersma, Ryan E Morgan, Kim L R Brouwer, Beat M Jucker, Paul D Hockings
Drug-induced liver injury (DILI) is a leading cause of acute liver failure and transplantation. DILI can be the result of impaired hepatobiliary transporters, with altered bile formation, flow, and subsequent cholestasis. We used gadoxetate dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI), combined with pharmacokinetic modelling, to measure hepatobiliary transporter function in vivo in rats. The sensitivity and robustness of the method was tested by evaluating the effect of a clinical dose of the antibiotic rifampicin in four different preclinical imaging centers...
2018: PloS One
https://www.readbyqxmd.com/read/29761207/omics-based-responses-induced-by-bosentan-in-human-hepatoma-heparg-cell-cultures
#11
Robim M Rodrigues, Laxmikanth Kollipara, Umesh Chaudhari, Agapios Sachinidis, René P Zahedi, Albert Sickmann, Annette Kopp-Schneider, Xiaoqi Jiang, Hector Keun, Jan Hengstler, Marlies Oorts, Pieter Annaert, Eef Hoeben, Eva Gijbels, Joery De Kock, Tamara Vanhaecke, Vera Rogiers, Mathieu Vinken
Bosentan is well known to induce cholestatic liver toxicity in humans. The present study was set up to characterize the hepatotoxic effects of this drug at the transcriptomic, proteomic, and metabolomic levels. For this purpose, human hepatoma-derived HepaRG cells were exposed to a number of concentrations of bosentan during different periods of time. Bosentan was found to functionally and transcriptionally suppress the bile salt export pump as well as to alter bile acid levels. Pathway analysis of both transcriptomics and proteomics data identified cholestasis as a major toxicological event...
May 14, 2018: Archives of Toxicology
https://www.readbyqxmd.com/read/29761168/outcomes-of-surgical-management-of-familial-intrahepatic-cholestasis-1-and-bile-salt-export-protein-deficiencies
#12
Laura N Bull, Ludmila Pawlikowska, Sandra Strautnieks, Irena Jankowska, Piotr Czubkowski, Jennifer L Dodge, Karan Emerick, Catherine Wanty, Sami Wali, Samra Blanchard, Florence Lacaille, Jane A Byrne, Albertien M van Eerde, Kaija-Leena Kolho, Roderick Houwen, Steven Lobritto, Vera Hupertz, Patricia McClean, Giorgina Mieli-Vergani, Etienne Sokal, Philip Rosenthal, Peter F Whitington, Joanna Pawlowska, Richard J Thompson
Progressive familial intrahepatic cholestasis (PFIC) with normal circulating gamma-glutamyl transpeptidase levels can result from mutations in the ATP8B1 gene (encoding familial intrahepatic cholestasis 1 [FIC1] deficiency) or the ABCB11 gene (bile salt export protein [BSEP] deficiency). We investigated the outcomes of partial external biliary diversion, ileal exclusion, and liver transplantation in these two conditions. We conducted a retrospective multicenter study of 42 patients with FIC1 deficiency (FIC1 patients) and 60 patients with BSEP deficiency (BSEP patients) who had undergone one or more surgical procedures (57 diversions, 6 exclusions, and 57 transplants)...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29761167/phenotypic-spectrum-and-diagnostic-pitfalls-of-abcb4-deficiency-depending-on-age-of-onset
#13
Stephanie Barbara Schatz, Christoph Jüngst, Verena Keitel-Anselmo, Ralf Kubitz, Christina Becker, Patrick Gerner, Eva-Doreen Pfister, Imeke Goldschmidt, Norman Junge, Daniel Wenning, Stephan Gehring, Stefan Arens, Dirk Bretschneider, Dirk Grothues, Guido Engelmann, Frank Lammert, Ulrich Baumann
Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 ( ABCB4 ) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29761098/discovery-of-natural-products-as-novel-and-potent-fxr-antagonists-by-virtual-screening
#14
Yanyan Diao, Jing Jiang, Shoude Zhang, Shiliang Li, Lei Shan, Jin Huang, Weidong Zhang, Honglin Li
Farnesoid X receptor (FXR) is a member of nuclear receptor family involved in multiple physiological processes through regulating specific target genes. The critical role of FXR as a transcriptional regulator makes it a promising target for diverse diseases, especially those related to metabolic disorders such as diabetes and cholestasis. However, the underlying activation mechanism of FXR is still a blur owing to the absence of proper FXR modulators. To identify potential FXR modulators, an in-house natural product database (NPD) containing over 4,000 compounds was screened by structure-based virtual screening strategy and subsequent hit-based similarity searching method...
2018: Frontiers in Chemistry
https://www.readbyqxmd.com/read/29756983/-endocrine-disruptors-in-artificial-nutrition
#15
Maria B Badia Tahull, Elisabet Leiva Badosa, Montserrat Colls González, Josep Llop Talaverón
Phthalates are chemical compounds classified as endocrine disruptors which are present in practically every environment of daily life. In the field of artificial nutrition, they are relevant because they are found as plasticizers in infusion lines made with PVC. They are lipophilic molecules which weakly pair with PVC and, therefore, they are easily extracted by the fatty compounds that are part of both the parenteral and enteral nutrition, as various studies show. As endocrine disruptors, they directly affect the reproductive organs because of their antiandrogenic and estrogenic effects...
February 27, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29744332/clinical-and-pathological-characteristics-of-autoimmune-hepatitis-with-acute-presentation
#16
Yi Shen, Changli Lu, Ruoting Men, Jianping Liu, Tinghong Ye, Li Yang
Aim. To study the differences between acute presentation-autoimmune hepatitis (A-AIH) and chronic autoimmune hepatitis (C-AIH). Methods. Through long-term follow-up, 80 patients were included in our study by using the revised international autoimmune hepatitis group (IAIHG) score and were divided into acute and chronic groups for comparison. Results. No significant difference was found in the gender, age, IAIHG score (pretreatment/posttreatment), definite diagnosis rate, extrahepatic autoimmune disease, onset time, or treatment before biopsy between the acute and chronic groups...
2018: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29740092/bile-acids-and-their-respective-conjugates-elicit-different-responses-in-neonatal-cardiomyocytes-role-of-gi-protein-muscarinic-receptors-and-tgr5
#17
Effendi Ibrahim, Ivan Diakonov, Dulasi Arunthavarajah, Teresa Swift, Mary Goodwin, Saraid McIlvride, Vanya Nikolova, Catherine Williamson, Julia Gorelik
Bile acids are recognised as bioactive signalling molecules. While they are known to influence arrhythmia susceptibility in cholestasis, there is limited knowledge about the underlying mechanisms. To delineate mechanisms underlying fetal heart rhythm disturbances in cholestatic pregnancy, we used FRET microscopy to monitor cAMP release and contraction measurements in isolated rodent neonatal cardiomyocytes. The unconjugated bile acids CDCA, DCA and UDCA and, to a lesser extent, CA were found to be relatively potent agonists for the GPBAR1 (TGR5) receptor and elicit cAMP release, whereas all glyco- and tauro- conjugated bile acids are weak agonists...
May 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29739259/role-of-ischemia-modified-albumin-in-the-evaluation-of-oxidative-stress-in-intrahepatic-cholestasis-of-pregnancy
#18
A T Tayyar, S Kozalı, G Y Yıldırım, R Karakus, I T Yuksel, O Erel, S Neselioglu, M Eroglu
OBJECTIVE: To investigate the ischemia-modified albumin (IMA) level, and the IMA/albumin ratio (IMAR) in healthy pregnant women, and pregnant women with intrahepatic cholestasis of pregnancy (ICP). MATERIAL AND METHOD: This cross-sectional study included 53 women with ICP and 52 healthy pregnant women. Their serum IMA and albumin levels were analyzed, and the women were followed up to delivery. RESULTS: No significant intergroup differences were identified in maternal age, body mass index, and gestational age at the time that the blood samples were taken...
May 8, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29737914/lipidomics-reveal-aryl-hydrocarbon-receptor-ahr-regulated-lipid-metabolic-pathway-in-alpha-naphthyl-isothiocyanate-anit-induced-intrahepatic-cholestasis
#19
Bao-Long Wang, Chang-Wen Zhang, Liang Wang, Kun-Long Tang, Naoki Tanaka, Frank J Gonzalez, Yong Xu, Zhong-Ze Fang
1. Ultra-performance liquid chromatography coupled with electrospray ionization quadrupole mass spectrometry (UPLC-ESI-QTOF MS)-based lipidomics was employ to elucidate new mechanism of alpha-naphthyl isothiocyanate (ANIT)-induced intrahepatic cholestasis in mice. 2. Multiple lipid components significantly increased in ANIT-induced intrahepatic cholestasis, including PC 16:0, 20:4, PC 16:0, 22:6, PC 16:0, 18:2, LPC 18:2, PC 18:2, LPC 18:1, PC 18:1, 14:0, SM 18:1, 16:0, oleoylcarnitine, and palmitoylcarnitine...
May 8, 2018: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/29737419/langerhans-cell-histiocytosis-presenting-as-crohn-s-disease-a-case-report
#20
Amelie Therrien, Zaki El Haffaf, Claire Wartelle-Bladou, Justin Côté-Daigneault, Bich N Nguyen
PURPOSE: We describe an exceptional case of Langerhans cell histiocytosis (LCH) that presented as Crohn's disease and primary sclerosing cholangitis. METHODS: The patient's clinical, endoscopic, and histologic data from the Centre Hospitalier de l'Universite de Montreal were reviewed, as well as the literature on LCH involving the digestive tract and the liver, with a focus on the similarities with Crohn's disease and primary sclerosing cholangitis. RESULTS: A 39 years-old man first presented with anal fissures and deep punctiform colonic ulcers...
May 8, 2018: International Journal of Colorectal Disease
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