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https://www.readbyqxmd.com/read/27893674/relapsing-remitting-lesions-in-a-woman-with-progressive-hemifacial-atrophy-and-chronic-hepatitis-b-virus-infection-a-case-report
#1
Ying Zhang, Mingqin Zhu, Xiaozhen Li, Jing Miao, Chenchen Duan, Li Cui, Xuefan Yu
INTRODUCTION: Progressive hemifacial atrophy (PHA) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and fat, muscle, and osteocartilagenous structures creating a sunken hemiface appearance.Etiopathogenesis of PHA is poorly understood; no definitive treatment is currently available. CLINICAL FINDINGS: We report a 41-year-old woman with PHA who showed an uncharacteristic "relapsing-remitting" evolution of brain lesions and was seropositive for hepatitis B virus (HBV)...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27890169/hepatitis-c-virus-infection-and-rheumatic-diseases-the-impact-of-direct-acting-antiviral-agents
#2
REVIEW
Patrice Cacoub, Cloé Commarmond, David Sadoun, Anne Claire Desbois
Chronic hepatitis C virus (HCV) infection is associated with liver and extrahepatic complications, including B-cell lymphoma, cardiovascular and kidney diseases, glucose metabolism impairment and rheumatic conditions ie, arthralgia, myalgia, cryoglobulinemia vasculitis, sicca syndrome and the production of autoantibodies. The treatment has long been based on interferon alpha (IFN) that was found poorly effective, and contraindicated in many autoimmune/inflammatory disorders because of possible exacerbation of rheumatic disorders...
February 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/27777888/ursodeoxycholic-acid-in-treatment-of-non-cholestatic-liver-diseases-a-systematic-review
#3
Jillian Reardon, Trana Hussaini, Majid Alsahafi, Vladimir Marquez Azalgara, Siegfried R Erb, Nilufar Partovi, Eric M Yoshida
Aims: To systematically evaluate the literature for evidence to support the use of bile acids in non-cholestatic liver conditions. Methods: Searches were conducted on the databases of Medline (1948-March 31, 2015), Embase (1980-March 31, 2015) and the Cochrane Central Register of Controlled Trials, and on Google and Google Scholar to identify articles describing ursodeoxycholic acid (UDCA) and its derivatives for non-cholestatic hepatic indications. Combinations of the following search terms were used: ursodeoxycholic acid, ursodiol, bile acids and/or salts, non alcoholic fatty liver, non alcoholic steatohepatitis, fatty liver, alcoholic hepatitis, alcohol, liver disease, autoimmune, autoimmune hepatitis, liver transplant, liver graft, transplant rejection, graft rejection, ischemic reperfusion injury, reperfusion injury, hepatitis B, hepatitis C, viral hepatitis, chronic hepatitis, acute hepatitis, transaminases, alanine transaminase, liver enzymes, aspartate aminotransferase, gamma-glutamyl transferase, gamma-glutamyl transpeptidase, bilirubin, alkaline phosphatase...
September 28, 2016: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/27759901/human-leucocyte-antigens-b-08-drb1-03-and-drb1-13-are-significantly-associated-with-autoimmune-liver-and-biliary-diseases-in-finnish-children
#4
E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leucocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged three years to 15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011, and followed up for four years and three months to 14...
October 19, 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27749626/acute-liver-failure-outcome-and-value-of-pediatric-end-stage-liver-disease-score-in-pediatric-cases
#5
Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27746620/acute-on-chronic-liver-failure-what-is-in-a-definition
#6
REVIEW
Anil C Anand, Radha K Dhiman
Acute on chronic liver failure (ACLF) is a recently recognized syndrome and its definition has been evolving over last two decades. Currently, there is no universal consensus about the definition as kind of cases being seen in the Western world appear to be somewhat different from those that are seen in Asia Pacific region. But every one agrees that definition of ACLF should include following components. (a) The status of pre-existing liver disease, (b) defining the acute insult that leads to rapid deterioration of liver status, (c) time frame during which the acute insult can lead to rapid deterioration, (d) the quantification and definition of liver failure status after deterioration, which will determine the severity of ACLF, and (e) prediction of prognosis after analyzing first four components in the short and long terms...
September 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27734496/pirfenidone-ameliorates-concanavalin-a-induced-hepatitis-in-mice-via-modulation-of-reactive-oxygen-species-nuclear-factor-kappa-b-signalling-pathways
#7
Dina S El-Agamy
OBJECTIVES: This study aimed to evaluate the potential protective effects of pirfenidone (PFD) against concanavalin A (Con A)-induced hepatitis in mice. METHODS: Autoimmune model of hepatitis was established using single intravenous injection of Con A. Mice were randomly assigned into four groups as follows: control group; Con A group; and two groups, receiving PFD in two dose levels (200, 300 mg/kg) for 5 days before Con A administration. Extent of hepatitis was studied using biochemical, histopathological and immunohistochemical estimations...
December 2016: Journal of Pharmacy and Pharmacology
https://www.readbyqxmd.com/read/27732962/soluble-fibrinogen-like-protein-2-sfgl2-the-novel-effector-molecule-for-immunoregulation
#8
Xin-Guang Liu, Yu Liu, Feng Chen
Soluble fibrinogen-like protein 2 (sFGL2) is the soluble form of fibrinogen-like protein 2 belonging to the fibrinogen-related protein superfamily. It is now well characterized that sFGL2 is mainly secreted by regulatory T cell (Treg) populations, and exerts potently immunosuppressive activities. By repressing not only the differentiation and proliferation of T cells but also the maturation of dendritic cells (DCs), sFGL2 acts largely as an immunosuppressant. Moreover, sFGL2 also induces apoptosis of B cells, tubular epithelial cells (TECs), sinusoidal endothelial cells (SECs), and hepatocytes...
October 8, 2016: Oncotarget
https://www.readbyqxmd.com/read/27723989/-extrahepatic-manifestations-of-hcv-infection
#9
Petr Husa
Extrahepatic manifestations of hepatitis C virus infection (HCV) are very common. The most common of these is mixed cryoglobulinaemia. Anti-HCV antibodies and viral ribonucleic acid, HCV RNA, can be found in the cryoprecipitates, together with the rheumatoid factor. Cryoglobulins consist of a complex of immunoglobulins that in vitro precipitate upon the cooling bellow the human body temperature. Vasculitis is caused by the deposition of such immune complexes in the small blood vessels. A link with the HCV infection is considered to be established with membranoproliferative glomerulonephritis, leukocytoclastic vasculitis, lymphoproliferative disorders (in particular B cell lymphoma), Sjögren and sicca syndrome, lichen planus, porfyria cutanea tarda and diabetes mellitus...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27696367/association-of-hepatitis-b-virus-infection-with-decreased-ischemic-stroke
#10
C-H Tseng, C-H Muo, C-Y Hsu, C-H Kao
OBJECTIVES: Inflammatory processes (both infections and autoimmune diseases) may cause endothelial dysfunction and arterial atherosclerosis, subsequently increasing the risk of acute ischemic stroke (AIS). In this investigation, we analyzed the association between hepatitis B virus (HBV) infection and AIS risk. METHODS: A Taiwan national insurance claims data set of 1,000,000 patients was used to extract 22,303 patients with HBV and 89,212 randomly selected sex- and age-matched controls from the beginning of 2000 to the end of 2006...
November 2016: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/27688687/incidence-and-characteristics-of-hepatitis-e-virus-infection-in-children-in-assiut-upper-egypt
#11
Gamal Hasan, Asaad Assiri, Naglaa Marzuuk, Enas Daef, Sayed Abdelwahab, Ahmed Ahmed, Ismail Mohamad, Ayman Al-Eyadhy, Ali Alhaboob, Mohamad-Hani Temsah
OBJECTIVE: To describe the characteristics of hepatitis E virus (HEV) infection in a cohort of children from Upper Egypt using data from a large multicentre prospective study of acute viral hepatitis (AVH). METHODS: Data from subjects aged 2-18 years with AVH or close contacts of those with AVH found to have asymptomatic AVH were included in the analysis. Information concerning medical history, clinical examination, liver function tests and screening for hepatotropic viruses was recorded and analysed...
September 29, 2016: Journal of International Medical Research
https://www.readbyqxmd.com/read/27688660/etiology-of-chronic-liver-diseases-in-the-northwest-of-italy-1998-through-2014
#12
Giorgio Maria Saracco, Andrea Evangelista, Sharmila Fagoonee, Giovannino Ciccone, Elisabetta Bugianesi, Gian Paolo Caviglia, Maria Lorena Abate, Mario Rizzetto, Rinaldo Pellicano, Antonina Smedile
AIM: To assess the etiology of chronic liver diseases (CLD) from 1998 to 2014 at the outpatient clinic of Gastroenterology of the main hospital in Northwest of Italy among those dedicated to hepatology. METHODS: A random sample of charts of patients referred to for increased liver enzymes between January 1998 and December 2006, and between January 2012 and December 2014 were reviewed. Etiology search included testing for hepatitis B virus (HBV), hepatitis C virus (HCV), autoimmune hepatitis, primary biliary cirrhosis, Wilson's disease and hereditary hemocromatosis...
September 28, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27649967/autoimmune-liver-diseases-in-the-asia-pacific-region-proceedings-of-apasl-symposium-on-aih-and-pbc-2016
#13
Atsushi Tanaka, Xiong Ma, Osamu Yokosuka, Martin Weltman, Hong You, Deepak N Amarapurkar, Yoon Jun Kim, Zaigham Abbas, Diana A Payawal, Ming-Ling Chang, Cumali Efe, Ersan Ozaslan, Masanori Abe, Robert Mitchell-Thain, Mikio Zeniya, Kwang Hyub Han, John M Vierling, Hajime Takikawa
During the 25th annual meeting of the Asia-Pacific Association for the Study of the Liver (APASL 2016) in Tokyo, we organized and moderated an inaugural satellite symposium on the autoimmune liver diseases, autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). Following the keynote lecture by John M. Vierling (USA), speakers from the Asia-Pacific region provided an up-to-date perspective on the epidemiology, clinical practice and research in AIH and PBC in the Asia-Pacific region. Although epidemiology and clinical features of AIH seem to be similar in East Asia compared to those in western countries, the majority of patients with AIH are detected at an advanced stage and have higher mortality rates in South Asia, indicating an unmet need for earlier diagnosis and the initiation of appropriate immunosuppressive treatment...
September 20, 2016: Hepatology International
https://www.readbyqxmd.com/read/27631819/characterization-of-the-specificity-functionality-and-durability-of-host-t-cell-responses-against-the-full-length-hepatitis-e-virus
#14
Anthony Brown, John S Halliday, Leo Swadling, Richie G Madden, Richard Bendall, Jeremy G Hunter, James Maggs, Peter Simmonds, Donald B Smith, Louisa Vine, Cara McLaughlin, Jane Collier, David Bonsall, Katie Jeffery, Susanna Dunachie, Paul Klenerman, Jacques Izopet, Nassim Kamar, Harry R Dalton, Eleanor Barnes
: The interplay between host antiviral immunity and immunopathology during hepatitis E virus (HEV) infection determines important clinical outcomes. We characterized the specificity, functionality, and durability of host T-cell responses against the full-length HEV virus and assessed a novel "Quantiferon" assay for the rapid diagnosis of HEV infection. Eighty-nine volunteers were recruited from Oxford, Truro (UK), and Toulouse (France), including 44 immune-competent patients with acute HEV infection, 18 HEV-exposed immunosuppressed organ-transplant recipients (8 with chronic HEV), and 27 healthy volunteers...
December 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27631617/quantitation-of-the-rank-rankl-axis-in-primary-biliary-cholangitis
#15
Ana Lleo, Zhaolian Bian, Haiyan Zhang, Qi Miao, Fang Yang, Yanshen Peng, Xiaoyu Chen, Ruqi Tang, Qixia Wang, Dekai Qiu, Jingyuan Fang, Cristina Sobacchi, Anna Villa, Luca Di Tommaso, Massimo Roncalli, M Eric Gershwin, Xiong Ma, Pietro Invernizzi
There is substantial data that suggests an abnormality of innate immunity in patients with primary biliary cholangitis (PBC) which includes the transcription factor nuclear factor-kB (NF-kB) and well as downstream inflammatory signaling pathways. In addition, ImmunoChip analysis has identified a novel PBC-associated locus near the receptor activator of NF-kB ligand (RANKL) gene. Based on these observations, we investigated the role of the RANKL axis in the liver of patients with PBC compared to controls. We used immunohistochemistry to quantitate liver expression of RANKL, its receptor (RANK), and importantly the decoy receptor osteoprotegerin (OPG), including a total of 122 liver samples (PBC = 37, primary sclerosing cholangitis = 20, autoimmune hepatitis = 26, chronic hepatitis B = 32 and unaffected controls = 7)...
2016: PloS One
https://www.readbyqxmd.com/read/27630169/galectin-3-regulates-inflammasome-activation-in-cholestatic-liver-injury
#16
Jijing Tian, Guoxiang Yang, Huan-Yuan Chen, Daniel K Hsu, Alexey Tomilov, Kristin A Olson, Ali Dehnad, Sarah R Fish, Gino Cortopassi, Bin Zhao, Fu-Tong Liu, M Eric Gershwin, Natalie J Török, Joy X Jiang
Macrophage activation is an important feature of primary biliary cholangitis (PBC) pathogenesis and other cholestatic liver diseases. Galectin-3 (Gal3), a pleiotropic lectin, is produced by monocytic cells and macrophages. However, its role in PBC has not been addressed. We hypothesized that Gal3 is a key to induce NOD-like receptor family, pyrin domain containing 3 (NLRP3) inflammasome in macrophages and in turn to propagate proinflammatory IL-17 signaling. In liver tissues from patients with PBC and dnTGF-βRII mice, a model of autoimmune cholangitis, the expression of Gal3, NLRP3, and the adaptor protein adaptor apoptosis-associated speck-like protein was induced, with the downstream activation of caspase-1 and IL-1β...
December 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/27612218/causes-of-altered-liver-function-tests-the-role-of-alpha-1-antitrypsin
#17
J Stollenwerk, M Schepke, E Biecker
BACKGROUND: Altered liver function tests are a common finding in clinical practice. Our retrospective study aimed to identify the diagnoses in a non-selected cohort of patients with altered liver tests and to investigate whether alpha-1 antitrypsin genotyping should be part of the diagnostic workup. PATIENTS AND METHODS: 501 patients who were admitted to our outpatient clinic for further evaluation of altered liver function tests were included in the study. The patients underwent a standardized diagnostic program with history taking, physical examination, laboratory tests and ultrasonography...
September 2016: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/27588307/redefined-clinical-features-and-diagnostic-criteria-in-autoimmune-polyendocrinopathy-candidiasis-ectodermal-dystrophy
#18
Elise M N Ferre, Stacey R Rose, Sergio D Rosenzweig, Peter D Burbelo, Kimberly R Romito, Julie E Niemela, Lindsey B Rosen, Timothy J Break, Wenjuan Gu, Sally Hunsberger, Sarah K Browne, Amy P Hsu, Shakuntala Rampertaap, Muthulekha Swamydas, Amanda L Collar, Heidi H Kong, Chyi-Chia Richard Lee, David Chascsa, Thomas Simcox, Angela Pham, Anamaria Bondici, Mukil Natarajan, Joseph Monsale, David E Kleiner, Martha Quezado, Ilias Alevizos, Niki M Moutsopoulos, Lynne Yockey, Cathleen Frein, Ariane Soldatos, Katherine R Calvo, Jennifer Adjemian, Morgan N Similuk, David M Lang, Kelly D Stone, Gulbu Uzel, Jeffrey B Kopp, Rachel J Bishop, Steven M Holland, Kenneth N Olivier, Thomas A Fleisher, Theo Heller, Karen K Winer, Michail S Lionakis
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare primary immunodeficiency disorder typically caused by homozygous AIRE mutations. It classically presents with chronic mucocutaneous candidiasis and autoimmunity that primarily targets endocrine tissues; hypoparathyroidism and adrenal insufficiency are most common. Developing any two of these classic triad manifestations establishes the diagnosis. Although widely recognized in Europe, where nonendocrine autoimmune manifestations are uncommon, APECED is less defined in patients from the Western Hemisphere...
August 18, 2016: JCI Insight
https://www.readbyqxmd.com/read/27580523/celecoxib-attenuates-depressive-like-behavior-associated-with-immunological-liver-injury-in-c57bl-6-mice-through-tnf-%C3%AE-and-nf-%C3%AE%C2%BAb-dependent-mechanisms
#19
Esraa Moustafa ELnahas, Mahdy Salama Abou Zeid, Hala Salah Abdel Kawy, Nevien Hendawy, Walaa Baher
AIM: Depression associating patients with chronic liver diseases is a major treatment goal. This study aimed to evaluate the potential hepatoprotective and antidepressant effects of celecoxib in a model of experimental autoimmune hepatitis (EAH) and depressive-like behavior in C57BL/6 mice. MAIN METHODS: EAH was induced by immunization with S-100 liver antigen emulsified in complete Freund's adjuvant (CFA). Mice were randomly allocated to 5 groups; control phosphate buffered saline group; control CFA group; EAH group, and 2 groups of EAH plus celecoxib (7...
October 15, 2016: Life Sciences
https://www.readbyqxmd.com/read/27577564/a-case-of-multiple-hepatic-lesions-associated-with-methotrexate-associated-lymphoproliferative-disorder
#20
Ruby Matsumoto, Kazushi Numata, Nobutaka Doba, Koji Hara, Makoto Chuma, Hiroyuki Fukuda, Akito Nozaki, Katsuaki Tanaka, Yoshimi Ishii, Shin Maeda
Patients receiving methotrexate (MTX) for the treatment of autoimmune disease are at a high risk of developing lymphoproliferative disorders (LPD), the so-called methotrexate-associated lymphoproliferative disorders (MTX-LPD). We recently performed abdominal ultrasonography (US) in a patient with rheumatoid arthritis (RA) who had developed hepatic dysfunction during the course of MTX therapy; the examination revealed multiple well-demarcated hepatic tumors with slightly irregular borders, the largest one measuring 9 cm in diameter...
October 2016: Journal of Medical Ultrasonics
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