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https://www.readbyqxmd.com/read/29774578/the-immunobiology-of-rankl-and-myeloid-derived-suppressor-cell-activation-in-igg4-related-sclerosing-cholangitis
#1
Min Lian, Qixia Wang, Xiang Jiang, Jun Zhang, Yiran Wei, Yanmei Li, Bo Li, Weihua Chen, Haiyan Zhang, Qi Miao, Yanshen Peng, Xiao Xiao, Li Sheng, Weici Zhang, Jingyuan Fang, Ruqi Tang, M Eric Gershwin, Xiong Ma
The primary function of myeloid derived suppressor cells (MDSCs) is reflected in their immune modulatory role in several immune-mediated diseases. In IgG4-related disease (IgG4-RD), it has been hypothesized that there are selective regulatory defects that lead to a Th2 bias immune response. Herein we have taken advantage of a large cohort of patients with IgG4-related sclerosing cholangitis (IgG4-SC), the most common extra-pancreatic involvement of IgG4-RD, as well as controls consisting of primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and healthy volunteers, to study MDSC...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29766815/old-and-new-treatments-for-pediatric-autoimmune-hepatitis
#2
Silvia Nastasio, Giuseppe Maggiore, Marco Sciveres, Lorenza Matarazzo
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29756667/soluble-fgl2-restricts-autoimmune-hepatitis-progression-via-suppressing-tc17-and-conventional-cd8-t-cells-function
#3
Guo Ai, Weiming Yan, Haijing Yu, Fang Xiao, Dong Xi, Ke Ma, Jiaquan Huang, Xiaoping Luo, Xiaoyang Wan, Qin Ning
OBJECTIVE: Autoimmune hepatitis (AIH) is an inflammatory disease caused by aberrant immune response to hepatic self-antigens in which regulatory T cells (Tregs) are critical to maintain immunosupression. Soluble form of Fibrinogen-like protein 2 (sFGL2), a novel effector molecule of Treg is rarely investigated in AIH. Here, we dissected the role of sFGL2 in the autoimmune hepatitis and its potential mechanism underlying AIH progression. METHODS: Plasma and intrahepatic sFGL2 levels, as well as Treg cells were measured in both AIH patients and experimental autoimmune hepatitis (EAH) mice...
May 14, 2018: Journal of Gene Medicine
https://www.readbyqxmd.com/read/29755125/clinical-narrative-autoimmune-hepatitis
#4
REVIEW
Paul A Schmeltzer, Mark W Russo
Autoimmune hepatitis (AIH) is an inflammatory liver disease that is characterized by circulating autoantibodies, hypergammaglobulinemia, and a lymphoplasmocytic infiltration with interface hepatitis on liver biopsy. Treatment with corticosteroids and other immunosuppressive agents is effective and early diagnosis can result in near-normal life expectancy. Untreated patients, however, can progress to cirrhosis and liver failure. The clinical presentation is heterogeneous and may pose diagnostic and therapeutic dilemmas...
May 14, 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29753567/junctional-adhesion-molecules-jam-b-and-jam-c-promote-autoimmune-mediated-liver-fibrosis-in-mice
#5
Edith Hintermann, Monika Bayer, Clara Benedetta Conti, Sina Fuchs, Michel Fausther, Patrick S Leung, Michel Aurrand-Lions, Richard Taubert, Josef M Pfeilschifter, Mireen Friedrich-Rust, Detlef Schuppan, Jonathan A Dranoff, M Eric Gershwin, Michael P Manns, Beat A Imhof, Urs Christen
Fibrosis remains a serious health concern in patients with chronic liver disease. We recently reported that chemically induced chronic murine liver injury triggers increased expression of junctional adhesion molecules (JAMs) JAM-B and JAM-C by endothelial cells and de novo synthesis of JAM-C by hepatic stellate cells (HSCs). Here, we demonstrate that biopsies of patients suffering from primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) display elevated levels of JAM-C on portal fibroblasts (PFs), HSCs, endothelial cells and cholangiocytes, whereas smooth muscle cells expressed JAM-C constitutively...
May 9, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29751878/primary-biliary-cholangitis-and-autoimmune-hepatitis
#6
REVIEW
Raul S Gonzalez, Kay Washington
Primary biliary cholangitis and autoimmune hepatitis are common autoimmune diseases of the liver. Both have typical clinical presentations, including certain autoantibodies on serologic testing. Histologic features are also often typical: primary biliary cholangitis shows bile duct destruction (sometimes with granulomas), and autoimmune hepatitis shows prominent portal and lobular lymphoplasmacytic inflammation. Both have a wide differential diagnosis, including one another; they may also simultaneously occur within the same patient...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29751148/salivary-immunoglobulin-levels-in-juvenile-autoimmune-hepatitis
#7
Juliana Ximenes Damasceno, Maria Izabel Florindo Guedes, Christiane Araujo Chaves Leite, André Jalles Monteiro, Manasses Claudino Fonteles, Maria Esther Zurita Jimenez, Vilma de Lima, Thyciana Rodrigues Ribeiro, Cristiane Sá Roriz Fonteles
AIMS: The aim of the present study was to investigate the presence of immunoglobulins (Ig) in whole saliva from patients affected by autoimmune hepatitis (AIH). DESIGN: Twelve individuals with AIH and 12 healthy individuals without (CON) autoimmune hepatitis, aged 8-18 years, participated in this study. Non-stimulated whole saliva was collected and centrifuged. Supernatants were separated and lyophilized. Salivary pH was measured and immunoglobulins were analyzed through ELISA technique...
May 6, 2018: Archives of Oral Biology
https://www.readbyqxmd.com/read/29748000/deletion-of-the-c6orf120-gene-with-unknown-function-ameliorates-autoimmune-hepatitis-induced-by-concanavalin-a
#8
Man-Ka Zhang, Hui-Min Ma, Jian Zhang, Xin-Cheng Song, Xiao-Hui Ye, Yu-Feng Li, Yi-Fan Zhang, Ling-Ling He, Hong-Shan Wei, Xin Li
The present study was conducted to characterize the C6orf120 gene, by using C6orf120 gene-deleted rats (C6orf120- / - ), to determine its role in the development and severity of autoimmune hepatitis induced by concanavalin A (Con A), as well as the underlying mechanisms. We found that following Con A injection, C6orf120- / - rats were less susceptible to developing autoimmune hepatitis with low levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) post challenge. Additionally, C6orf120 deficiency increased the frequency of cluster of differentiation (CD)4+ CD25+ Forkhead box P3+ regulatory T cells (Tregs) among intrahepatic lymphocytes, splenocytes, peripheral blood mononuclear cells, and CD4+ T in vitro...
May 2, 2018: Cellular Immunology
https://www.readbyqxmd.com/read/29744332/clinical-and-pathological-characteristics-of-autoimmune-hepatitis-with-acute-presentation
#9
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/29743801/acute-exacerbation-to-autoimmune-hepatitis-mimicking-acute-viral-hepatitis-a-case-series-and-review-of-literature
#10
Sunil Taneja, Pramod Kumar, Suvradeep Mitra, Ajay Duseja, Ranjana Minz, Ashim Das, Radha K Dhiman, Yogesh Chawla
Background: Acute exacerbation of Autoimmune Hepatitis (AIH) poses a significant challenge for diagnosis as it can mimic acute viral hepatitis especially in absence of autoantibodies and hypergammaglobulinemia. Aim: To determine the clinical, laboratory, histopathological characteristics and response to treatment in AIH patients with acute exacerbation. Methods: A retrospective analysis of 16 patients with acute exacerbation of AIH diagnosed over a period of eight years (2008-2016)...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29743797/natural-history-of-cirrhosis-of-liver-after-first-decompensation-a-prospective-study-in-india
#11
Apurva S Shah, Deepak N Amarapurkar
Background and aims: As liver cirrhosis is a dynamic condition, it is possible to improve survival in decompensated cirrhosis. Hence, we planned a prospective study to determine the natural history of cirrhosis after first decompensation. Methods: We enrolled all patients of liver cirrhosis who presented with first episode of decompensation defined by the presence of ascites, either overt or detected by Ultrasonography (UD), Gastroesophageal Variceal Bleeding (GEVB), and Hepatic Encephalopathy (HE)...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29742944/subcentimetric-incidental-intrahepatic-cholangiocarcinoma-in-an-explant-liver-diagnostic-difficulty-of-a-rare-entity
#12
Suvradeep Mitra, Gude Geethanjali, Sahaj Rathi, Arunanshu Behera, Ashim Das
Incidental intrahepatic cholangiocarcinoma (iCCA) is a rare neoplastic lesion in the explant liver specimens with an approximate incidence of 0.7%. The detection of iCCA is associated with poor prognosis in the posttransplant setting. The occurrence of a subcentimetric iCCA is very rare and poses a major diagnostic challenge to the pathologist. This article presents a rare case of subcentimetric iCCA in a young male in the background of advanced stage chronic liver disease resulting from autoimmune hepatitis possibly with chronic cholangiopathy along with the histomorphological differentials...
May 1, 2018: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29739065/the-activity-of-alcohol-dehydrogenase-isoenzymes-and-aldehyde-dehydrogenase-in-the-sera-of-patients-with-autoimmune-hepatitis
#13
Wojciech Jelski, Blanka Wolszczak-Biedrzycka, Elzbieta Zasimowicz-Majewska, Karolina Orywal, Joanna Piechota, Tadeusz W Lapinski, Marzena Bednarczyk, Maciej Szmitkowski
BACKGROUND: Autoimmune hepatitis (AIH) is a progressive inflammatory hepatopathy and an important cause of end-stage liver. The liver cells' destruction is reflected by increased activity of different enzymes in the serum. These enzymes include alcohol dehydrogenase (ADH) and aldehyde dehydrogenase (ALDH), which play a significant role in the metabolism of many biological substances and exist mainly in the liver. In this study we investigated the activity of alcohol dehydrogenase and its isoenzymes and the total activity of ALDH in the sera of patients with autoimmune hepatitis...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737057/-clinicopathological-analysis-of-autoimmune-hepatitis-with-sj%C3%A3-gren-s-syndrome
#14
Lin-Lin Zhu, Li-Hua Yang, Qi-Bing Xie, Geng Yin
OBJECTIVE: To explore the biochemical-immune and pathological characteristics of autoimmune hepatitis (AIH) with Sjögren's syndrome (SS) . METHODS: A total of 76 cases of AIH patients were included from January 2009 to April 2017. Among them,there were 40 cases of AIH with SS and 36 cases without SS. The liver function,immunological index,histological features,length of first diagnosis and treatment costs were compared between the two groups. RESULTS: For AIH+SS group and AIH group,the proportion of women were 97...
March 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29737040/marked-accumulation-of-fdg-and-inflammatory-cells-expressing-glucose-transporter-3-in-igg4-related-autoimmune-hepatitis
#15
Toshihiro Araki, Teruko Arinaga-Hino, Hironori Koga, Jun Akiba, Tatsuya Ide, Yoshinobu Okabe, Reiichiro Kuwahara, Keisuke Amano, Makiko Yasumoto, Toshihiro Kawaguchi, Tomoya Sano, Reiichiro Kondou, Seiji Kurata, Keiichi Mitsuyama, Takuji Torimura
Immunoglobulin G (IgG) 4 related-autoimmune hepatitis (AIH) is a recently proposed subtype that responds well to steroid treatment; however, its pathogenesis remains unclear. We report here a 65-year-old Japanese female with skin itching and lip swelling. She had liver injury with jaundice, which persisted despite stopping of anti-allergic agents. Blood chemistry revealed highly elevated serum IgG and IgG4 (535 mg/dL) levels, and positive anti-nuclear antibody. The diagnosis of AIH was based on liver biopsy...
May 7, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29734949/a-case-of-hyperparathyroidism-associated-parkinsonism-successfully-treated-with-cinacalcet-hydrochloride-a-calcimimetic
#16
Yuichiro Ohya, Masato Osaki, Shota Sakai, Shunsuke Kimura, Chiharu Yasuda, Tetsuro Ago, Takanari Kitazono, Shuji Arakawa
BACKGROUND: Some metabolic disorders, including abnormal calcium metabolism, can develop and worsen parkinsonism. However, whether hyperparathyroidism can cause parkinsonism remains controversial. CASE PRESENTATION: An 83-year-old woman with a history of right thalamic hemorrhage and drug-induced parkinsonism, was admitted due to worsening of parkinsonian symptoms including mask-like face, bradykinesia, freezing of gait, and rigidity. She had been diagnosed with autoimmune hepatitis and was being treated with prednisolone...
May 7, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29731495/a-case-of-sj%C3%A3-gren-s-syndrome-complicated-with-interstitial-nephritis-and-delayed-onset-autoimmune-hepatitis
#17
Takehisa Yamada, Megumi Fukui, Tetsuya Kashiwagi, Taeang Arai, Norio Itokawa, Masanori Atsukawa, Akira Shimizu, Shuichi Tsuruoka
A 61-year-old woman was admitted to our hospital because of muscle paralysis and was found to have severe hypokalemia. A gallium-67 scintigram revealed a positive accumulation in the bilateral salivary glands, and a labial minor salivary gland biopsy demonstrated a massive lymphocyte infiltrate around the salivary ducts. She was diagnosed with Sjögren's syndrome (SS) associated with renal tubular acidosis. Renal biopsy revealed tubulointerstitial nephritis with a mild focal infiltration of lymphocytes and plasma cells...
2018: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29729445/emerging-role-of-il-35-in-inflammatory-autoimmune-diseases
#18
REVIEW
Lin-Chong Su, Xiao-Yan Liu, An-Fang Huang, Wang-Dong Xu
Interleukin 35 (IL-35) is the recently identified member of the IL-12 family of cytokines and provides the possibility to be a target for new therapies for autoimmune, inflammatory diseases. It is composed of an α chain (p35) and a β chain (EBI3). IL-35 mediates signaling by binding to its receptors, activates subsequent signaling pathways, and therefore, regulates the differentiation, function of T, B cells, macrophages, dendritic cells. Recent findings have shown abnormal expression of IL-35 in inflammatory autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, multiple sclerosis, type 1 diabetes, psoriasis, multiple sclerosis, autoimmune hepatitis, experimental autoimmune uveitis...
May 3, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29729087/liver-injury-after-pulsed-methylprednisolone-therapy-in-multiple-sclerosis-patients
#19
Viviana Nociti, Marco Biolato, Chiara De Fino, Assunta Bianco, Francesco Antonio Losavio, Matteo Lucchini, Giuseppe Marrone, Antonio Grieco, Massimiliano Mirabella
OBJECTIVES: High-dose pulsed methylprednisolone-related liver injury cases have been reported in the literature, but a prospective study in patients with multiple sclerosis (MS) has never been performed. The aim of this study was to evaluate the prevalence and severity of liver injury in patients with MS after pulsed methylprednisolone therapy. METHODS: We performed a prospective observational single-center study on patients with MS treated with i.v. methylprednisolone 1,000 mg/day for 5 days...
May 4, 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29725696/-autoimmune-hepatitis-standard-and-second-line-therapy
#20
REVIEW
R Taubert, E Jaeckel
BACKGROUND: Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease. Like other autoimmune diseases, the incidence continues to rise and, if left untreated, it has a detrimental natural course. AIM: Description of the current etiology, diagnosis and management of AIH. MATERIALS AND METHODS: Summary of current national as well as international guidelines and highlighting recently published studies. RESULTS AND CONCLUSION: In addition to autoantibody testing and liver histology, the diagnosis of AIH is based on the exclusion of other relevant liver diseases...
May 3, 2018: Der Internist
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