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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/28203139/spontaneous-splenic-infarcts-in-a-cirrhotic-patient-with-primary-biliary-cirrhosis
#1
Fredy Nehme, Kyle Rowe, Ahmad Haris, Imad Nassif
Spontaneous splenic infarction has been rarely reported as a complication of cirrhosis and portal hypertension. We describe the case of a 67-year-old female with past medical history of primary biliary cirrhosis presenting for a 1-day history of left upper quadrant pain. Investigations were in favor of splenic infarcts secondary to portal hypertension. The patient improved with conservative management and no recurrence was noted on further follow-up. Splenic infarction must be kept in mind when a patient with cirrhosis presents with left upper quadrant abdominal pain without a clear source...
January 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/28197333/a-case-of-primary-biliary-cirrhosis-mimicking-acute-hepatitis-b-in-the-clinic-republic-of-korea
#2
Woo Hyuk Kwon, Hong Min Park, Jeong Jun Park, Sung Hoon Lee, Yong Kyu Lee
Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen...
January 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28187190/widespread-gli-expression-but-limited-canonical-hedgehog-signaling-restricted-to-the-ductular-reaction-in-human-chronic-liver-disease
#3
Candice Alexandra Grzelak, Nicholas David Sigglekow, Janina Elke Eleonore Tirnitz-Parker, Elizabeth Jane Hamson, Alessandra Warren, Bharvi Maneck, Jinbiao Chen, Bramilla Patkunanathan, Jade Boland, Robert Cheng, Nicholas Adam Shackel, Devanshi Seth, David Geoffrey Bowen, Luciano Gastón Martelotto, D Neil Watkins, Geoffrey William McCaughan
Canonical Hedgehog (Hh) signaling in vertebrate cells occurs following Smoothened activation/translocation into the primary cilia (Pc), followed by a GLI transcriptional response. Nonetheless, GLI activation can occur independently of the canonical Hh pathway. Using a murine model of liver injury, we previously identified the importance of canonical Hh signaling within the Pc+ liver progenitor cell (LPC) population and noted that SMO-independent, GLI-mediated signals were important in multiple Pc-ve GLI2+ intrahepatic populations...
2017: PloS One
https://www.readbyqxmd.com/read/28127155/the-histone-modification-code-in-the-pathogenesis-of-autoimmune-diseases
#4
REVIEW
Yasuto Araki, Toshihide Mimura
Autoimmune diseases are chronic inflammatory disorders caused by a loss of self-tolerance, which is characterized by the appearance of autoantibodies and/or autoreactive lymphocytes and the impaired suppressive function of regulatory T cells. The pathogenesis of autoimmune diseases is extremely complex and remains largely unknown. Recent advances indicate that environmental factors trigger autoimmune diseases in genetically predisposed individuals. In addition, accumulating results have indicated a potential role of epigenetic mechanisms, such as histone modifications, in the development of autoimmune diseases...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28114749/risk-of-secondary-osteoporosis-due-to-lobular-cholestasis-in-non-cirrhotic-primary-biliary-cholangitis
#5
Anna Seki, Fusao Ikeda, Hirokazu Miyatake, Koichi Takaguchi, Shosaku Hayashi, Toshiya Osawa, Shin-Ichi Fujioka, Ryoji Tanaka, Masaharu Ando, Hiroyuki Seki, Yoshiaki Iwasaki, Kazuhide Yamamoto, Hiroyuki Okada
BACKGROUND AND AIM: It remains unclear whether primary biliary cholangitis (PBC) represents a risk factor for secondary osteoporosis. METHODS: A case-control study was conducted to examine bone mineral density and bone turnover markers in middle-aged postmenopausal PBC patients without liver cirrhosis. We compared the incidence of low bone mineral density between propensity-score matched subgroups of PBC patients and healthy controls, and investigated the mechanisms underlying unbalanced bone turnover in terms of the associations between bone turnover markers and PBC-specific histological findings...
January 23, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28073410/-correlation-of-fibrotouch-and-fibroscan-with-the-stage-of-primary-biliary-cirrhosis
#6
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
#7
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
#8
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
#9
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
#10
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...
March 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28003721/primary-biliary-cholangitis-disease-pathogenesis-and-implications-for-established-and-novel-therapeutics
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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