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https://www.readbyqxmd.com/read/29164395/primary-biliary-cholangitis-a-comprehensive-overview
#1
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 21, 2017: Hepatology International
https://www.readbyqxmd.com/read/29159718/genetics-and-epigenetics-in-the-pathogenesis-of-primary-biliary-cholangitis
#2
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
#3
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/29155355/molecular-ellipticity-of-circulating-albumin-bilirubin-complex-associates-with-mortality-in-patients-with-severe-alcoholic-hepatitis
#4
Sukanta Das, Jaswinder Singh Maras, Rakhi Maiwall, S M Shasthry, Md Shabir Hussain, Shvetank Sharma, S Sukriti, T P Singh, Shiv Kumar Sarin
BACKGROUND & AIMS: Hyperbilirubinemia and hypoalbuminemia are features of hepatic dysfunction that associate with disease severity. This is because hepatic insufficiency causes hypoalbuminemia, which indirectly increases the circulating levels of free bilirubin. Circular dichroism (CD) spectroscopy can be used to quantify the molecular ellipticity (ME) of the albumin-bilirubin complex, and might associate with the severity or outcome of severe alcoholic hepatitis (SAH). METHODS: We performed a cross-sectional study of 265 patients with SAH admitted in the Department of Hepatology, Institute of Liver and Biliary Sciences in New Delhi, India from January 2014 through January 2016...
November 16, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29152767/development-and-validation-of-a-primary-sclerosing-cholangitis-specific-patient-reported-outcomes-instrument-the-psc-pro
#5
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
https://www.readbyqxmd.com/read/29149973/hepatitis-c-virus-recurrence-occurs-earlier-in-patients-receiving-donation-after-circulatory-death-liver-transplant-grafts-compared-with-those-receiving-donation-after-brainstem-death-grafts
#6
S A Townsend, M A Monga, P Nightingale, D Mutimer, A M Elsharkawy, A Holt
INTRODUCTION: Hepatitis C virus (HCV)-related cirrhosis remains the commonest indication for liver transplantation worldwide, yet few studies have investigated the impact of donation after circulatory death (DCD) graft use on HCV recurrence and patient outcomes. DCD grafts have augmented the limited donor organ pool and reduced wait-list mortality, although concerns regarding graft longevity and patient outcome persist. METHODS: This was a single-center study of all HCV + adults who underwent DCD liver transplantation between 2004 and 2014...
November 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29138587/primary-sclerosing-cholangitis-diagnostic-and-management-challenges
#7
REVIEW
Sanjeev Sirpal, Natasha Chandok
Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/29132130/effect-of-huagantongluofang-a-chinese-traditional-medicine-in-hepatic-fibrogenesis-in-a-mouse-model-of-biliary-cirrhosis
#8
Ji Xuan, Wei Wen, Yong Wang, Feng Wang, Hua-Bing Xu, Mei Shao, Ya Yang, Yao-Zhou Tian
BACKGROUND: Biliary cirrhosis (BC) is a chronic cholestatic liver disease, in which hepatic fibrosis is an early symptom. This study aimed to identify the biological function and the therapeutic effect of a Chinese traditional medicine, HuaGanTongLuoFang (HGTLF), in a mouse model of BC. METHODS: The mice (n = 72) were randomly divided into a sham group (n =12) and BC group (n = 60). The animals in the BC group were then randomly divided into five groups (n = 12 in each) and treated with three different doses of HGTLF, ureodeoxycholic acid (UDCA), or normal saline (the model group)...
November 13, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29127685/the-first-case-of-ischemia-free-organ-transplantation-in-humans-a-proof-of-concept
#9
Xiaoshun He, Zhiyong Guo, Qiang Zhao, Weiqiang Ju, Dongping Wang, Linwei Wu, Lu Yang, Fei Ji, Yunhua Tang, Zhiheng Zhang, Shanzhou Huang, Linhe Wang, Zebin Zhu, Kunpeng Liu, Yanling Zhu, Yifang Gao, Wei Xiong, Ming Han, Bing Liao, Maogen Chen, Yi Ma, Xiaofeng Zhu, Wenqi Huang, Changjie Cai, Xiangdong Guan, Xian Chang Li, Jiefu Huang
Ischemia and reperfusion injury (IRI) is an inevitable event in conventional organ transplant procedure and is associated with significant mortality and morbidity post-transplantation. We hypothesize that IRI is avoidable if the blood supply for the organ is not stopped, thus resulting in optimal transplant outcomes. Here we described the first case of a novel procedure called ischemia-free organ transplantation (IFOT) for patients with end-stage liver disease. The liver graft with severe macrovesicular steatosis was donated from a 25-year-old man...
November 10, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/29127360/a-mouse-model-of-autoimmune-cholangitis-via-syngeneic-bile-duct-protein-immunization
#10
Wen-Tao Ma, Qing-Zhi Liu, Jing-Bo Yang, Yan-Qing Yang, Zhi-Bin Zhao, Hong-Di Ma, M Eric Gershwin, Zhe-Xiong Lian
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the destruction of interlobular biliary ductules, which progressively leads to cholestasis, hepatic fibrosis, cirrhosis, and eventually liver failure. Several mouse models have been used to clarify the pathogenesis of PBC and are generally considered reflective of an autoimmune cholangitis. Most models focus on issues of molecular mimicry between the E2 subunit of the pyruvate dehydrogenase complex (PDC-E2), the major mitochondrial autoantigen of PBC and xenobiotic cross reactive chemicals...
November 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29113177/potential-role-of-indoleamine-2-3-dioxygenase-in-primary-biliary-cirrhosis
#11
Kashif Asghar, John Brain, Jeremy M Palmer, Stephen Douglass, Fatmah M A Naemi, Graeme O'Boyle, John Kirby, Simi Ali
Indoleamine 2,3-dioxygenase (IDO)-induced immunosuppression can be clinically beneficial for autoimmune diseases. Primary biliary cirrhosis (PBC) is characterized by autoimmune lesions of intrahepatic bile duct epithelial cells that may lead to irreversible cirrhosis or hepatocellular carcinoma. The present study assessed the expression and function of IDO in a cell culture model and in PBC patients. IDO expression was monitored in a human immortalized but non-malignant biliary epithelial cell (iBEC) line. Increased expression of IDO1/2 was observed in the iBECs following stimulation with interferon-γ (IFN-γ)...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29110825/long-term-outlook-in-biliary-atresia
#12
Arun Kelay, Mark Davenport
The oldest survivors from the Kasai portoenterostomy originate in Sendai, Japan and are approaching their 60th birthday. These represent the tip of an expanding cohort of adults born with this previously fatal condition. Increasingly transition to adult-biased hepatologists and physicians will be the expectation of many with this condition. However unlike their usual patients with alcohol, drugs, virally mediated liver disease these are different with different expectations of health and quality of life. Cure is not on the cards for most of these and they survive still with impaired bile flow and increased liver fibrosis and cirrhosis with the threat of cholangitis and portal hypertension still apparent...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29109128/sox9-predicts-progression-toward-cirrhosis-in-patients-while-its-loss-protects-against-liver-fibrosis
#13
Varinder S Athwal, James Pritchett, Jessica Llewellyn, Katherine Martin, Elizabeth Camacho, Sayyid Ma Raza, Alexander Phythian-Adams, Lindsay J Birchall, Aoibheann F Mullan, Kim Su, Laurence Pearmain, Grace Dolman, Abed M Zaitoun, Scott L Friedman, Andrew MacDonald, William L Irving, Indra N Guha, Neil A Hanley, Karen Piper Hanley
Fibrosis and organ failure is a common endpoint for many chronic liver diseases. Much is known about the upstream inflammatory mechanisms provoking fibrosis and downstream potential for tissue remodeling. However, less is known about the transcriptional regulation in vivo governing fibrotic matrix deposition by liver myofibroblasts. This gap in understanding has hampered molecular predictions of disease severity and clinical progression and restricted targets for antifibrotic drug development. In this study, we show the prevalence of SOX9 in biopsies from patients with chronic liver disease correlated with fibrosis severity and accurately predicted disease progression toward cirrhosis...
November 6, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/29099543/vitamin-d-supplementation-for-chronic-liver-diseases-in-adults
#14
REVIEW
Goran Bjelakovic, Dimitrinka Nikolova, Marko Bjelakovic, Christian Gluud
BACKGROUND: Vitamin D deficiency is often reported in people with chronic liver diseases. Therefore, improving vitamin D status could have a beneficial effect on people with chronic liver diseases. OBJECTIVES: To assess the beneficial and harmful effects of vitamin D supplementation in people with chronic liver diseases. SEARCH METHODS: We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, Science Citation Index Expanded, and Conference Proceedings Citation Index - Science...
November 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29096343/non-anastomotic-strictures-after-transplanting-a-liver-graft-with-an-accidentally-ligated-and-unflushed-common-bile-duct-a-case-report
#15
Nicolas Meurisse, Jacques Pirenne, Diethard Monbaliu
INTRODUCTION: Non-anastomotic biliary strictures (NAS) represent a major cause of morbidity, graft loss, and mortality after liver transplantation (LTx). NAS can result from an ischemic/immune-mediated injury, or from the cytotoxic effect that bile salts have on the biliary mucosa under hypothermic conditions. For this reason it is crucial to flush the bile duct at the time of procurement. PRESENTATION OF CASE: We report a case of an imported liver with an accidentally ligated and subsequently completely unflushed common bile duct...
October 26, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29062305/morbid-sequences-suggest-molecular-mimicry-between-microbial-peptides-and-self-antigens-a-possibility-of-inciting-autoimmunity
#16
Susanta Pahari, Deepyan Chatterjee, Shikha Negi, Jagdeep Kaur, Balvinder Singh, Javed N Agrewala
Understanding etiology of autoimmune diseases has been a great challenge for designing drugs and vaccines. The pathophysiology of many autoimmune diseases may be attributed to molecular mimicry provoked by microbes. Molecular mimicry hypothesizes that a sequence homology between foreign and self-peptides leads to cross-activation of autoreactive T cells. Different microbial proteins are implicated in various autoimmune diseases, including multiple sclerosis, human type 1 diabetes, primary biliary cirrhosis and rheumatoid arthritis...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29057174/concurrent-occurrence-of-primary-biliary-cirrhosis-and-rheumatoid-arthritis
#17
Stella Pak, Umar Darr, Zubair Khan, Andrew Kobalka, Zayd Safadi, Christine Dee
Primary biliary cirrhosis (PBC) is an autoimmune cholestatic disorder of the liver. A diagnostic serum marker for PBC is an anti-mitochondrial antibody. Most prominent histologic findings of PBC are portal inflammation and destruction of interlobular bile ducts. The PBC occurs only in 40 to 400 individuals per million in the general population. About 1.8 - 5.6% of individuals with this rare disorder have rheumatoid arthritis (RA). This case report describes a 56-year-old female with concurrent rheumatoid arthritis and primary biliary cirrhosis...
August 13, 2017: Curēus
https://www.readbyqxmd.com/read/29056017/-research-advances-of-relationship-between-non-alcoholic-fatty-liver-disease-and-biliary-tract-diseases
#18
R X Huang, B C Wang, J G Fan
Recent studies have found that non-alcoholic fatty liver disease(NAFLD) has great impact on the development of biliary tract diseases. Here in this review, we summarized the relationship between NAFLD and the occurrence and development, risk factors and severity of cholestasis, gallstones, intrahepatic cholangiocarcinoma, primary biliary cirrhosis and bile microbiota, so as to further illuminate the pathogenesis of NAFLD and biliary tract diseases, obtain better diagnostic and therapeutic outcomes on NAFLD and biliary tract diseases...
August 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29055922/endoscopic-management-of-primary-sclerosing-cholangitis
#19
Jodie A Barkin, Cynthia Levy, Enrico O Souto
Primary sclerosing cholangitis (PSC) remains a rare but potentially devastating chronic, cholestatic liver disease. PSC causes obstruction of intra- and/or extra-hepatic bile ducts by inflammation and fibrosis, leading to biliary obstruction, cirrhosis and portal hypertension with all associated sequelae. The most dreaded consequence of PSC is cholangiocarcinoma, occurring in 10-20% of patients with PSC, and with population-based estimates of a 398-fold increased risk of cholangiocarcinoma in patients with PSC compared to the general population...
October 16, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/29051789/novel-strategies-and-therapeutic-options-for-the-management-of-primary-biliary-cholangitis
#20
REVIEW
Amardeep Khanna, David E Jones
Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease. It has a varied course of progression ranging from being completely asymptomatic to aggressive disease leading to cirrhosis and resulting in liver transplantation. In addition, symptoms can be debilitating and can have a major impact on quality of life. For decades, there was only one anti-cholestatic agent available to target this disease and that was only effective in around half of patients, with little or no effect on symptoms. With increasing understanding of the pathogenic mechanisms of PBC and potential targets for drug treatment, pharmaceutical companies have shown a greater interest in this rare disease...
October 2017: Therapeutic Advances in Gastroenterology
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