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https://www.readbyqxmd.com/read/27906472/long-term-clinical-impact-and-cost-effectiveness-of-obeticholic-acid-for-the-treatment-of-primary-biliary-cholangitis
#1
Sumeyye Samur, Matthew Klebanoff, Reiner Banken, Daniel S Pratt, Rick Chapman, Daniel A Ollendorf, Anne M Loos, Kathleen Corey, Chin Hur, Jagpreet Chhatwal
: Primary biliary cholangitis (PBC) is a chronic, progressive autoimmune liver disease that mainly affects middle-aged women. Obeticholic acid (OCA), which was recently approved by the Food and Drug Administration for PBC treatment, has demonstrated positive effects on biochemical markers of liver function. Our objective was to evaluate the long-term clinical impact and cost-effectiveness of OCA as a second-line treatment for PBC in combination with ursodeoxycholic acid (UDCA) in adults with an inadequate response to UDCA...
November 7, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27896807/fdg-pet-ct-imaging-of-therapeutic-response-in-granulomatous-lymphocytic-interstitial-lung-disease-glild-in-common-variable-immunodeficiency-cvid
#2
S Jolles, E Carne, M Brouns, T El-Shanawany, P Williams, C Marshall, P Fielding
Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ...
November 28, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27891589/critical-roles-of-conventional-dendritic-cells-in-promoting-t-cell-dependent-hepatitis-through-regulating-nkt-cells
#3
Jingya Wang, Xueting Cao, Jiliang Zhao, Huiyuan Zhao, Jun Wei, Qiuying Li, Xiaofei Qi, Zishan Yang, Li Wang, Hongru Zhang, Li Bai, Zhenzhou Wu, Liqing Zhao, Zhangyong Hong, Zhinan Yin
Dendritic cells (DC) play critical roles in initiating and regulating the innate immunity as well as the adaptive immune responses. However, the role of conventional dendritic cells (cDC) in Con A-induced fulminant hepatitis is unknown. In this study, we demonstrated that depletion of cDC using either CD11c-DTR Tg mice or anti-CD11c antibody significantly reduced the severity of liver injury, indicating a detrimental role of cDC in Con A-induced hepatitis. We further elucidated the pathological role of cDC through being the critical source of IL-12, which induced the secretion of IFN-γ by NKT cells...
November 28, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27890169/hepatitis-c-virus-infection-and-rheumatic-diseases-the-impact-of-direct-acting-antiviral-agents
#4
REVIEW
Patrice Cacoub, Cloé Commarmond, David Sadoun, Anne Claire Desbois
Chronic hepatitis C virus (HCV) infection is associated with liver and extrahepatic complications, including B-cell lymphoma, cardiovascular and kidney diseases, glucose metabolism impairment and rheumatic conditions ie, arthralgia, myalgia, cryoglobulinemia vasculitis, sicca syndrome and the production of autoantibodies. The treatment has long been based on interferon alpha (IFN) that was found poorly effective, and contraindicated in many autoimmune/inflammatory disorders because of possible exacerbation of rheumatic disorders...
February 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/27883375/analysis-of-risk-factors-for-severe-acute-respiratory-infection-and-pneumonia-and-among-adult-patients-with-acute-respiratory-illness-during-2011-2014-influenza-seasons-in-korea
#5
Seong Hui Kang, Hee Jin Cheong, Joon Young Song, Ji Yun Noh, Ji Ho Jeon, Min Joo Choi, Jacob Lee, Yu Bin Seo, Jin Soo Lee, Seong Heon Wie, Hye Won Jeong, Young Keun Kim, Kyung Hwa Park, Shin Woo Kim, Eun Joo Jeong, Sun Hee Lee, Won Suk Choi, Woo Joo Kim
BACKGROUND: The World Health Organization recommends the surveillance of influenza-like illness (ILI) and severe acute respiratory infection (SARI) to respond effectively to both seasonal influenza epidemics and pandemics. In Korea, the "Hospital-based Influenza Morbidity and Mortality (HIMM)" surveillance system has been operated to monitor ILI and SARI occurrences. MATERIALS AND METHODS: A multi-center prospective observational study was conducted. Adult patients with acute respiratory infection (ARI) were enrolled during the 2011-2012, 2012-2013, and 2013-2014 influenza seasons at the 10 university hospitals using the HIMM surveillance system...
November 22, 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#6
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27871114/bone-marrow-stromal-stem-cells-in-tissue-engineering-and-regenerative-medicine
#7
A Polymeri, W V Giannobile, D Kaigler
Bone marrow stromal stem cells (BMSCs) are adult multipotent cells, which have the potential to differentiate into cell types of mesodermal origin, namely osteocytes, adipocytes, and chondrocytes. Due to their accessibility and expansion potential, BMSCs have historically held therapeutic promise in tissue engineering and regenerative medicine applications. More recently, it has been demonstrated that not only can bone marrow stromal stem cells directly participate in tissue regeneration, but they also have the capacity to migrate to distant sites of tissue injury, where they can participate in tissue repair either directly through their differentiation or indirectly through paracrine mechanisms...
November 2016: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#8
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic Microangiopathy is a broad pathophysiological process that leads to microangiopathic hemolytic anemia, thrombocytopenia and involves capillary and small vessel platelet aggregates. The most common cause being disseminated intravascular coagulation (DIC), which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT), including cancer, infection, transplantation, drugs, autoimmune disease and pre-eclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome in pregnancy...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27860118/association-between-serum-soluble-cd14-and-il-8-levels-and-clinical-outcome-in-primary-biliary-cholangitis
#9
Takeji Umemura, Tomohiro Sekiguchi, Satoru Joshita, Tomoo Yamazaki, Naoyuki Fujimori, Soichiro Shibata, Yuki Ichikawa, Michiharu Komatsu, Akihiro Matsumoto, Zakera Shums, Gary L Norman, Eiji Tanaka, Masao Ota
BACKGROUND & AIMS: Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by portal inflammation and immune-mediated destruction of intrahepatic bile ducts that often leads to liver decompensation, and liver failure. Although the biochemical response to ursodeoxycholic acid (UDCA) can predict disease outcome in PBC, few biomarkers have been identified as prognostic tools applicable prior to UDCA treatment. We therefore sought to identify such indicators of long-term outcome in PBC in the Japanese population...
November 17, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27855457/-cholestatic-liver-disease
#10
Christine Pichler, Tobias Boettler, Robert Thimme
Common cholestatic liver diseases are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both diseases are considered autoimmune disorders; however, the precise pathogenesis remains elusive. Patients usually show no symptoms or present with pruritus and fatigue. High alkaline phosphatase and the presence of antimitochondrial antibodies are sufficient to diagnose PBC. Ursodeoxycholic acid is approved and recommended for the treatment of PBC. The diagnosis of PSC is established by elevated alkaline phosphatase-levels and typical cholangiographic findings...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27852547/prevalence-of-igg-4-associated-cholangiopathy-based-on-serum-igg-4-levels-in-patients-with-primary-sclerosing-cholangitis-and-its-relationship-with-inflammatory-bowel-disease
#11
Seyed Alireza Taghavi, Shaghayegh Kaedi Majd, Mahnoosh Sianati, Masood Sepehrimanesh
BACKGROUND/AIMS: Autoimmune cholangiopathy is part of a fibro-inflammatory immunoglobulin G-4 (IgG-4)-related systemic disease that causes biliary tract strictures. Its clinical presentation is quite similar to that of more common diseases such as primary sclerosing cholangitis (PSC) and pancreatobilliary malignancies. The aims of the present study were to evaluate the prevalence of IgG-4-associated cholangiopathy (IAC) in patients diagnosed with PSC and its relationship with inflammatory bowel disease (IBD)...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27848888/immunotherapy-in-liver-diseases-a-balance-between-immunity-and-tolerance
#12
Alaknanda Mishra, Pramod K Upadhyay, Perumal Nagarajan
In today's context when liver diseases have spread across countries and people of all ages, it is of high importance to consider novel methods of non-toxic and long lived therapeutics. Among various therapies, immunotherapy for acute and chronic liver diseases is rapidly moving to the forefront among treatment options in hepatology medicine. Liver has a unique immuno- biological advantage which is utilized to maintain a balance between immunity and tolerance. This intricate balance of hepatic immune cells can be modulated to effect treatments in various liver diseases...
November 16, 2016: Current Drug Metabolism
https://www.readbyqxmd.com/read/27848226/atypical-hemolytic-uremic-syndrome-in-the-setting-of-complement-amplifying-conditions-case-reports-and-a-review-of-the-evidence-for-treatment-with-eculizumab
#13
REVIEW
Arif Asif, Ali Nayer, Christian S Haas
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive, life-threatening form of thrombotic microangiopathy (TMA) predominantly caused by dysregulation of the alternative pathway of the complement system. Complement-amplifying conditions (CACs), including pregnancy complications [preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome], malignant hypertension, autoimmune diseases, transplantation, and others, are associated with the onset of TMA in up to 69 % of cases of aHUS...
November 15, 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27846740/prognostic-value-of-clinical-variables-and-liver-histology-for-development-of-fibrosis-and-cirrhosis-in-autoimmune-hepatitis
#14
Lauri Puustinen, Sonja Boyd, Harri Mustonen, Perttu Arkkila, Johanna Arola, Martti Färkkilä
OBJECTIVE: In autoimmune hepatitis, data on the prognostic value of baseline liver biopsy and the sequential histology is controversial. Our aim was to evaluate the prognostic value of clinical variables and biopsy at the time of diagnosis and during the disease course. MATERIALS AND METHODS: All 98 patients in our hospital during 1995-2012 were included. Sequential biopsies were available in 66 patients. Analyses based on clinical and histological variables were performed to find parameters predicting the progression of fibrosis, and development of cirrhosis...
November 16, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27845509/anti-gliadin-antibodies-in-ncgs
#15
Maria Infantino, Francesca Meacci, Valentina Grossi, Donatella Macchia, Mariangela Manfredi
Although no biomarker has been identified to date, previous studies have reported that about 50% of patients with suspected Non Celiac Gluten Sensitivity (NCGS) had positive first generation anti- gliadin antibodies (AGAs), expecially of the IgG class. These antibodies are not specific for NCGS, being also found in CD (80-90%), autoimmune liver disorders (21.5%), connective tissue disease (9%) and IBS (20%), as well as in healthy controls (2-8%), but their finding in patients with a clinical phenotype consistent with NCGS has been regarded as an element supporting this diagnosis...
November 15, 2016: Minerva Gastroenterologica e Dietologica
https://www.readbyqxmd.com/read/27834858/impact-of-microbes-on-the-pathogenesis-of-primary-biliary-cirrhosis-pbc-and-primary-sclerosing-cholangitis-psc
#16
REVIEW
Jochen Mattner
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host...
November 9, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27812306/current-practice-in-laboratory-diagnostics-of-autoimmune-diseases-in-croatia-%C3%A2-survey-of-the-working-group-for-laboratory-diagnostics-of-autoimmune-diseases-of-the-croatian-society-of-medical-biochemistry-and-laboratory-medicine
#17
Andrea Tešija Kuna, Lovorka Đerek, Ana Kozmar, Vedrana Drvar
INTRODUCTION: With the trend of increasing incidence of autoimmune diseases, laboratories are faced with exponential growth of the requests for tests relating the diagnosis of these diseases. Unfortunately, the lack of laboratory personnel experienced in this specific discipline of laboratory diagnostic, as well as an unawareness of a method limitation often results in confusion for clinicians. The aim was to gain insight into number and type of Croatian laboratories that perform humoral diagnostics with the final goal to improve and harmonize laboratory diagnostics of autoimmune diseases in Croatia...
October 15, 2016: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/27807192/suppression-of-irak1-or-irak4-catalytic-activity-but-not-type-1-ifn-signaling-prevents-lupus-nephritis-in-mice-expressing-a-ubiquitin-binding-defective-mutant-of-abin1
#18
Sambit K Nanda, Marta Lopez-Pelaez, J Simon C Arthur, Francesco Marchesi, Philip Cohen
Polymorphisms in the TNIP1 gene encoding A20-binding inhibitor of NF-κB1 (ABIN1) predispose to lupus and other autoimmune diseases in at least eight human populations. We found previously that knock-in mice expressing a ubiquitin-binding-defective mutant of ABIN1 (ABIN1[D485N]) develop autoimmunity as they age and succumb to a disease resembling lupus nephritis in humans. In this article, we report that Flt3-derived dendritic cells from these mice overproduced type 1 IFNs upon stimulation with ligands that activate TLR7 or TLR9...
December 1, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27799976/association-of-macrophage-activating-syndrome-with-castleman-s-syndrome-in-systemic-lupus-erythematosus
#19
Shamsa Shariatpanahi, Shahryar Pourfarzam, Mohammadhosein Gheini
Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion...
2016: Iranian Journal of Pathology
https://www.readbyqxmd.com/read/27796295/betulin-from-hedyotis-hedyotidea-ameliorates-concanavalin-a-induced-and-t-cell-mediated-autoimmune-hepatitis-in-mice
#20
Yong-Qin Zhou, Xiu-Fang Weng, Rui Dou, Xiao-Sheng Tan, Tian-Tian Zhang, Jin-Bo Fang, Xiong-Wen Wu
AIM: Hedyotis hedyotidea has been used in traditional Chinese medicine for the treatment of autoimmune diseases. However, the mechanisms underlying for the effect remain unknown. We previously showed that, among 11 compounds extracted from H hedyotidea, betulin produced the strongest suppressive effect on T cell activation. Here, we examined the hepatoprotective effects of betulin against acute autoimmune hepatitis in mice and the mechanisms underlying the effects. METHODS: Freshly isolated mouse splenocytes were stimulated with concanavalin A (Con A, 5 μg/mL) in the presence of betulin, the cell proliferation was assessed with CSFE-dilution assay...
October 31, 2016: Acta Pharmacologica Sinica
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