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Primary biliary cirrhosis

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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/27876037/the-efficacy-of-adalimumab-in-psoriatic-arthritis-concomitant-to-overlapping-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis-a-case-report
#2
Teresa Del Ross, Amelia Ruffatti, Annarosa Floreani, Ariela Hoxha, Leonardo Punzi
BACKGROUND: The overlap syndrome of primary biliary cholangitis (formerly called primary biliary cirrhosis) and primary sclerosing cholangitis is an extremely rare condition that has never been described in association with other immune-mediated diseases, including psoriatic arthritis. While treatment with anti-Tumour Necrosis Factor-alpha (TNF-α) agents has proved to be effective in inflammatory arthropathies such as psoriatic arthritis, they have been employed in only a limited number of patients with autoimmune hepatitis, and their effectiveness is unclear...
November 22, 2016: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/27864100/clinical-relevance-of-combined-anti-mitochondrial-m2-detection-assays-for-primary-biliary-cirrhosis
#3
Eunhee Han, Sung Jin Jo, Hyeyoung Lee, Ae-Ran Choi, Jihyang Lim, Eun-Sun Jung, Eun-Jee Oh
BACKGROUND: Antimitochondrial antibody (AMA) is a specific serologic marker in primary biliary cirrhosis (PBC). The aim of this study was to evaluate the clinical relevance of combined AMA assays. METHODS: Sera were obtained from 79 patients with PBC and 108 patients with other liver disease. They were tested by indirect immunofluorescence (IIF) using rat kidney/stomach tissue and HEp2 cells as substrate, 4 AMA-M2 assays, anti-sp100, and anti-gp210 assays. RESULTS: Using IIF-AMA with cut-off titer of 1:40, the sensitivity and specificity for PBC were 88...
November 15, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/27855457/-cholestatic-liver-disease
#4
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/27848930/systemic-sclerosis-and-organ-specific-antibodies
#5
Ewa Wielosz, Maria Majdan, Arkadiusz Koszarny, Magdalena Dryglewska, Jacek Tabarkiewicz
INTRODUCTION    According to literature, some organ-specific antibodies may be present in systemic sclerosis (SSc).  OBJECTIVES    The objective of this study was to assess the prevalence of antithyroid antibodies (aTPO and/or aTG) and antimitochondrial antibodies (AMAs) in SSc patients (pts) moreover, to evaluate their clinical consequences.  PATIENTS AND METHODS    Analysis involved 86 consecutive patients with SSc hospitalized in the Department of Rheumatology; 32 patients had diffuse cutaneous (dcSSc) and 54 had limited cutaneous (lcSSc)...
October 5, 2016: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/27843932/a-case-of-frontal-fibrosing-alopecia-in-a-patient-with-primary-biliary-cirrhosis-and-polymyalgia-rheumatica
#6
Ariana N Eginli, Courtney W Bagayoko, Amy J McMichael
Frontal fibrosing alopecia (FFA) is a form of scarring hair loss that is characterized by hair follicle destruction in a fronto-temporo-parietal distribution. Its etiology is unknown; however, most authors presently favor an immune pathogenesis. Associated autoimmune connective tissue diseases have been reported in patients with FFA. We present a case of FFA in a woman with primary biliary cirrhosis and polymyalgia rheumatica, suggesting an association between these clinical entities and supporting a potential autoimmune etiology of FFA...
September 2016: Skin Appendage Disorders
https://www.readbyqxmd.com/read/27834858/impact-of-microbes-on-the-pathogenesis-of-primary-biliary-cirrhosis-pbc-and-primary-sclerosing-cholangitis-psc
#7
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/27832649/bile-acids-and-the-potential-role-in-primary-biliary-cirrhosis
#8
Hang Yang, Zhijun Duan
BACKGROUND: Bile acids (BAs) play a potential role in regulating the whole-body metabolic homeostasis via the interaction with gut microbiome and the signal transduction as messengers, which establish a link between the primary biliary cirrhosis (PBC) and gut microbiome in many aspects, particularly with regard to the immune system of the body. PBC, as a chronic cholestatic liver disease characterised by the destruction of small intrahepatic bile ducts, causes fibrosis and potential cirrhosis without efficient therapies...
November 11, 2016: Digestion
https://www.readbyqxmd.com/read/27784437/-environmental-factors-and-primary-biliary-cirrhosis
#9
L P Chen, H Zhao, B Lyu, J L Cheng
The complex interplay between immune factors and genetic susceptibility plays an essential role in autoimmune diseases. This is especially true for primary biliary cirrhosis (PBC). PBC is an autoimmune cholestatic liver disease characterized by the destruction of the small intrahepatic bile ducts and the presence of high-titer antimitochondrial antibodies (AMA). Among the currently known risk factors, genetic predisposition remains to be the dominant one. However, it is insufficient to explain the different geographic distributions of PBC and the incomplete concordance in identical twins...
July 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27778443/plasmapheresis-exerts-a-long-lasting-antipruritic-effect-in-severe-cholestatic-itch
#10
Marcin Krawczyk, Roman Liebe, Michał Wasilewicz, Ewa Wunsch, Joanna Raszeja-Wyszomirska, Piotr Milkiewicz
BACKGROUND & AIMS: The amelioration of refractory cholestatic pruritus after plasmapheresis has been reported in single patients. Here we analyse the efficacy of plasmapheresis in a cohort of patients with primary biliary cholangitis (PBC). METHODS: Seventeen consecutive patients with PBC (age range 39-85 years, 16 females, 9 with cirrhosis) with refractory pruritus underwent 129 plasmapheresis procedures during 40 admissions. Pruritus was quantified by the 10 point numeric rating scale (NRS) before and after plasmapheresis, as well as ~30 and ~90 days later...
October 25, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27777894/pulmonary-manifestations-among-patients-with-primary-biliary-cirrhosis
#11
Deniz Koksal, Aydin Seref Koksal, Ahmet Gurakar
Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC...
September 28, 2016: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/27754960/red-blood-cell-distribution-width-and-mean-platelet-volume-are-potential-prognostic-indices-for-patients-with-primary-biliary-cirrhosis
#12
Li-Li Wang, Ting-Ting Wei, Jian-Rong Yin, Bao-Dong Qin, Ning Ma, Qing-Qin Tang, Lin Zhou, Ren-Qian Zhong
No abstract text is available yet for this article.
October 18, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/27754570/primary-biliary-cirrhosis-has-high-wait-list-mortality-among-patients-listed-for-liver-transplantation
#13
Ashwani K Singal, Xiao Fang, Mohamed Kaif, Mohsen Hasanin, Brendan M Mcguire, Yong-Fang Kuo, Russell H Wiesner
Patients with primary sclerosing cholangitis (PSC) have frequent episodes of cholangitis with potential for high mortality while waiting for liver transplantation. However, data on wait-list mortality specific to liver disease etiology are limited. Using United Network for Organ Sharing (UNOS) database (2002-2013), of 81 592 listed patients, 11 284 (13.8%) died while waiting for transplant. Primary biliary cirrhosis (PBC) patients (N = 3491) compared to PSC (N = 4905) differed with age (56 vs. 47 years), female gender (88% vs...
October 18, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27729952/cirrhosis-and-autoimmune-liver-disease-current-understanding
#14
Rodrigo Liberal, Charlotte R Grant
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues...
October 8, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/27728258/a-case-of-primary-biliary-cirrhosis
#15
G Ramesh
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27721424/dual-roles-of-ifn-%C3%AE-and-il-4-in-the-natural-history-of-murine-autoimmune-cholangitis-il-30-and-implications-for-precision-medicine
#16
Bi-Jhen Syu, Chia-En Loh, Yu-Hsin Hsueh, M Eric Gershwin, Ya-Hui Chuang
Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease with a long natural history. The pathogenesis of PBC is thought to be orchestrated by Th1 and/or Th17. In this study, we investigated the role of CD4(+) helper T subsets and their cytokines on PBC using our previous established murine model of 2-OA-OVA immunization. We prepared adeno-associated virus (AAV)-IFN-γ and AAV-IL-4 and studied their individual influences on the natural history of autoimmune cholangitis in this model. Administration of IFN-γ significantly promotes recruitment and lymphocyte activation in the earliest phases of autoimmune cholangitis but subsequently leads to downregulation of chronic inflammation through induction of the immunosuppressive molecule IL-30...
October 10, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27717116/clinical-significance-of-liver-histology-on-outcomes-in-biliary-atresia
#17
Nicholas L Webb, Ashish Jiwane, Chee Y Ooi, Scott Nightinghale, Susan E Adams, Usha Krishnan
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014...
October 7, 2016: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27707675/serum-proteomic-signatures-as-biomarkers-of-primary-biliary-cirrhosis-diagnosis-and-prognosis
#18
Bruno Baudin, Arnaud Bruneel, Raoul Poupon, Michel Vaubourdolle
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease considered as an autoimmune disease. To identify new biomarkers of PBC, serum profiling analysis using Surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF-MS) was employed. Twelve patients with either asymptomatic PBC (group 1, n=6) or PBC with a poor response to UDCA (group 2, n=6), were compared to healthy controls (group 3, n=6). Analysing the 18 sera by using four SELDI-TOF arrays under various conditions, we found four biomarkers of PBC at 5...
October 1, 2016: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/27700211/primary-biliary-cholangitis-medical-and-specialty-pharmacy-management-update
#19
Christopher L Bowlus, James T Kenney, Gary Rice, Robert Navarro
BACKGROUND: Chronic liver disease and cirrhosis are a leading cause of morbidity and mortality in the United States. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis and which has been designated an orphan condition, is a chronic autoimmune disease resulting in the destruction of the small bile ducts in the liver. Without effective treatment, disease progression frequently leads to liver failure and death. Until May 2016, the only FDA-approved treatment for PBC was ursodiol (UDCA), an oral hydrophilic bile acid, which can slow progression of liver damage due to PBC...
October 2016: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/27688660/etiology-of-chronic-liver-diseases-in-the-northwest-of-italy-1998-through-2014
#20
Giorgio Maria Saracco, Andrea Evangelista, Sharmila Fagoonee, Giovannino Ciccone, Elisabetta Bugianesi, Gian Paolo Caviglia, Maria Lorena Abate, Mario Rizzetto, Rinaldo Pellicano, Antonina Smedile
AIM: To assess the etiology of chronic liver diseases (CLD) from 1998 to 2014 at the outpatient clinic of Gastroenterology of the main hospital in Northwest of Italy among those dedicated to hepatology. METHODS: A random sample of charts of patients referred to for increased liver enzymes between January 1998 and December 2006, and between January 2012 and December 2014 were reviewed. Etiology search included testing for hepatitis B virus (HBV), hepatitis C virus (HCV), autoimmune hepatitis, primary biliary cirrhosis, Wilson's disease and hereditary hemocromatosis...
September 28, 2016: World Journal of Gastroenterology: WJG
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