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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/29351806/isolated-septic-arthritis-of-hip-joint-a-rare-presentation-of-melioidosis-a-case-report
#2
N P Weerasinghe, H M M Herath, T M U Liyanage
BACKGROUND: Despite, Sri Lanka lies in the melioidosis endemic belt between 5°N and 10°N surrounded by countries known to have endemic melioidosis for many years, comparatively fewer cases of melioidosis infection have been reported in Sri Lanka. Melioidosis has a wide spectrum of clinical presentation, ranging from severe pneumonia to abscess formation in various organs. Isolated septic arthritis, which is a rare but well-recognized manifestation of melioidosis, could be the sole presenting problem in some patients with melioidosis...
January 19, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29349620/fatal-talaromyces-marneffei-infection-in-a-patient-with-autoimmune-hepatitis
#3
Sally C Y Wong, Siddharth Sridhar, Antonio H Y Ngan, Jonathan H K Chen, Rosana W S Poon, Susanna K P Lau, Patrick C Y Woo
Talaromyces marneffei, previously known as Penicillium marneffei, is the most important pathogenic thermally dimorphic fungus causing systemic mycosis in Southeast Asia. Traditionally, T. marneffei infection in human was mainly associated with acquired immunodeficiency syndrome caused by HIV infection. In recent years, there has been an increasing number of T. marneffei infections reported in non-HIV-infected patients with other immunocompromised conditions, including autoantibodies against interferon-gamma, systemic lupus erythematosis, solid organ transplantation, Job's syndrome, hematological malignancies, and use of novel targeted therapies...
January 18, 2018: Mycopathologia
https://www.readbyqxmd.com/read/29348111/leveraging-social-networking-sites-for-an-autoimmune-hepatitis-genetic-repository-pilot-study-to-evaluate-feasibility
#4
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/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#5
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29339102/spirulina-ameliorates-methotrexate-hepatotoxicity-via-antioxidant-immune-stimulation-and-proinflammatory-cytokines-and-apoptotic-proteins-modulation
#6
Asmaa F Khafaga, Yasser S El-Sayed
AIMS: Methotrexate (MTX) is an efficient cytotoxic drug used against various carcinogenic, inflammatory and autoimmune diseases; however, the hepatotoxicity of MTX limits its use. Therefore, the present study aimed to evaluate the potential hepatoprotective and immune-stimulant effect of Spirulina platensis (SP) against MTX acute toxicity. MAIN METHODS: Thirty-two male Wistar rats were randomly allocated into the following four groups (n = 8): control, MTX (20 mg/kg bwt, single ip injection), SP (500 mg/kg bwt, oral gavage daily for 21 days), and MTX+SP...
January 12, 2018: Life Sciences
https://www.readbyqxmd.com/read/29336235/rapid-remission-of-graves-hyperthyroidism-without-thionamides-under-immunosuppressive-treatment-for-concomitant-autoimmune-hepatitis
#7
Georgios E Papadakis, Faiza Lamine, Haithem Chtioui, Eleni Moschouri, Monserrat Fraga Christinet, Laura Marino, Lucie Favre, Amedeo Sciarra, Christine Sempoux, Alexandra Schneider, Francois-Regis Duss, Claudio Sartori, Darius Moradpour, Gerasimos P Sykiotis
No abstract needed for a Letter to the Editor according to the journal's guidelines for Authors.
January 16, 2018: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/29333079/type-1-autoimmune-hepatitis-presenting-with-severe-autoimmune-neutropenia
#8
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/29332510/localized-autoimmune-pancreatitis-mimicking-pancreatic-cancer-case-report-and-literature-review
#9
Wen-Ling Hsu, Shu-Min Chang, Pei-Yin Wu, Chin-Chuan Chang
Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29331407/decreased-health-related-quality-of-life-in-children-and-adolescents-with-autoimmune-hepatitis
#10
Ana Beatriz Bozzini, Luciana Neder, Clovis A Silva, Gilda Porta
OBJECTIVE: The objective of this study was to evaluate the health-related quality of life in children and adolescents with autoimmune hepatitis. METHODS: A cross-sectional assessment with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was completed for 80 patients with autoimmune hepatitis and 45 healthy controls. Demographic data, prednisone dose, disease remission state, disease severity, and abdominal pain were also evaluated. RESULTS: Based on the child self-reports, physical, emotional, school, and total scores were significantly lower in autoimmune hepatitis patients when compared with controls (p<0...
January 10, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29328499/mait-cells-are-chronically-activated-in-patients-with-autoimmune-liver-disease-and-promote-pro-fibrogenic-hepatic-stellate-cell-activation
#11
Katrin Böttcher, Krista Rombouts, Francesca Saffioti, Davide Roccarina, Matteo Rosselli, Andrew Hall, TuVinh Luong, Emmanuel A Tsochatzis, Douglas Thorburn, Massimo Pinzani
Autoimmune liver diseases (AILD) are chronic liver pathologies characterised by fibrosis and cirrhosis due to immune-mediated liver damage. In this study, we addressed the question whether mucosal-associated invariant T (MAIT) cells, innate-like T cells, are functionally altered in patients with AILD and whether MAIT cells can promote liver fibrosis through activation of hepatic stellate cells. We analysed the phenotype and function of MAIT cells from AILD patients and healthy controls by multi-colour flow cytometry and investigated the interaction between human MAIT cells and primary human hepatic stellate cells (hHSCs)...
January 12, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29327413/itpa-activity-in-children-treated-by-azathioprine-relationship-to-the-occurrence-of-adverse-drug-reactions-and-inflammatory-response
#12
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/29325864/infections-genetic-and-environmental-factors-in-pathogenesis-of-autoimmune-thyroid-diseases
#13
REVIEW
Sanjeev Kumar Shukla, Govind Singh, Shahzad Ahmad, Prabhat Pant
In Autoimmune disease a combination of infection, genetic and environmental factors causes an autoimmune response to the thyroid gland (characterized by lymphocytic infiltrations), thyroid stimulating hormone receptor (TSHR) and different thyroid antigens. Graves' and Hashimoto disease are autoimmune disorders with genetic predisposition. CD40 that stimulates the proliferation and differentiation of lymphocytes is an essential immunomodulatory component for follicular cells in the thyroid and the cell that present the antigen...
January 8, 2018: Microbial Pathogenesis
https://www.readbyqxmd.com/read/29325302/-research-advances-in-pathogenesis-of-autoimmune-hepatitis
#14
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
#15
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/29325274/-an-interpretation-of-2017-easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#16
Y Han, Y Chen
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic disease and may progress to liver fibrosis, liver cirrhosis, decompensated cirrhosis, and even end-stage liver disease without effective treatment. The diagnosis of PBC is mainly based on the biochemical parameters indicating cholestatic hepatitis and the presence of specific autoantibody in circulation. The goals of the treatment and management of PBC are to prevent the development of end-stage liver disease, to improve related clinical symptoms, and to improve patients' quality of life...
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29324588/both-%C3%AE-1-antitrypsin-z-phenotypes-and-low-caeruloplasmin-levels-are-over-represented-in-alcohol-and-nonalcoholic-fatty-liver-disease-cirrhotic-patients-undergoing-liver-transplant-in-ireland
#17
El-Gaily A El-Rayah, Patrick J Twomey, Eleanor M Wallace, Peter A McCormick
OBJECTIVES: Alcoholic liver disease and nonalcoholic fatty liver disease (NAFLD) are steatotic liver diseases and major causes of cirrhosis. Only a minority of patients with risk factors develop cirrhosis and genetic cofactors may be important in pathogenesis. Mutations in the Wilson's and α-1-antitrypsin genes are not uncommon and we speculated that they may act as cofactors. METHODS: We investigated α-1-antitrypsin phenotyes and caeruloplasmin levels in patients undergoing elective liver transplantation...
January 10, 2018: European Journal of Gastroenterology & 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
#18
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
#19
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/29318378/mac-2-binding-protein-glycan-isomer-m2bpgi-is-a-new-serum-biomarker-for-assessing-liver-fibrosis-more-than-a-biomarker-of-liver-fibrosis
#20
REVIEW
Ken Shirabe, Yuki Bekki, Dolgormaa Gantumur, Kenichiro Araki, Norihiro Ishii, Atsushi Kuno, Hisashi Narimatsu, Masashi Mizokami
Assessing liver fibrosis is important for predicting the efficacy of antiviral therapy and patient prognosis. Liver biopsy is the gold standard for diagnosing liver fibrosis, despite its invasiveness and problematic diagnostic accuracy. Although noninvasive techniques to assess liver fibrosis are becoming important, reliable serum surrogate markers are not available. A glycoproteomics study aimed at identifying such markers discovered Mac 2-Binding Protein Gylcan Isomer (M2BPGi), which is a reliable marker for assessing liver fibrosis in patients with viral hepatitis and other fibrotic liver diseases such as primary biliary cholangitis, biliary atresia, autoimmune hepatitis, and nonalcoholic fatty liver disease...
January 9, 2018: Journal of Gastroenterology
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