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https://www.readbyqxmd.com/read/28716871/igg4-related-disease-long-term-natural-history-and-management-of-a-relapsing-multisystem-disease-entity
#1
Sreelakshmi Kotha, Giovanni Tritto, Terry Wong, Philip Berry
Although awareness of IgG4-related disease has grown over the past decade, with earlier diagnosis and treatment, understanding of its natural history over the long term and the optimal management remains unclear. We report the case of a 48-year-old man who presented with a pancreatic pseudotumour causing bile duct obstruction with coexisting autoimmune hepatitis and multisystem involvement. His symptoms settled on steroids and maintenance with azathioprine was commenced, however periodic relapses occurred involving multiple organs...
July 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28712788/antimalarial-agent-artesunate-protects-concanavalin-a-induced-autoimmune-hepatitis-in-mice-by-inhibiting-inflammatory-responses
#2
Xin Zhao, MingJiang Liu, JinGui Li, ShaoJie Yin, YangYang Wu, AnYuan Wang
The anti-malarial drug artesunate (ARS) has been shown to possess anti-inflammatory activity. Its effect on autoimmune hepatitis remains unclear. Concanavalin A (Con A)-induced hepatitis was used in this study to reveal the potential action of ARS and the related mechanism. Mice were pretreated with ARS followed by Con A challenge. Con A caused obvious hepatic injury with higher levels of liver enzymes, elevated pro-inflammatory cytokines and activation of nuclear factor-κB (NF-κB) and mitogen activated protein kinase (MAPK) signaling pathways...
July 13, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28711687/methotrexate-therapy-for-autoimmune-hepatitis
#3
James Haridy, Amanda Nicoll, Siddharth Sood
No abstract text is available yet for this article.
July 12, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28710658/global-disparities-and-their-implications-in-the-occurrence-and-outcome-of-autoimmune-hepatitis
#4
REVIEW
Albert J Czaja
Autoimmune hepatitis has a variable occurrence, clinical phenotype, and outcome, and the factors contributing to this variability are uncertain. The goals of this review are to examine the global disparities in the occurrence and outcome of autoimmune hepatitis, suggest bases for these disparities, and encourage investigations that extend beyond single-center experiences. Disparities in the incidence and prevalence of autoimmune hepatitis in different age groups, genders, ethnicities, and geographical regions suggest that factors other than genetic predisposition are involved...
July 14, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28706981/outcomes-of-liver-transplant-recipients-with-autoimmune-liver-disease-using-long-term-dual-immunosuppression-regimen-without-corticosteroid
#5
Sanjaya K Satapathy, Ollie D Jones, Jason M Vanatta, Faisal Kamal, Satish K Kedia, Yu Jiang, Satheesh P Nair, James D Eason
BACKGROUND: Liver transplant (LT) recipients with autoimmune liver disease (primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis) are at increased risk of developing acute cellular rejection (ACR), and in many cases graft failure due to recurrent disease. We describe our experience with dual immunosuppression without steroid maintenance and analyze its effect on disease recurrence; ACR; patient and graft survivals; and complications, such as sepsis and de novo malignancy...
July 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28703133/association-of-autoimmune-hepatitis-with-src-homology-2-adaptor-protein-3-gene-polymorphisms-in-japanese-patients
#6
Takeji Umemura, Satoru Joshita, Hideaki Hamano, Kaname Yoshizawa, Shigeyuki Kawa, Eiji Tanaka, Masao Ota
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease characterized by an autoimmune reaction to hepatocytes. The Src homology 2 adaptor protein 3 (SH2B3) gene is a member of the SH2B family of adaptor proteins that has been implicated in the integration and regulation of multiple signaling events. SH2B3 is involved in cytokine signaling pathways and serves as a negative mediator of T-cell receptor signaling. Genome-wide association analyses in Caucasians have linked a missense mutation at rs3184504 in SH2B3 with AIH...
July 13, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28700730/pediatric-autoimmune-hepatitis-shows-a-disproportionate-decline-of-regulatory-t-cells-in-the-liver-and-of-il-2-in-the-blood-of-patients-undergoing-therapy
#7
Jana Diestelhorst, Norman Junge, Jerome Schlue, Christine S Falk, Michael P Manns, Ulrich Baumann, Elmar Jaeckel, Richard Taubert
BACKGROUND & AIMS: The autoimmune hepatitis (AIH) is a chronic hepatitis driven by the adaptive immunity that affects all age groups. A functional and numerical regulatory T cell (Treg) defect has been reported in pediatric AIH (pAIH), while an intrahepatic increase in adult AIH (aAIH) patients has been detected in current research findings. METHODS: Therefore, we quantified the intrahepatic numbers of Treg, T and B cells, as well as serum cytokine levels before and during therapy in pAIH...
2017: PloS One
https://www.readbyqxmd.com/read/28694671/substantial-hepatic-necrosis-is-prognostic-in-fulminant-liver-failure
#8
Paul Ndekwe, Marwan S Ghabril, Yong Zang, Steven A Mann, Oscar W Cummings, Jingmei Lin
AIM: To evaluate if any association existed between the extent of hepatic necrosis in initial liver biopsies and patient survival. METHODS: Thirty-seven patients with fulminant liver failure, whose liver biopsy exhibited substantial necrosis, were identified and included in the study. The histological and clinical data was then analyzed in order to assess the relationship between the extent of necrosis and patient survival, with and without liver transplantation...
June 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28694068/jkb-122-is-effective-alone-or-in-combination-with-prednisolone-in-con-a-induced-hepatitis
#9
Mei-Chi Hsu, Sheng-Hung Liu, Chiung-Wen Wang, Nai-Yun Hu, Edwin S C Wu, Ying-Chu Shih, Peter J S Chiu
Con A-induced hepatitis in mice is an established model of autoimmune hepatitis (AIH). JKB-122, a toll-like receptor 4 (TLR4) antagonist, was tested for hepatotprotectant activity. Within several hours of Con A challenge (15mg/kg iv), increased production of proinflammatory cytokines with inflammatory infiltrate occurred in the liver. The severity of tissue necrosis and the amount of circulating liver enzymes peak at 24h post Con A challenge. JKB-122 was given 24 and 16h before, then concurrently, and 4 and 8h (× 5 doses) after challenge with Con A...
July 8, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28691216/statins-and-ace-inhibitors-are-associated-with-reduced-mortality-and-morbidity-in-chronic-liver-disease
#10
Knut Stokkeland, Christine Takami Lageborn, Anders Ekbom, Jonas Höijer, Matteo Bottai, Per Stål, Karin Söderberg-Löfdal
Liver fibrosis is a common response to many chronic liver diseases. The aim of our study was to explore whether pharmacotherapy for concurrent diseases affects overall mortality, liver-related mortality and liver-related morbidity in patients with chronic liver disease. We performed a register-based cohort study of all patients with a first-time diagnosis of chronic liver disease between 2005 and 2012 in Sweden (n=70 546). Data from the Patient Register, the Prescribed Drug Register and the Death Certificate Register were linked...
July 10, 2017: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28690845/diagnostic-autoantibodies-for-autoimmune-liver-diseases
#11
REVIEW
Ban-Hock Toh
Autoimmune liver diseases are conditions of low prevalence that comprise the triad of autoimmune hepatitis, primary biliary cholangitis (cirrhosis) and primary sclerosing cholangitis and their poorly characterised overlapping syndromes. Diagnostic autoantibodies are associated with autoimmune hepatitis and primary biliary cholangitis but not with primary sclerosing cholangitis. Autoantibodies are useful disease markers that facilitate early diagnosis of autoimmune hepatitis and primary biliary cholangitis and allow for therapeutic intervention to prevent progression to liver cirrhosis and associated complications...
May 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/28680339/exceptional-manifestation-of-polyautoimmunity-in-a-very-young-girl-a-case-report
#12
Agnieszka Mroczkowska-Juchkiewicz, Jacek Postępski, Edyta Olesińska, Paulina Krawiec, Elżbieta Pac-Kożuchowska
Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. The exact pathogenic mechanisms responsible for the coexistence of distinct autoimmune diseases within an individual have not been clearly explained. We report a case of a very young girl with the extremely rare co-existence of four distinct autoimmune diseases i.e. juvenile idiopathic arthritis, type 1 diabetes mellitus, coeliac disease and autoimmune hepatitis, recognized based on validated international classification criteria...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28675685/challenges-and-difficulties-in-pathological-diagnosis-of-autoimmune-hepatitis
#13
REVIEW
Kenichi Harada, Nguyen Canh Hiep, Hiromasa Ohira
Autoimmune hepatitis (AIH) with acute presentation is widely recognized as a distinct clinical entity, and its clinicopathology has been extensively studied. In most cases, AIH with acute presentation is merely acute exacerbation of classical chronic AIH, but pure acute-onset AIH without previous symptoms of chronic liver disease is also encountered. Rapid diagnosis and initiation of immunosuppressive treatment are necessary for both acute exacerbation and acute-onset to prevent fatal liver failure. The diagnostic criteria commonly used for classical AIH are generally applicable to acute exacerbation, but acute-onset AIH may present with additional pathological features...
July 3, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28667665/the-relationship-between-portopulmonary-hypertension-and-splenectomy-mayo-clinic-experience-and-literature-review
#14
Justin M Segraves, Rodrigo Cartin-Ceba, Michael D Leise, Michael J Krowka
BACKGROUND & AIMS: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: Retrospective analysis of patients diagnosed with portopulmonary hypertension between 01/01/1988 - 06/30/2015 at Mayo Clinic, Rochester, MN...
July 1, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#15
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28660716/diagnostic-utility-of-radiological-heterogeneity-in-acute-severe-fulminant-autoimmune-hepatitis
#16
Keiichi Fujiwara, Shin Yasui, Osamu Yokosuka, Shigeto Oda, Naoya Kato
BACKGROUND: Histological examination is useful for the diagnosis of acute severe (fulminant) autoimmune hepatitis (AIH), but it is sometimes difficult to perform liver biopsy due to the complicated coagulopathy and ascites. We have shown that heterogeneous hypoattenuation on unenhanced computed tomography (CT) is a characteristic imaging feature of acute severe (fulminant) AIH. In the present study, we examined the utility of the imaging feature by applying the score to diagnose acute severe (fulminant) AIH...
June 29, 2017: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/28655408/autoimmune-hepatitis-immune-mediated-liver-injury-induced-by-rosuvastatin
#17
Mara Sánchez, Agustin Castiella, Eva Zapata, Leire Zubiaurre, Josu Pérez-Yeboles, Leire Mendibil, Arantxa Iribarren
No abstract text is available yet for this article.
June 24, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28644343/second-line-agents-in-pediatric-patients-with-autoimmune-hepatitis-a-systematic-review-and-meta-analysis
#18
Andréanne N Zizzo, Pamela L Valentino, Prakesh S Shah, Binita M Kamath
BACKGROUND AND AIMS: Ten percent to 20% of children with autoimmune hepatitis (AIH) require second-line therapy to achieve remission. Although current guidelines exist on first-line management, evidence for second-line therapy in treatment-refractory patients is lacking. Our aim was to perform a systematic review and meta-analysis of the efficacy and safety of second-line treatments used in this population. METHODS: Electronic and manual searches were used to identify potential studies for inclusion...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644341/pediatric-autoimmune-hepatitis-what-do-you-do-when-first-line-therapy-fails
#19
Nanda Kerkar
No abstract text is available yet for this article.
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28638227/relationship-between-serum-adenosine-deaminase-levels-and-liver-histology-in-autoimmune-hepatitis
#20
Murat Torgutalp, Cumali Efe, Hakan Babaoglu, Taylan Kav
AIM: To evaluate the relationship between serum adenosine deaminase (ADA) levels and histological features in patients with autoimmune hepatitis (AIH). METHODS: A total of 80 subjects (52 AIH cases and 28 healthy controls) were included in the study. Patients were diagnosed according to the simplified criteria suggested by the International Autoimmune Hepatitis Group. All of the cases had been diagnosed with AIH between 2010-2015 at Hacettepe University, Department of Gastroenterology...
June 7, 2017: World Journal of Gastroenterology: WJG
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