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autoimmune liver diseases

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https://www.readbyqxmd.com/read/28720215/autoimmune-meningitis-and-encephalitis-in-adult-onset-still-disease-case-report
#1
Bożek Milena, Konopko Magdalena, Wierzba-Bobrowicz Teresa, Witkowski Grzegorz, Makowicz Grzegorz, Sienkiewicz-Jarosz Halina
INTRODUCTION: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs' involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis - potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis...
July 8, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28716651/significance-of-a-to-i-rna-editing-of-transcripts-modulating-pharmacokinetics-and-pharmacodynamics
#2
REVIEW
Masataka Nakano, Miki Nakajima
RNA editing is a post-transcriptional process that alters the nucleotide sequence of RNA transcripts to generate transcriptome diversity. Among the various types of RNA editing, adenosine-to-inosine (A-to-I) RNA editing is the most frequent type of RNA editing in mammals. Adenosine deaminases acting on RNA (ADAR) enzymes, ADAR1 and ADAR2, convert adenosines in double-stranded RNA structures into inosines by hydrolytic deamination. Inosine forms a base pair with cytidine as if it were guanosine; therefore, the conversion may affect the amino acid sequence, splicing, microRNA targeting, and miRNA maturation...
July 14, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28706981/outcomes-of-liver-transplant-recipients-with-autoimmune-liver-disease-using-long-term-dual-immunosuppression-regimen-without-corticosteroid
#3
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/28704189/chronic-hepatitis-due-to-gluten-enteropathy-a-case-report
#4
Irina I Ivanova, Denitsa Y Dukova, Pavlina G Boikova, Lili S Grudeva, Ivan B Shalev, Iskren A Kotzev
BACKGROUND: Celiac disease is an immune-mediated enteropathy precipitated by exposure to dietary gluten in genetically predisposed individuals. CASE DESCRIPTION: A 45-year-old Caucasian woman presented with severe iron-deficient anemia and mild elevation of liver enzymes. Upper endoscopy was done in the context of evaluation of anemia, which revealed reduced duodenal folds and mosaic pattern of the mucosa, but also grade II esophageal varices and portal hypertensive gastropathy...
June 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28703133/association-of-autoimmune-hepatitis-with-src-homology-2-adaptor-protein-3-gene-polymorphisms-in-japanese-patients
#5
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
#6
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/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#7
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28698093/bile-acids-and-intestinal-microbiota-in-autoimmune-cholestatic-liver-diseases
#8
REVIEW
You Li, Ruqi Tang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
Cholestatic liver diseases, including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), are manifested as an impairment of normal bile flow and excessive accumulation of potentially toxic bile acids. Endogenous bile acids are involved in the pathogenesis and progression of cholestasis. Consequently, chronic cholestasis affects the expression of bile acids transporters and nuclear receptors, and results in liver injury. Several lines of evidence suggest that intestinal microbiota plays an important role in the etiopathogenesis of cholestatic liver diseases by regulating metabolism and immune responses...
July 8, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28691216/statins-and-ace-inhibitors-are-associated-with-reduced-mortality-and-morbidity-in-chronic-liver-disease
#9
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
#10
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/28680642/long-lasting-response-to-oral-therapy-in-a-young-male-with-monogenic-diabetes-as-part-of-hnf1b-related-disease
#11
Elena Carrillo, Amparo Lomas, Pedro J Pinés, Cristina Lamas
Mutations in hepatocyte nuclear factor 1β gene (HNF1B) are responsible for a multisystemic syndrome where monogenic diabetes (classically known as MODY 5) and renal anomalies, mostly cysts, are the most characteristic findings. Urogenital malformations, altered liver function tests, hypomagnesemia or hyperuricemia and gout are also part of the syndrome. Diabetes in these patients usually requires early insulinization. We present the case of a young non-obese male patient with a personal history of renal multicystic dysplasia and a debut of diabetes during adolescence with simple hyperglycemia, negative pancreatic autoimmunity and detectable C-peptide levels...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28675685/challenges-and-difficulties-in-pathological-diagnosis-of-autoimmune-hepatitis
#12
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/28672215/silymarin-impacts-on-immune-system-as-an-immunomodulator-one-key-for-many-locks
#13
REVIEW
Nafiseh Esmaeil, Sima Balouchi Anaraki, Marjan Gharagozloo, Behjat Moayedi
Silymarin is a flavonoid complex extracted from the Silybum marianum plant. It acts as a strong antioxidant and free radical scavenger by different mechanisms. But in addition to antioxidant effects, silymarin/silybin reveals immunomodulatory affects with both immunostimulatory and immunosuppression activities. Different studies have shown that silymarin has the anti-inflammatory effect through the suppression of NF-κB signaling pathway and TNF-α activation. It also has different immunomodulatory activities in a dose and time-dependent manner...
June 30, 2017: International Immunopharmacology
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/28665300/transplant-trials-with-tregs-perils-and-promises
#15
Qizhi Tang, Flavio Vincenti
Modern immunosuppression regimens effectively control acute rejection and decrease graft loss in the first year after transplantation; however, these regimens do not have a durable effect on long-term graft survival owing to a combination of drug toxicities and the emergence of chronic alloimmune responses. Eliminating drugs and their toxicities while maintaining graft acceptance has been the primary aim of cellular therapies. Tregs suppress both autoimmune and alloimmune responses and are particularly effective in protecting allografts in experimental transplant models...
June 30, 2017: Journal of Clinical Investigation
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
#16
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/28661429/effective-suckling-c57bl-6-kunming-and-balb-c-mouse-models-with-remarkable-neurological-manifestation-for-zika-virus-infection
#17
Jianhai Yu, Xuling Liu, Changwen Ke, Qinghua Wu, Weizhi Lu, Zhiran Qin, Xiaoen He, Yujing Liu, Jieli Deng, Suiqi Xu, Ying Li, Li Zhu, Chengsong Wan, Qiwei Zhang, Weiwei Xiao, Qian Xie, Bao Zhang, Wei Zhao
Since 2015, 84 countries and territories reported evidence of vector-borne Zika Virus (ZIKV) transmission. The World Health Organization (WHO) declared that ZIKV and associated consequences especially the neurological autoimmune disorder Guillain-Barré syndrome (GBS) and microcephaly will remain a significant enduring public health challenge requiring intense action. We apply a standardization of the multi-subcutaneous dorsal inoculation method to systematically summarize clinical neurological manifestation, viral distribution, and tissue damage during the progress of viremia and systemic spread in suckling mouse models...
June 29, 2017: Viruses
https://www.readbyqxmd.com/read/28660010/liver-immunology-and-herbal-treatment
#18
REVIEW
Yasemin H Balaban, Ceylan Aka, Ufuk Koca-Caliskan
Beyond the metabolic functions, the liver recently has been defined as an organ of immune system (IS), which have central regulatory role for innate and adaptive immunity. The liver keeps a delicate balance between hepatic screening of pathogenic antigens and immune tolerance to self-antigens. Herbal treatments with immunological effects have potential to alter this hepatic immune balance towards either therapeutic side or diseases side by inducing liver injury via hepatotoxicity or initiation of autoimmune diseases...
June 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28651900/potential-clinical-applications-of-placental-stem-cells-for-use-in-fetal-therapy-of-birth-defects
#19
Melissa Vanover, Aijun Wang, Diana Farmer
Placental stem cells are of growing interest for a variety of clinical applications due to their multipotency and ready availability from otherwise frequently discarded biomaterial. Stem cells derived from the placenta have been investigated in a number of disease processes, including wound healing, ischemic heart disease, autoimmune disorders, and chronic lung or liver injury. Fetal intervention for structural congenital defects, such as spina bifida, has rapidly progressed as a field due to advances in maternal-fetal medicine and improving surgical techniques...
May 18, 2017: Placenta
https://www.readbyqxmd.com/read/28649247/an-antigen-presenting-and-apoptosis-inducing-polymer-microparticle-prolongs-alloskin-graft-survival-by-selectively-and-markedly-depleting-alloreactive-cd8-t-cells
#20
Wei Wang, Khawar Ali Shahzad, Miaochen Li, Aifeng Zhang, Lei Zhang, Tao Xu, Xin Wan, Chuanlai Shen
Selectively depleting the pathogenic T cells is a fundamental strategy for the treatment of allograft rejection and autoimmune disease since it retains the overall immune function of host. The concept of killer artificial antigen-presenting cells (KaAPCs) has been developed by co-coupling peptide-major histocompatibility complex (pMHC) multimer and anti-Fas monoclonal antibody (mAb) onto the polymeric microparticles (MPs) to induce the apoptosis of antigen-specific T cells. But little information is available about its in vivo therapeutic potential and mechanism...
2017: Frontiers in Immunology
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