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Autoimmune liver disease

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https://www.readbyqxmd.com/read/28438569/methylprednisolone-liver-toxicity-a-new-case-and-a-french-regional-pharmacovigilance-survey
#1
Jérôme Dumortier, Judith Cottin, Caroline Lavie, Olivier Guillaud, Valérie Hervieu, Christine Chambon-Augoyard, Jean-Yves Scoazec, Sandra Vukusic, Thierry Vial
Reported hepatotoxicity induced by corticosteroids is very rare, and the diagnosis is highly challenging in the context of auto-immune disease. We report here a case of high-dose methylprednisolone (MP)-induced acute hepatitis confirmed by liver histology in a patient with multiple sclerosis (MS) and a case series (n=4) notified to the French Pharmacovigilance center of Lyon. In all 5 cases, other common causes of hepatitis were excluded. The causal relationship with MP pulse therapy was supported by the fact that MP was the only culprit drug...
April 21, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28435998/a-6-year-old-boy-with-wilson-disease-a-diagnostic-dilemma
#2
Ramaswamy Ganesh, N Suresh, T Vasanthi, Malathi Sathiyasekaran, R Thulasiraman
A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, and coagulopathy. Viral markers and slit lamp examination for Kayser-Fleischer ring were negative. Serum ceruloplasmin and 24-h urinary copper post-D-pencillamine challenge were normal. Anti-smooth muscle antibody was positive 1:20, and liver biopsy showed micronodular cirrhosis with abundant Mallory hyaline and stainable copper deposits...
April 24, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28433708/toxicological-characterisation-of-two-novel-selective-aryl-hydrocarbon-receptor-modulators-in-sprague-dawley-rats
#3
Selma Mahiout, Jere Lindén, Javier Esteban, Ismael Sánchez-Pérez, Satu Sankari, Lars Pettersson, Helen Håkansson, Raimo Pohjanvirta
The aryl hydrocarbon receptor (AHR) mediates the toxicity of dioxins, but also plays important physiological roles. Selective AHR modulators, which elicit some effects imparted by this receptor without causing the marked toxicity of dioxins, are presently under intense scrutiny. Two novel such compounds are IMA-08401 (N-acetyl-N-phenyl-4-acetoxy-5-chloro-1,2-dihydro-1-methyl-2-oxo-quinoline-3-carboxamide) and IMA-07101 (N-acetyl-N-(4-trifluoromethylphenyl)-4-acetoxy-1,2-dihydro-5-methoxy-1-methyl-2-oxo-quinoline-3-carboxamide)...
April 19, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28433112/wilson-disease-liver-pathology
#4
Maciej Pronicki
The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28432836/autoimmune-hepatitis-from-current-knowledge-and-clinical-practice-to-future-research-agenda
#5
REVIEW
Marcial Sebode, Johannes Hartl, Diego Vergani, Ansgar W Lohse
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease. Unknown triggers lead to a mainly T cell-mediated immune response targeting the liver, the main auto-antigen of which has not been identified yet. The diagnosis of AIH is based on the elevation of immunoglobulin G / hypergammaglobulinemia, detection of characteristic autoantibodies as well as a typical pattern on liver histology. Exclusion of other causes of hepatitis and response to immunosuppressive treatment support the diagnosis of AIH...
April 22, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28428284/acute-presentation-of-autoimmune-hepatitis-a-multicentre-study-with-detailed-histological-evaluation-in-a-large-cohort-of-patients
#6
Hiep Nguyen Canh, Kenichi Harada, Hirofumi Ouchi, Yasunori Sato, Koichi Tsuneyama, Masayoshi Kage, Masayuki Nakano, Kaname Yoshizawa, Atsushi Takahashi, Masanori Abe, Jong-Hon Kang, Kazuhiko Koike, Ayano Inui, Tomoo Fujisawa, Akinobu Takaki, Teruko Arinaga-Hino, Takuji Torimura, Yoshiyuki Suzuki, Keiichi Fujiwara, Mikio Zeniya, Hiromasa Ohira, Atsushi Tanaka, Hajime Takikawa
AIMS: Although liver biopsy is crucial to diagnose and guide treatment decisions, a detailed histological analysis of autoimmune hepatitis (AIH) with clinically acute presentations has not yet been performed. This study aimed to characterise the histological features and explore potential histological hallmarks to diagnose the acute presentation of AIH. METHODS: We systematically evaluated liver specimens of 87 adult patients with acute presentation of AIH retrospectively enrolled from Japanese multicentre facilities...
April 20, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#7
Gideon M Hirschfield, Ulrich Beuers, Christophe Corpechot, Pietro Invernizzi, David Jones, Marco Marzioni, Christoph Schramm
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 17, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28425483/a-genome-wide-association-study-identifies-six-novel-risk-loci-for-primary-biliary-cholangitis
#8
Fang Qiu, Ruqi Tang, Xianbo Zuo, Xingjuan Shi, Yiran Wei, Xiaodong Zheng, Yaping Dai, Yuhua Gong, Lan Wang, Ping Xu, Xiang Zhu, Jian Wu, Chongxu Han, Yueqiu Gao, Kui Zhang, Yuzhang Jiang, Jianbo Zhou, Youlin Shao, Zhigang Hu, Ye Tian, Haiyan Zhang, Na Dai, Lei Liu, Xudong Wu, Weifeng Zhao, Xiaomin Zhang, Zhidong Zang, Jinshan Nie, Weihao Sun, Yi Zhao, Yuan Mao, Po Jiang, Hualiang Ji, Qing Dong, Junming Li, Zhenzhong Li, Xinli Bai, Li Li, Maosong Lin, Ming Dong, Jinxin Li, Ping Zhu, Chan Wang, Yanqiu Zhang, Peng Jiang, Yujue Wang, Rohil Jawed, Jing Xu, Yu Zhang, Qixia Wang, Yue Yang, Fan Yang, Min Lian, Xiang Jiang, Xiao Xiao, Yanmei Li, Jingyuan Fang, Dekai Qiu, Zhen Zhu, Hong Qiu, Jianqiong Zhang, Wenyan Tian, Sufang Chen, Ling Jiang, Bing Ji, Ping Li, Guochang Chen, Tianxue Wu, Yan Sun, Jianjiang Yu, Huijun Tang, Michun He, Min Xia, Hao Pei, Lihua Huang, Zhuye Qing, Jianfang Wu, Qinghai Huang, Junhai Han, Wei Xie, Zhongsheng Sun, Jian Guo, Gengsheng He, M Eric Gershwin, Zhexiong Lian, Xiang Liu, Michael F Seldin, Xiangdong Liu, Weichang Chen, Xiong Ma
Primary biliary cholangitis (PBC) is an autoimmune liver disease with a strong hereditary component. Here, we report a genome-wide association study that included 1,122 PBC cases and 4,036 controls of Han Chinese descent, with subsequent replication in a separate cohort of 907 PBC cases and 2,127 controls. Our results show genome-wide association of 14 PBC risk loci including previously identified 6p21 (HLA-DRA and DPB1), 17q12 (ORMDL3), 3q13.33 (CD80), 2q32.3 (STAT1/STAT4), 3q25.33 (IL12A), 4q24 (NF-κB) and 22q13...
April 20, 2017: Nature Communications
https://www.readbyqxmd.com/read/28417923/anemia-in-kawasaki-disease-hepcidin-as-a-potential-biomarker
#9
REVIEW
Ying-Hsien Huang, Ho-Chang Kuo
Kawasaki disease (KD) is an autoimmune-like disease and acute childhood vasculitis syndrome that affects various systems but has unknown etiology. In addition to the standard diagnostic criteria, anemia is among the most common clinical features of KD patients and is thought to have a more prolonged duration of active inflammation. In 2001, the discovery of a liver-derived peptide hormone known as hepcidin began revolutionizing our understanding of anemia's relation to a number of inflammatory diseases, including KD...
April 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28400566/serum-metabolomics-analysis-reveals-a-distinct-metabolic-profile-of-patients-with-primary-biliary-cholangitis
#10
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/28396038/severe-reduction-of-blood-lysosomal-acid-lipase-activity-in-cryptogenic-cirrhosis-a-nationwide-multicentre-cohort-study
#11
Francesco Angelico, Stefano Ginanni Corradini, Daniele Pastori, Silvia Fargion, Anna Ludovica Fracanzani, Mario Angelico, Luigi Bolondi, Giulia Tozzi, Pietro Luigi Pujatti, Giancarlo Labbadia, Gino Roberto Corazza, Maurizio Averna, Francesco Perticone, Giuseppe Croce, Marcello Persico, Tommaso Bucci, Francesco Baratta, Licia Polimeni, Maria Del Ben, Francesco Violi
BACKGROUND AND AIMS: Blood lysosomal acid lipase (LAL) is reduced in non-alcoholic steatohepatitis, which is the major cause of cryptogenic cirrhosis (CC); few data on LAL activity in CC do exist. We investigated LAL activity in a cohort of patients with liver cirrhosis. METHODS: This is a multicentre cohort study including 274 patients with liver cirrhosis of different aetiology from 19 centres of Internal Medicine, Gastroenterology and Hepatology distributed throughout Italy...
March 31, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28392163/current-progress-in-non-viral-rnai-based-delivery-strategies-to-lymphocytes
#12
REVIEW
Shoshy Mizrahy, Inbal Hazan-Halevy, Niels Dammes, Dalit Landesman-Milo, Dan Peer
RNAi-based therapy holds great promise, as it can be utilized for the treatment of multiple conditions in an accurate manner via sequence-specific manipulation of gene expression. To date, RNAi therapeutics have advanced into clinical trials for liver diseases and solid tumors; however, delivery of RNAi to leukocytes in general and to lymphocytes in particular remains a challenge. Lymphocytes are notoriously hard to transduce with RNAi payloads and are disseminated throughout the body, often located in deep tissues; therefore, developing an efficient systemic delivery system directed to lymphocytes is not a trivial task...
April 6, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#13
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28381383/serum-soluble-st2-is-a-promising-prognostic-biomarker-in-hbv-related-acute-on-chronic-liver-failure
#14
Shao-Wen Jiang, Peng Wang, Xiao-Gang Xiang, Rui-Dong Mo, Lan-Yi Lin, Shi-San Bao, Jie Lu, Qing Xie
BACKGROUND: The IL-33/ST2 axis is involved in the pathogenesis of many diseases such as autoimmune diseases, cancer, and heart failure. However, studies of the IL-33/ST2 pathway in HBV-related acute-on-chronic liver failure (HBV-ACLF) are lacking. The present study aimed to determine the prognostic role of serum IL-33/soluble ST2 (sST2) in HBV-ACLF. METHODS: Serum levels of IL-33 and sST2 in healthy controls (HC, n=18), chronic hepatitis B (CHB, n=27) and HBV-ACLF (n=51) patients at the 1st and 4th week after enrollment were detected using ELISA, and clinical data were collected...
April 2017: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/28378419/successful-treatment-of-pemphigus-foliaceus-in-a-berrichon-du-cher-ram-with-methylprednisolone-acetate
#15
Bianca Lambacher, Julia Schoiswohl, Rene Brunthaler, Thomas Wittek, Reinhild Krametter-Frötscher
BACKGROUND: Pemphigus foliaceus is a severe, autoimmune blistering skin disease, which is described in humans and some animal species. In small ruminants pemphigus foliaceus has rarely been described and, to the best of the authors' knowledge, little information is available about successful treatment in sheep. AIM: This case report describes a Berrichon du Cher ram with the presumed diagnosis of pemphigus foliaceus. METHODS: The ram was treated with methylprednisolone acetate 40 mg at a dosage of 2...
April 4, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28377802/an-observational-study-on-the-association-of-nonalcoholic-fatty-liver-disease-and-metabolic-syndrome-with-gall-stone-disease-requiring-cholecystectomy
#16
Farah Ahmed, Qamaruddin Baloch, Zahid Ali Memon, Iqra Ali
OBJECTIVE: Recognition of Non alcoholic fatty liver disease (NAFLD) and metabolic syndrome in patients with gallstones undergoing laparoscopic or open cholecystectomy, along with it we will also study the life style of patients with gall stones. BACKGROUND: Patients with gallstones have associated NAFLD, with concurrent metabolic syndrome and these ailments share similar factors for example obesity, hypertriglyceridemia and diabetes mellitus. Factors like body mass index, gender, raised lipid levels, use of contraceptives and alcohol and having diabetes, physical inactiveness, multiparous women, water with excessive iron content, metabolic syndrome, and NAFLD are accountable factors for gallstones formation...
May 2017: Annals of Medicine and Surgery
https://www.readbyqxmd.com/read/28376624/autochthonous-acute-hepatitis-e-an-increasingly-frequent-diagnosis-clinical-epidemiological-analysis-of-our-experience
#17
Luzdivina Monteserín Ron, Marcos Jiménez Palacios, Pedro Linares Torres, Aleida Miguel Peña, Begoña Álvarez Cuenllas, María Isabel Fernández-Natal, Emilio Valverde Romero, Francisco Jorquera Plaza
BACKGROUND: In Europe, acute hepatitis caused by the hepatitis E virus (HEV) traditionally was an infection found in people who had travelled to endemic zones, mainly Asia and Africa. However, a growing number of sporadic autochthonous cases are now being diagnosed in the Western world. OBJECTIVE: To analyze the cases of acute HEV hepatitis diagnosed in our setting, with the identification of the clinical-epidemiological characteristics. MATERIAL AND METHODS: We included the cases of acute HEV hepatitis diagnosed (positive anti-HEV IgM and/or HEV RNA present in serum) between January 2008 and December 2014...
April 5, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28376623/the-clinical-extremes-of-autoimmune-cholangitis
#18
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/28376538/-hepatic-manifestation-of-a-macrophage-activation-syndrome-mas
#19
Michael Nagel, Andreas Schwarting, Beate K Straub, Peter R Galle, Tim Zimmermann
Background Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure...
April 4, 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/28372806/multiple-immune-disorders-after-natalizumab-discontinuation-after-the-ciris-the-siris
#20
E K Van Obberghen, M Cohen, F Rocher, C Lebrun-Frenay
Natalizumab (NTZ) is an effective treatment for patients with highly active relapsing remitting multiple sclerosis (MS). However, when the therapy must be interrupted, it is important to anticipate the withdrawal to avoid reactivation or disease rebound. Described here is the case of a 35-year-old woman, with a past history of beta thalassemia, bulimia and asthma, who was diagnosed with MS at age 26. She was treated initially with first-line subcutaneous (sc) immunomodulatory treatments. However, due to liver toxicity, interferon beta-1a sc was interrupted and replaced by glatiramer acetate treatment, which was well tolerated and used for several years...
March 31, 2017: Revue Neurologique
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