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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/29333908/false-positive-acetylcholine-receptor-antibodies-in-a-case-of-unilateral-chronic-progressive-external-ophthalmoplegia-case-report-and-review-of-literature
#1
Rehan Rajput, Amun Sachdev, Nizar Din, Erika Marie Damato, Aidan Murray
METHODS: We present a rare case with atypical presenting features of unilateral CPEO with a false positive Acetylcholine Receptor Antibody (AchRA) test resulting in diagnostic delay. We illustrate the unilateral nature of this case and demonstrate the caveats of performing myogenic ptosis correction in such patients. We also discuss the differential diagnosis of false positive AchRA, a test commonly performed in the investigation of ptosis. RESULTS: A 34-year old female presented with a more than 3-year history of slowly-progressive, unilateral, right-sided restriction in eye movements and ptosis...
January 15, 2018: Orbit
https://www.readbyqxmd.com/read/29333041/pharmacological-evaluation-of-hepatoprotective-activity-of-ahpl-aytab-0613-tablet-in-carbon-tetrachloride-ethanol-and-paracetamol-induced-hepatotoxicity-models-in-wistar-albino-rats
#2
Sanjay U Nipanikar, Sohan S Chitlange, Dheeraj Nagore
Background: Hepatotoxicity ultimately leads to liver failure. Conventional treatment options for hepatotoxicity are limited and not safe. Objective: Formulation AHPL/AYTAB/0613 is developed to provide safer and effective hepatoprotective drug of natural origin. A study was conducted to evaluate hepatoprotective activity of AHPL/AYTAB/0613 (three dosages) in comparison with marketed formulations in carbon tetrachloride (CCl4), ethanol, and paracetamol-induced hepatotoxicity in Wistar albino rats...
December 2017: Pharmacognosy Research
https://www.readbyqxmd.com/read/29325274/-an-interpretation-of-2017-easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#3
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/29325272/-advances-in-the-treatment-of-primary-biliary-cholangitis
#4
Y M Li, Q X Wang, X Ma
Primary biliary cholangitis (PBC) is an autoimmune liver disease mainly involving intrahepatic interlobular bile ducts and can progress to liver fibrosis, liver cirrhosis, and even liver failure. Ursodeoxycholic acid (UDCA) is the first-line therapeutic drug for PBC and can delay disease progression, but as high as 40% of patients have suboptimal response to UDCA. Obeticholic acid, a farnesoid X receptor agonist, has been approved by FDA in May 2016 for patients who have no response to UDCA treatment or cannot tolerate such treatment...
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
#5
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/29318378/mac-2-binding-protein-glycan-isomer-m2bpgi-is-a-new-serum-biomarker-for-assessing-liver-fibrosis-more-than-a-biomarker-of-liver-fibrosis
#6
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
https://www.readbyqxmd.com/read/29317337/heart-and-bile-acids-clinical-consequences-of-altered-bile-acid-metabolism
#7
REVIEW
Tharni Vasavan, Elisa Ferraro, Effendi Ibrahim, Peter Dixon, Julia Gorelik, Catherine Williamson
Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart...
January 6, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29311728/editorial-itching-to-know-role-of-fibrates-in-pbc
#8
Cynthia Levy, Keith D Lindor
Approximately one-third of patients with primary biliary cholangitis (PBC) fail to respond to ursodeoxycholic acid (UDCA) and are at risk for progression to biliary cirrhosis and end-stage liver disease. In this paper by Pares et al., the authors evaluate the effect of long-term use of bezafibrate in patients with primary biliary cholangitis (PBC) and inadequate response to UDCA. They found that addition of bezafibrate led to normalization of serum alkaline phosphatase in half of the study subjects and major improvement in pruritus...
January 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29307284/environmental-basis-of-primary-biliary-cholangitis
#9
Atsushi Tanaka, Patrick Sc Leung, M Eric Gershwin
Autoimmunity is a consequence of both genetic and environmental factors, occurring in genetically susceptible hosts with environmental triggers. While genome-wide association studies have revealed a number of susceptible genes contributing to etiology, the environmental triggers remain poorly understood. Primary biliary cholangitis, formally known as primary biliary cirrhosis, is considered a model autoimmune disease for which our group has extensively evaluated environmental factors involved in its etiology...
January 2018: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/29307132/current-epidemiology-and-clinical-characteristics-of-autoimmune-liver-diseases-in-south-korea
#10
Sook-Hyang Jeong
Autoimmune liver diseases including autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are rare diseases. The aim of this review is to examine the epidemiology and clinical characteristics of AIH and PBC in South Korea. There were 4,085 patients registered as AIH in the Rare Intractable Disease Registry of Korea between 2009-2013, with a median age of 56 years and female-to male ratio of 6.4. The age-adjusted incidence and prevalence of AIH were 1.07/100,000/year and 4.82/100,000 persons, respectively...
January 5, 2018: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/29297981/primary-biliary-cholangitis-in-british-columbia-first-nations-clinical-features-and-discovery-of-novel-genetic-susceptibility-loci
#11
Sirisha Asuri, Sarah McIntosh, Valerie Taylor, Andrew Rokeby, James Kelly, Karey Shumansky, L Leigh Field, Eric M Yoshida, Laura Arbour
BACKGROUND AND AIMS: Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease characterized by destruction of intrahepatic bile ducts, portal inflammation, and cirrhosis. Although rare in most populations, it is prevalent and often familial in British Columbia First Nations. We hypothesized that major genetic factors increased the risk in First Nations. METHODS: 44 individuals with PBC and 61 unaffected relatives from 32 First Nations families participated...
January 3, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29246416/hepatobiliary-involvement-in-systemic-sclerosis-and-the-cutaneous-subsets-characteristics-and-survival-of-patients-from-the-spanish-rescle-registry
#12
Begoña Marí-Alfonso, Carmen Pilar Simeón-Aznar, Alfredo Guillén-Del Castillo, Manuel Rubio-Rivas, Luis Trapiella-Martínez, José Antonio Todolí-Parra, Mónica Rodríguez Carballeira, Adela Marín-Ballvé, Nerea Iniesta-Arandia, Dolores Colunga-Argüelles, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, José Antonio Vargas Hitos, Antonio-J Chamorro, Ana Belen Madroñero-Vuelta, Isabel Perales-Fraile, Xavier Pla-Salas, Rafael A Fernández-De-La-Puebla, Vicent Fonollosa-Pla, Carles Tolosa-Vilella
OBJECTIVE: To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets. METHODS: In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc)...
October 6, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29231188/major-hepatic-complications-in-ursodeoxycholic-acid-treated-patients-with-primary-biliary-cholangitis-risk-factors-and-time-trends-in-incidence-and-outcome
#13
Maren H Harms, Willem J Lammers, Douglas Thorburn, Christophe Corpechot, Pietro Invernizzi, Harry L A Janssen, Pier M Battezzati, Frederik Nevens, Keith D Lindor, Annarosa Floreani, Cyriel Y Ponsioen, Marlyn J Mayo, George N Dalekos, Tony Bruns, Albert Parés, Andrew L Mason, Xavier Verhelst, Kris V Kowdley, Jorn C Goet, Gideon M Hirschfield, Bettina E Hansen, Henk R van Buuren
OBJECTIVES: In this era of near universal ursodeoxycholic acid (UDCA) treatment for primary biliary cholangitis (PBC), progression to cirrhosis still occurs in an important proportion of patients. The aim of this study was to describe the incidence of cirrhosis-associated complications in patients with PBC and assess risk factors and impact on survival. METHODS: Cohorts of UDCA-treated patients from 16 European and North-American liver centers were included. We used Cox proportional hazards assumptions and Kaplan-Meier estimates...
December 12, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29209127/prediction-of-hepatocellular-carcinoma-development-by-aminotransferase-to-platelet-ratio-index-in-primary-biliary-cholangitis
#14
Ka-Shing Cheung, Wai-Kay Seto, James Fung, Lung-Yi Mak, Ching-Lung Lai, Man-Fung Yuen
AIM: To investigate the usefulness of aspartate aminotransferase to platelet ratio index (APRI) in predicting hepatocellular carcinoma (HCC) risk in primary biliary cholangitis (PBC). METHODS: We identified PBC patients between 2000 and 2015 by searching the electronic medical database of a tertiary center. The hazard ratio (HR) of HCC with different risk factors was determined by Cox proportional hazards model. RESULTS: One hundred and forty-four PBC patients were recruited...
November 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29201778/liver-disease-in-sri-lanka
#15
REVIEW
Hasitha S Wijewantha
Liver disease in Sri Lanka is mainly due to alcoholic liver disease and nonalcoholic fatty liver disease. In contrast to other South Asian countries, the prevalence of hepatitis B and C is low in Sri Lanka and prevalence of hepatitis A is intermediate. The few reported cases of hepatitis E in Sri Lanka are mainly in people who have traveled to neighboring South Asian countries. Wilson's disease, autoimmune hepatitis, hemochromatosis, drug-induced liver disease, and primary biliary cirrhosis are recognized causes of liver disease in Sri Lanka...
January 2017: Euroasian Journal of Hepato-Gastroenterology
https://www.readbyqxmd.com/read/29199390/autoimmune-inflammatory-syndrome-induced-by-adjuvants-asia-related-to-biomaterials-analysis-of-45-cases-and-comprehensive-review-of-the-literature
#16
Jaume Alijotas-Reig, Enrique Esteve-Valverde, Natalia Gil-Aliberas, Victor Garcia-Gimenez
Systemic autoimmune or granulomatous disorders related to biomaterials of human use have rarely been described. The aim of this study was to report cases of autoimmune/inflammatory syndrome induced by adjuvants (ASIA) related to biomaterial injections and prostheses, mainly silicone, hyaluronic acid, acrylamides and methacrylate compounds in a Spanish patient cohort. This study is a retrospective analysis of clinical, laboratory, histopathological and follow-up data of 45 cases of patients suffering from late-onset, non-infectious inflammatory/autoimmune disorders related to bioimplants...
December 3, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29164395/primary-biliary-cholangitis-a-comprehensive-overview
#17
REVIEW
Ana Lleo, Simona Marzorati, Juan-Manuel Anaya, M Eric Gershwin
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear...
November 2017: Hepatology International
https://www.readbyqxmd.com/read/29159718/genetics-and-epigenetics-in-the-pathogenesis-of-primary-biliary-cholangitis
#18
REVIEW
Satoru Joshita, Takeji Umemura, Eiji Tanaka, Masao Ota
Primary biliary cholangitis (PBC) is a chronic, slowly progressive cholestatic autoimmune liver disease predominantly afflicting women. PBC is characterized by the presence of disease-specific antimitochondrial antibodies and the histological destruction of intrahepatic bile ducts, which eventually lead to cirrhosis and hepatic failure. Fortunately, ursodeoxycholic acid therapy has improved the outcome of the vast majority of PBC cases. Although the etiology of PBC has not yet been elucidated, human leukocyte antigen (HLA) class II alleles have been consistently associated with disease onset for decades...
November 20, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#19
Marlen Ivón Castellanos Fernández, Deyanira la Rosa Hernández, Diego Enrique Cabrera Eugenio, Wilson Palanca, Zaily Dorta Guridi, Licet González Fabián
Background: Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean countries. Objectives: The aim of our study was to investigate the clinical patterns, laboratory findings, and immunologic features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. Methods: A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba, from May 2012 to April 2016...
2017: Current Therapeutic Research, Clinical and Experimental
https://www.readbyqxmd.com/read/29152767/development-and-validation-of-a-primary-sclerosing-cholangitis-specific-patient-reported-outcomes-instrument-the-psc-pro
#20
Zobair M Younossi, Arian Afendy, Maria Stepanova, Andrei Racila, Fatema Nader, Rachel Gomel, Ricky Safer, William R Lenderking, Anne Skalicky, Leah Kleinman, Robert P Myers, G Mani Subramanian, John G McHutchison, Cynthia Levy, Christopher L Bowlus, Kris Kowdley, Andrew J Muir
BACKGROUND: PSC is a chronic liver disease associated with inflammation and biliary fibrosis that leads to cholangitis, cirrhosis, and impaired quality of life. Our objective was to develop and validate a PSC-specific PRO instrument. METHODS: We developed a 42-item PSC PRO instrument that contains 2 modules (Symptoms and Impact of Symptoms), and conducted external validation. Reliability and validity were evaluated using clinical data and a battery of other validated instruments...
November 20, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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