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https://www.readbyqxmd.com/read/29352427/preserved-in-vitro-immunoreactivity-in-children-receiving-long-term-immunosuppressive-therapy-due-to-inflammatory-bowel-disease-or-autoimmune-hepatitis
#1
Teresa Schleker, Eva-Maria Jacobsen, Benjamin Mayer, Gudrun Strauss, Klaus-Michael Debatin, Carsten Posovszky
BACKGROUND: Children with inflammatory bowel disease (IBD) or autoimmune hepatitis (AIH) are at risk for severe infections. This is partially a result of their chronic disease condition but, moreover, a side effect of their immunosuppressive therapy. Currently, vaccinations with live vaccines are regarded as contraindicated under immunosuppressive therapy, mainly because of concerns about side effects and a lack of data showing an adequate immune reaction. As there is no systematic study on the individual immunoreactivity under immunosuppressive therapy in this patient group, we analyzed the lymphocyte subgroups and immunoreactivity of lymphocytes in children with IBD or AIH with and without immunosuppressive therapy in vitro...
January 19, 2018: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/29348111/leveraging-social-networking-sites-for-an-autoimmune-hepatitis-genetic-repository-pilot-study-to-evaluate-feasibility
#2
Megan Comerford, Rachel Fogel, James Robert Bailey, Prianka Chilukuri, Naga Chalasani, Craig Steven Lammert
BACKGROUND: Conventional approaches to participant recruitment are often inadequate in rare disease investigation. Social networking sites such as Facebook may provide a vehicle to circumvent common research limitations and pitfalls. We report our preliminary experience with Facebook-based methodology for participant recruitment and participation into an ongoing study of autoimmune hepatitis (AIH). OBJECTIVE: The goal of our research was to conduct a pilot study to assess whether a Facebook-based methodology is capable of recruiting geographically widespread participants into AIH patient-oriented research and obtaining quality phenotypic data...
January 18, 2018: Journal of Medical Internet Research
https://www.readbyqxmd.com/read/29333079/type-1-autoimmune-hepatitis-presenting-with-severe-autoimmune-neutropenia
#3
Petros Doumtsis, Theodora Oikonomou, Ioannis Goulis, Kaliopi Zachou, George Dalekos, Evangelos Cholongitas
Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders...
January 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29327413/itpa-activity-in-children-treated-by-azathioprine-relationship-to-the-occurrence-of-adverse-drug-reactions-and-inflammatory-response
#4
Antony Citterio-Quentin, Mustapha Moulsma, Marie-Paule Gustin, Alain Lachaux, Roselyne Boulieu
Azathioprine (AZA), a thiopurine drug, is widely used in the treatment of children with immunological disease such as inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH); however, interindividual variability in the occurrence of adverse drug reaction (ADRs) and drug response is observed. This study investigated 1) the relationships between inosine triphosphate pyrophosphatase (ITPA) activity, an enzyme involved in thiopurine metabolism, and the occurrence of ADRs in children with immunological disease on AZA therapy, 2) the relationship between ITPA activity and the inflammatory activity observed in children with IBD...
January 11, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29325302/-research-advances-in-pathogenesis-of-autoimmune-hepatitis
#5
P P Zhao, Z H Deng
Autoimmune hepatitis (AIH) is liver parenchymal inflammation mediated by the autoimmune response of hepatocytes. Its clinical features include elevation of aminotransferases in serum, presence of various autoantibodies in circulation, hypergammaglobulinemia, and interface hepatitis in liver tissue. Although the etiology and pathogenesis of AIH have not been fully elucidated, a consensus has been reached on the theories of "genetic susceptibility" and "molecular simulation" , and "immunoregulatory disorder" remains a hot research topic for many scholars...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29325280/-clinical-value-of-anti-liver-kidney-microsomal-1-antibody-in-patients-with-liver-disease
#6
Y Han, H P Yan, H Y Liao, L M Sun, Y L Huang, C Y Huang, H P Zhang, X D Zhang, X Q Bian, M X Ren, X F Du, Y M Liu
Objective: To investigate the clinical and laboratory features of patients with liver disease and positive anti-liver/kidney microsomal-1 (anti-LKM-1) antibody, and to provide a reference for clinical diagnosis and differential diagnosis. Methods: The clinical data of patients with positive anti-LKM-1 antibody who were treated in our hospital from 2006 to 2016 were collected, and clinical and laboratory features were analyzed and compared. An analysis was also performed for special cases. Results: The measurement of related autoantibodies was performed for about 100 thousand case-times, and 15 patients were found to have positive anti-LKM-1 antibody...
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29325273/-how-to-understand-the-clinical-significance-of-autoantibodies-in-primary-biliary-cholangitis
#7
H P Yan, H P Zhang, X X Chen
Autoantibodies are important indicators for the diagnosis of primary biliary cholangitis (PBC). The autoantibodies in PBC patients are mainly antimitochondrial antibodies (AMAs) and antinuclear antibodies (ANAs). AMAs are one of the diagnostic indices of PBC. PBC-specific ANAs (nuclear dots or nuclear envelope, anti-sp100, and anti-gp210) have a high specificity in the diagnosis of AMA-negative PBC. This article reviews the clinical significance of these autoantibodies and analyzes some misconceptions about the clinical diagnosis of AMA-negative PBC and PBC-AIH overlap syndrome...
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29323192/baseline-il-2-and-the-aih-score-can-predict-the-response-to-standard-therapy-in-paediatric-autoimmune-hepatitis
#8
Jana Diestelhorst, Norman Junge, Danny Jonigk, Jerome Schlue, Christine S Falk, Michael P Manns, Ulrich Baumann, Elmar Jaeckel, Richard Taubert
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. So far accepted predictors of the subsequent treatment response of AIH patients are lacking. Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). In contrast to adults, elevated serum markers indicating iron overload were not commonly found in children. Therefore, ferritin was not predictive of the treatment response in pAIH...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29321432/acute-onset-autoimmune-hepatitis-in-a-young-woman-with-type-1-diabetes-mellitus
#9
Satoshi Takai, Jun Inoue, Takayuki Kogure, Eiji Kakazu, Masashi Ninomiya, Tomoaki Iwata, Teruyuki Umetsu, Takuya Nakamura, Akitoshi Sano, Tooru Shimosegawa
Autoimmune hepatitis (AIH) and type 1 diabetes mellitus (T1DM) are thought to be induced by autoimmunity, but their coexistence has rarely been reported. We herein report a case in which a patient with T1DM developed acute-onset AIH. A 26-year-old woman, who had been diagnosed with T1DM in childhood, was transferred to our hospital because of acute liver failure of unknown etiology. The administration of corticosteroids including steroid pulse therapy was effective. Based on the histological finding of massive centrilobular necrosis and a good response to steroid therapy, we diagnosed the patient with acute-onset AIH...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29288086/autoimmune-hepatitis-association-with-single-nucleotide-polymorphism-of-interleukin-2-but-not-interferon-gamma
#10
Azizollah Yousefi, Elham Mahmoudi, Behnoud Baradaran Noveiry, Alireza Zare Bidoki, Maryam Sadr, Farzaneh Motamed, Mehri Najafi, Fatemeh Farahmand, Ahmad Khodadad, Gholam Hossein Fallahi, Nima Rezaei
BACKGROUND: Autoimmune hepatitis (AIH) is a chronic inflammation in hepatocellular tissues associated with circulating autoantibodies. Imbalance in T-cells population and dysregulation in several cytokine profiles has been implicated in pathogenesis of AIH. This study was performed to assess potential association of AIH with interleukin-2 (IL-2) and interferon-gamma (IFN-γ) genes single nucleotide polymorphisms (SNPs). METHODS: Fifty-six patients with AIH and 139 healthy individuals were enrolled in this study...
December 26, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29283990/the-effects-of-berberine-on-concanavalin-a-induced-autoimmune-hepatitis-aih-in-mice-and-the-adenosine-5-monophosphate-amp-activated-protein-kinase-ampk-pathway
#11
Yangyang Wang, Lu Zhou, Yanni Li, Liping Guo, Zhe Zhou, Haoran Xie, Yingjian Hou, Bangmao Wang
BACKGROUND Berberine, a herbal extract, has been reported to protect against inflammatory disorders. The adenosine 5'-monophosphate (AMP)-activated protein kinase (AMPK) signaling pathway can be activated by berberine and inhibited by the synthetic, reversible AMP-competitive inhibitor, Compound C. The aim of this study was to investigate the effects of berberine on concanavalin A (Con A)-induced autoimmune hepatitis (AIH) in mice via the AMPK pathway. MATERIAL AND METHODS BALB/c mice were treated with berberine, with or without Compound C, followed by treatment with Con A...
December 28, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29279846/autoantibodies-against-cyp-2c19-a-novel-serum-marker-in-pediatric-de-novo-autoimmune-hepatitis
#12
Maria Grazia Clemente, Roberto Antonucci, Claudia Mandato, Lucia Cicotto, Antonella Meloni, Bruno Gridelli, Stefano De Virgiliis, Michael P Manns, Pietro Vajro
Diagnosis of de novo autoimmune hepatitis (AIH) after orthotopic liver transplantation (OLT) is challenging especially in the absence of hyper-γ-globulinemia. Circulating autoantibodies are not sensitive nor specific in de novo AIH but when positive increase the diagnostic probability. We report the discovery of novel liver microsomal (LM) autoantibodies against CYP-2C19 in a 9-year-old boy with "de novo" AIH developed 7 years after OLT. Graft dysfunction presented with hypertransaminasemia (up to 400 IU/L), while serum γ-globulins remained within the normal range for age...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29233784/altered-natural-killer-cell-cytokine-profile-in-type-2-autoimmune-hepatitis
#13
Dalila Mele, Grazia Bossi, Giuseppe Maggiore, Barbara Oliviero, Stefania Mantovani, Beatrice Bonelli, Mario U Mondelli, Stefania Varchetta
Type 2 autoimmune hepatitis (AIH-2) is a rare disease presenting in early childhood. The immunopathogenetic mechanisms are poorly characterized, although a defect of regulatory T cells (Treg) has been shown. There is virtually no information on innate immune responses and natural killer (NK) cells in particular. We have performed an extended immunophenotypic and functional analysis of NK cells in children with AIH-2. We show that NK cell frequency is reduced in this setting and that the balance between NK activating and inhibitory receptors is skewed toward activation...
December 9, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29233741/cyclooxygenase-enzyme-activity-does-not-impair-respiratory-motor-plasticity-after-one-night-of-intermittent-hypoxia
#14
Adrianne G Huxtable, Elizabeth Kopp, Brendan J Doughtery, Jyoti J Watters, Gordon S Mitchell
Although inflammation is prevalent in many clinical disorders challenging breathing, we are only beginning to understand the impact of inflammation on neural mechanisms of respiratory control. We recently demonstrated one form of respiratory motor plasticity is extremely sensitive to even mild inflammation induced by a single night (8 h) of intermittent hypoxia (IH-1), mimicking aspects of obstructive sleep apnea. Specifically, phrenic long-term facilitation (pLTF) following moderate acute intermittent hypoxia (AIH) is abolished by IH-1, but restored by high doses of the non-steroidal anti-inflammatory drug, ketoprofen...
December 9, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/29227329/a-systematic-review-and-meta-analysis-of-second-line-immunosuppressants-for-autoimmune-hepatitis-treatment
#15
Michele De Lemos-Bonotto, Cristiane Valle-Tovo, Ane M Costabeber, Angelo A Mattos, André L F Azeredo-da-Silva
INTRODUCTION: The gold-standard treatment for autoimmune hepatitis (AIH) is a prednisone/azathioprine combination. However, subgroups of patients may be unresponsive to this treatment. The aim of this study is to evaluate the efficacy of second-line immunosuppressive therapies for AIH through a systematic review and meta-analysis in adult patients. PATIENTS AND METHODS: The systematic review was registered at the PROSPERO platform under number 42015019831. Databases MEDLINE (PubMed), Lilacs, Cochrane, and Scielo were searched...
February 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29215462/liver-transplantation-in-cryptogenic-cirrhosis-outcome-comparisons-between-nash-alcoholic-and-aih-cirrhosis
#16
Paul Joseph Thuluvath, Steven Hanish, Yulia Savva
BACKGROUND: The outcomes of liver transplantation (LT) in patients with cryptogenic cirrhosis (CC) have not been adequately examined except for small case series. We believe that patients currently listed as CC have truly cryptogenic liver disease and may have different post-LT outcomes compared to nonalcoholic steatohepatitis (NASH). METHODS: We compared the post-LT outcomes of adults with CC (n=3241) and compared them with cirrhosis from NASH (n=4089), alcohol (AC, n=7837) and autoimmune hepatitis (AIH, n=1435) using the UNOS database from 2002-16...
December 5, 2017: Transplantation
https://www.readbyqxmd.com/read/29212793/pacap-6-38-or-kynurenate-microinjections-into-the-rvlm-prevent-development-of-sympathetic-long-term-facilitation-following-acute-intermittent-hypoxia
#17
Zohra M Kakall, Paul M Pilowsky, Melissa Mj Farnham
BACKGROUND AND PURPOSE: Intermittent hypoxia causes a persistent increase in sympathetic activity, which progresses to hypertension in chronic conditions such as obstructive sleep apnea (OSA). Pituitary adenylate cyclase activating polypeptide (PACAP) is an excitatory neurotransmitter that causes long-lasting sympathetic excitation. We aimed to determine if intermittent activation of the rostral ventrolateral medulla (RVLM) causes PACAP-mediated elevation of sympathetic nerve activity (sSNA), termed sympathetic long-term facilitation (sLTF)...
December 6, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29207801/autoimmune-haemolytic-anaemia-a-spectrum-of-presentation-in-children
#18
Amitabh Singh, Anirban Mandal, Ankita Patel, Sataroopa Mishra
Autoimmune Haemolytic Anaemias (AIHAs) are rare in children. They can be either a primary disease or secondary to/triggered by a host of other clinical conditions. We present five interesting cases of paediatric AIHA associated with infections (viral, bacterial and atypical) and autoimmune diseases {Systemic Lupus Erythematosus (SLE) and Autoimmune Hepatitis (AIH)}. The H1N1 influenza associated AIHA responded to oseltamivir and Intravenous Immunoglobulin (IvIg) while the cases secondary to Mycoplasma pneumoniae and pneumococcal bacteraemia required only treatment of the primary infection...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29180000/usefulness-of-biochemical-remission-and-transient-elastography-in-monitoring-disease-course-in-autoimmune-hepatitis
#19
Johannes Hartl, Hanno Ehlken, Marcial Sebode, Moritz Peiseler, Till Krechl, Roman Zenouzi, Johann von Felden, Christina Weiler-Normann, Christoph Schramm, Ansgar W Lohse
BACKGROUND: Liver fibrosis regression but also progression may occur in patients with autoimmune hepatitis (AIH) under treatment. There is a need for non-invasive surrogate markers for fibrosis development in AIH to better guide immunosuppressive treatment. AIMS: The aims of the study were to assess the impact of complete biochemical remission defined as normalisation of transaminases and IgG on histological activity and fibrosis development, and the value of repeat transient elastography (TE) measurement for monitoring disease progression in AIH...
November 24, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29162389/is-cryptogenic-cirrhosis-different-from-nash-cirrhosis
#20
Paul J Thuluvath, Sergey Kantsevoy, Avesh J Thuluvath, Yulia Savva
BACKGROUND & AIMS: We hypothesized that patients currently diagnosed as CC have truly 'cryptogenic' liver disease, and unlikely to have evolved from NASH. METHODS: To investigate this, we compared the clinical characteristics of adults with CC (n=7,999) with NASH (n=11,302), alcohol (AC, n=21,714) and autoimmune hepatitis (AIH, n=3,447) using the UNOS database from 2002-16. We performed an age, gender and year of listing matched comparison of CC and NASH (n= 7,201 in each group), and also stratified by the presence of obesity or diabetes mellitus (DM)...
November 18, 2017: Journal of Hepatology
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