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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/28073410/-correlation-of-fibrotouch-and-fibroscan-with-the-stage-of-primary-biliary-cirrhosis
#1
Y G Zhang, S X Zhao, G D Zhou, W C Li, W G Ren, H J Du, R Q Wang, Y M Nan
Objective: To investigate the diagnostic value of FibroTouch and FibroScan for the stage of primary biliary cirrhosis (PBC). Methods: A total of 66 PBC patients who visited our hospital from January 2014 to March 2016 were enrolled, and all the patients underwent liver biopsy and FibroTouch and FibroScan tests. Liver stiffness measurement (LSM) was used to assess fibrosis degree, and the receiver operating characteristic (ROC) curve was used to compare the cut-off values, sensitivities, and specificities of these two methods in determining fibrosis stage...
December 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28070198/clinical-course-and-genetic-susceptibility-of-primary-biliary-cirrhosis-analysis-of-a-prospective-cohort
#2
Piero Luigi Almasio, Anna Licata, Marcello Maida, Fabio Salvatore Macaluso, Andrea Costantino, Nicola Alessi, Stefania Grimaudo, Giulia Accardi, Calogero Caruso, Antonio Craxi
BACKGROUND: Natural history of primary biliary cirrhosis (PBC) is partially characterized in patients from the Mediterranean area whose genetic background differs from that of Northern Europeans. OBJECTIVES: We aimed to describe genetic susceptibility and clinical course of PBC in patients from Southern Italy. METHODS: Socio-demographic, clinical, biochemical and histological data at diagnosis as well as disease progression of 81 PBC consecutive patients were collected...
November 2016: Hepatitis Monthly
https://www.readbyqxmd.com/read/28063180/do-occupational-exposures-to-vinyl-chloride-cause-hepatocellular-carcinoma-and-cirrhosis
#3
Marcello Lotti
Controversy exists about the association between occupational exposures to vinyl chloride and hepatocellular carcinoma and cirrhosis. Two large multicentre mortality cohort studies, one American and another European, reported higher mortality for primary cancer of liver and biliary tract. However, the American study was not able to rule out misclassification, because based on death certificates and under the heading primary liver cancers, some angiosarcomas, the typical neoplasia associated with vinyl chloride, may have been included...
January 7, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#4
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28030370/the-evolution-of-natural-history-of-primary-sclerosing-cholangitis
#5
Will R Takakura, James H Tabibian, Christopher L Bowlus
PURPOSE OF REVIEW: Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. RECENT FINDINGS: PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis...
December 24, 2016: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28003721/primary-biliary-cholangitis-disease-pathogenesis-and-implications-for-established-and-novel-therapeutics
#6
REVIEW
Amitkumar Patel, Anil Seetharam
Primary Biliary Cholangitis is a progressive, autoimmune cholestatic liver disorder. Cholestasis with disease progression may lead to dyslipidemia, osteodystrophy and fat-soluble vitamin deficiency. Portal hypertension may develop prior to advanced stages of fibrosis. Untreated disease may lead to cirrhosis, hepatocellular cancer and need for orthotopic liver transplantation. Classically, diagnosis is made with elevation of alkaline phosphatase, demonstration of circulating antimitochondrial antibody, and if performed: asymmetric destruction/nonsupperative cholangitis of intralobular bile ducts on biopsy...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27999888/imaging-of-autoimmune-hepatitis-and-overlap-syndromes
#7
Neera Malik, Sudhakar K Venkatesh
Autoimmune hepatitis (AIH) is an uncommon, chronic inflammatory, and relapsing liver disease of unknown origin that may lead to liver cirrhosis, hepatocellular carcinoma, liver transplantation, or death. AIH occurs in all age groups and races but can frequently manifest as acute fulminant hepatitis. Clinical presentation of AIH can have features similar to primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC), and these diseases may coexist leading to overlap syndromes. Although histological diagnosis is necessary, imaging features often can demonstrate characteristics that may be helpful to distinguish these diseases...
January 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/27997975/primary-biliary-cirrhosis-and-the-microbiome
#8
Eamonn M M Quigley
Primary biliary cirrhosis is a rather uncommon, slowly progressive, cholestatic liver disease that predominantly affects middle-aged women. Apart from the changes in the gut microbiome that have been described in liver disease in general, little is known of the composition of the microbiome in primary biliary cirrhosis. Nevertheless, epidemiological, clinical, and some experimental evidence points to the possible role of a bacterium (or bacteria) in the initiation of the autoimmune process that leads to the development of this unique clinical phenotype...
September 2016: Seminars in Liver Disease
https://www.readbyqxmd.com/read/27995739/survival-in-patients-with-child-pugh-class-c-cirrhosis-analysis-of-the-liver-transplant-registry-in-japan
#9
Takuya Genda, Takafumi Ichida, Shotaro Sakisaka, Eiji Tanaka, Satoshi Mochida, Yoshiyuki Ueno, Ayano Inui, Hiroto Egawa, Koji Umeshita, Hiroyuki Furukawa, Seiji Kawasaki, Yukihiro Inomata
AIM: To clarify the survival and prognostic factors in patients with Child-Turcotte-Pugh class C (CTP-C) cirrhosis. METHODS: From all candidates for deceased donor liver transplantation in Japan between 2007 and 2015, 1014 adult patients with CTP-C cirrhosis were retrospectively enrolled in this study. Hazard ratio (HR) of factors associated with mortality was estimated by Cox proportional hazard model. The survival probabilities were evaluated by Kaplan-Meier analysis and the log-rank test...
December 20, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/27986173/liver-function-assessment-by-magnetic-resonance-imaging
#10
Emre Ünal, Deniz Akata, Musturay Karcaaltincaba
Liver function assessment by hepatocyte-specific contrast-enhanced magnetic resonance imaging is becoming a new biomarker. Liver function can be assessed by T1 mapping (reduction rate) and signal intensity measurement (relative enhancement ratio) before and after GD-EOB-DTPA (gadoxetic acid) administration, as alternative to Tc-99m galactosyl serum albumin scintigraphy, 99m Tc-labeled mebrofenin scintigraphy, and indocyanine green clearance test. Magnetic resonance imaging assessment of liver function can enable diagnosis of cirrhosis, nonalcoholic fatty liver disease associated fibrosis and steatohepatitis, primary sclerosing cholangitis, toxic hepatitis, and chemotherapy and radiotherapy-related changes, which may be only visible on hepatobiliary phase images...
December 2016: Seminars in Ultrasound, CT, and MR
https://www.readbyqxmd.com/read/27981308/primary-biliary-cirrhosis-and-primary-sclerosing-cholangitis-an-update-on-mr-imaging-findings-with-recent-developments
#11
REVIEW
Jelena Djokić Kovač, Marc-André Weber
Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are the most common immune-mediated chronic cholestatic liver diseases leading to cirrhosis and liver failure. Although magnetic resonance imaging (MRI) is not a necessary procedure for the diagnosis of PBC, MRI is recommended for monitoring disease progression and early detection of complications. Even though liver cirrhosis subtypes have similar MR imaging features, there are some findings which could indicate PBC, such as the periportal halo sign...
December 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/27978928/-changes-in-peripheral-blood-25-hydroxyvitamin-d3-th17-cells-and-cd4-regulatory-t-cells-and-their-clinical-significance-in-patients-with-primary-biliary-cirrhosis
#12
Y M Zhong, X R Wu, Q Wang, M F Yu, T Lu, M Y Zhao
Objective: To investigate the changes in peripheral blood 25-hydroxyvitamin D3[25-(OH)D3], CD4(+)regulatory T (Treg) cells, and Th17 cells in patients with primary biliary cirrhosis (PBC) and their mechanism of action in PBC. Methods: A total of 22 patients with PBC were enrolled and the male/female ratio was 1:21, with a mean age of 61±12 years. There were 7 healthy volunteers matched for age in the normal control group. Electrochemiluminescence immunoassay was used to measure the peripheral blood 25-(OH)D3level in the PBC group and normal control group, and flow cytometry was used to analyze the changes in Th17 cells and CD4(+)Treg cells...
November 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27965673/the-role-of-lymphatic-endothelial-cells-in-liver-injury-and-tumor-development
#13
REVIEW
Veronika Lukacs-Kornek
Lymphatics and lymphatic endothelial cells (LECs) possess multiple immunological functions besides affecting immune cell migration, such as inhibiting T cell proliferation and antigen presentation by dendritic cells. Moreover, they control the trans-endothelial transport of multiple molecules and antigens. Emerging evidence suggest their active involvements in immunregulation, tumor, and metastases formation. In the liver, increased lymphangiogenesis, specifically at the portal area has been associated with multiple liver diseases in particular primary biliary cirrhosis, idiopathic portal hypertension, and liver malignancies...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27957759/systematic-review-recurrent-autoimmune-liver-diseases-after-liver-transplantation
#14
REVIEW
A J Montano-Loza, R A Bhanji, S Wasilenko, A L Mason
BACKGROUND: Autoimmune liver diseases (AILD) constitute the third most common indication for liver transplantation (LT) worldwide. Outcomes post LT are generally good but recurrent disease is frequently observed. AIMS: To describe the frequency and risk factors associated with recurrent AILD post-LT and provide recommendations to reduce the incidence of recurrence based on levels of evidence. METHODS: A systematic review was performed for full-text papers published in English-language journals, using the keywords 'autoimmune hepatitis (AIH)', 'primary biliary cholangitis and/or cirrhosis (PBC)', 'primary sclerosing cholangitis (PSC)', 'liver transplantation' and 'recurrent disease'...
December 12, 2016: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/27957241/recent-advances-in-the-diagnosis-and-treatment-of-primary-biliary-cholangitis
#15
REVIEW
Ying-Qiu Huang
Primary biliary cholangitis (PBC), formerly referred to as primary biliary cirrhosis, is an infrequent progressive intrahepatic cholestatic autoimmune illness that can evolve into hepatic fibrosis, hepatic cirrhosis, hepatic failure, and, in some cases, hepatocellular carcinoma. The disease itself is characterized by T-lymphocyte-mediated chronic non-suppurative destructive cholangitis and elevated serum levels of extremely specific anti-mitochondrial autoantibodies (AMAs). In this article, we will not only review epidemiology, risk factors, natural history, predictive scores, radiologic approaches (e...
November 28, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/27920708/cutaneous-metastasis-due-to-breast-cancer-in-a-patient-with-primary-biliary-cirrhosis-a-case-report
#16
Sailaja Kamaraju, Jill Depke, Janice Povletich, Adam Currey, Elizabeth Weil
BACKGROUND: Breast cancer is the most common solid tumor to cause cutaneous metastases. These are incurable and the treatment goal is geared toward local control with surgical excision, radiation, and chemotherapy. However, treatment can be challenging in subjects with end-stage liver disease and a multidisciplinary approach is warranted. CASE REPORT: In this case report, we present a 61-year-old female with primary biliary cirrhosis and human epidermal growth factor-2 (HER-2)-positive breast cancer, who subsequently developed cutaneous metastases...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27916760/interleukin-17a-induced-epithelial-mesenchymal-transition-of-human-intrahepatic-biliary-epithelial-cells-implications-for-primary-biliary-cirrhosis
#17
Qingshui Huang, Shuai Chu, Xiaofeng Yin, Xiaobin Yu, Chunmin Kang, Xin Li, Yurong Qiu
Primary biliary cirrhosis (PBC) is an autoimmune chronic liver disease with worldwide increasing morbidity. However, the etiology of PBC is still unclear. Recently, the epithelial-mesenchymal transition (EMT) and interleukin-17A (IL-17A), a pro-inflammatory cytokine, were proposed to be involved in the pathogenesis of PBC. Therefore, in this study, we aimed to clarify the roles of IL-17A and/or EMT in the onset of PBC. The results showed that the median serum IL-17A level was significantly higher in 29 PBC patients (average course of 40...
2016: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27916754/-autoantibodies-of-inflammatory-myopathies-update
#18
Shigeaki Suzuki
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve the skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by the presence of various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, the correlation between histological features and autoantibodies has not been fully elucidated. Immune-mediated necrotizing myopathy (IMNM), which is characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration, is associated with the presence of autoantibodies...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27916102/-increased-expression-of-il-9-in-peripheral-blood-and-liver-tissues-of-patients-with-primary-biliary-cirrhosis
#19
Qian Wang, Demei Luo, Xiaofeng Sun, Ning Feng, Huijun Li, Jinwei Hu, Wenqiang Yang, Xiumin Ma
Objective To investigate the expression and clinical significance of interleukin 9 (IL-9) in patients with primary biliary cirrhosis (PBC). Methods The study enrolled 80 PBC patients and 100 healthy subjects. Levels of serum IL-9, interferon γ (IFN-γ), IL-6, tumor necrosis factor α (TNF-α), IL-10 and IL-17 were determined by cytometric beads array (CBA); level of IL-9 mRNA in peripheral blood mononuclear cells (PBMCs) was detected by quantitative real-time PCR; the expression and location of IL-9 in liver tissues were detected by immunohistochemistry...
December 2016: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
https://www.readbyqxmd.com/read/27915160/histone-deacetylase-9-plays-a-role-in-the-antifibrogenic-effect-of-astaxanthin-in-hepatic-stellate-cells
#20
Yue Yang, Minkyung Bae, Young-Ki Park, Yoojin Lee, Tho X Pham, Swetha Rudraiah, José Manautou, Sung I Koo, Ji-Young Lee
Activation of hepatic stellate cells (HSCs) is critical for liver fibrosis development. Previously, we showed that astaxanthin (ASTX), a xanthophyll carotenoid, has antifibrogenic effects in LX-2 cells, a human HSC cell line. We sought to determine the effect of ASTX on HSC activation, and to identify molecular mediators that are critically involved in the processes. ASTX prevented the activation of mouse primary HSCs, as evidenced by attenuated induction of procollagen type I α1. In human primary HSCs, ASTX also inhibited transforming growth factor β1 (TGFβ1)-induced fibrogenic gene expression...
November 12, 2016: Journal of Nutritional Biochemistry
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