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https://www.readbyqxmd.com/read/29691599/-primary-biliary-cholangitis-established-and-novel-therapies
#1
REVIEW
M Vetter, A E Kremer
BACKGROUND: Patients with primary biliary cholangitis (PBC, formerly primary biliary cirrhosis) and insufficient treatment response or risk factors exhibit a remarkably increased risk for disease progression and associated complications. Furthermore, extrahepatic manifestations may considerably reduce quality of life in affected patients. OBJECTIVES: This article presents an overview on standard therapy with ursodeoxycholic acid (UDCA) and further therapeutic options in patients with insufficient treatment response...
April 24, 2018: Der Internist
https://www.readbyqxmd.com/read/29681633/secondary-sclerosing-cholangitis-in-critically-ill-patients
#2
Carlos Alberto Peña-Pérez, Juan Alberto Díaz Ponce-Medrano
Primary sclerosing cholangitis (PSC) is a rare idiopathic condition with immunopathogenic mechanisms where there is chronic progressive destruction of the biliary tree. Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, but is caused by specific processes which directly damage the biliary tree; examples include recurrent pancreatitis, bile duct malignancy, congenital bile duct abnormalities. A new cause of SSC has been described during or following significant critical illness associated with severe respiratory insufficiency, vasopressor requirement, shock and sepsis...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29678444/biliary-epithelium-a-neuroendocrine-compartment-in-cholestatic-liver-disease
#3
REVIEW
Laurent Ehrlich, Marinda Scrushy, Fanyin Meng, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis is characterized by abnormal accumulation of extracellular matrix (ECM) that can lead to ductopenia, cirrhosis, and even malignant transformation. In this review, we examine cholestatic liver diseases characterized by extensive biliary fibrosis such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), polycystic liver disease (PLD), and MDR2-/- and BDL mouse models. Following biliary injury, cholangiocytes, the epithelial cells that line the bile ducts, become reactive and adopt a neuroendocrine phenotype in which they secrete and respond to neurohormones and neuropeptides in an autocrine and paracrine fashion...
April 17, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29661431/stratifying-mortality-in-a-model-for-end-stage-liver-disease-waiting-list-a-brazilian-single-center-study
#4
R B Martino, D R Waisberg, A P M Dias, V B S Inoue, R M Arantes, L B P Haddad, W Andraus, L D Lopes, F H F Galvão, A T W Song, L A C D'Albuquerque
BACKGROUND: The Model for End-Stage Liver Disease (MELD) system reliably predicts mortality in cirrhotic patients. However, the etiology of liver disease and presence of portal vein thrombosis are not directly taken into account in MELD score. Its impact on the outcomes of patients on the waiting list is still unclear. The aim of this study was to investigate mortality and access to transplantation regarding etiology of liver disease and portal vein thrombosis (PVT). METHODS: A total of 465 adult patients on the liver waiting list from August 2015 to August 2016 were followed up until August 2017...
April 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29658839/comparison-of-emergency-cholecystectomy-with-delayed-cholecystectomy-after-percutaneous-transhepatic-gallbladder-drainage-in-patients-with-moderate-acute-cholecystitis
#5
Chang-Wei Ke, Shuo-Dong Wu
INTRODUCTION: Patients with moderate (grade II) acute cholecystitis patients, as defined by the 2013 Tokyo Guidelines, were retrospectively compared with respect to emergency cholecystectomy (EC) and delayed cholecystectomy (DC) after percutaneous transhepatic gallbladder drainage (PTGBD) to determine the better treatment strategy. METHODS: Forty-nine of 103 patients with PTGBD and 47 of 54 patients with EC were assessed for eligibility from January 2013 to January 2017...
April 16, 2018: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29620413/portosystemic-venous-shunt-portocaval-fistula-in-a-patient-with-biliary-cirrhosis-secondary-to-a-right-hepatectomy-for-hydatidosis
#6
Marta Isabel Díaz de la Torre, Cristina Suárez Ferrer, Antonio Olveira Martín
Major intrahepatic venous shunts are rare conditions where a communication between hepatic veins and intrahepatic portal vessels is established. Potential spontaneous development has been proposed in patients with cirrhosis and portal hypertension (PHT).
April 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29617396/the-impact-of-depression-and-antidepressant-usage-on-primary-biliary-cholangitis-clinical-outcomes
#7
Abdel-Aziz Shaheen, Gilaad G Kaplan, Wagdi Almishri, Isabelle Vallerand, Alexandra D Frolkis, Scott Patten, Mark G Swain
BACKGROUND: Depression is prevalent in primary biliary cholangitis (PBC) patients. Our aims were to examine the effects of depression and antidepressants on hepatic outcomes of PBC patients. METHODS: We used the UK Health Improvement Network database to identify PBC patients between 1974 and 2007. Our primary outcome was one of three clinical events: decompensated cirrhosis, liver transplantation and death. We assessed depression and each class of antidepressant medication in adjusted multivariate Cox proportional hazards models to identify independent predictors of outcomes...
2018: PloS One
https://www.readbyqxmd.com/read/29604222/biliary-atresia-clinical-and-research-challenges-for-the-21-st-century
#8
Jorge A Bezerra, Rebecca G Wells, Cara L Mack, Saul J Karpen, Jay H Hoofnagle, Edward Doo, Ronald J Sokol
Biliary atresia (BA) is a fibroinflammatory disease of the intra- and extrahepatic biliary tree. Without medical treatment, surgical hepatic portoenterosmy (HPE) may restore bile drainage, but progression of the intrahepatic disease results in complications of portal hypertension and advanced cirrhosis in most children. Recognizing that further progress in the field is unlikely without a better understanding of the underlying cause(s) and pathogenesis of the disease, the National Institutes of Diabetes and Digestive and Kidney Diseases sponsored a research workshop focused on innovative and promising approaches and on identifying future areas of research...
March 31, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29599836/acute-kidney-injury-following-the-first-stage-of-the-alpps-procedure-a-case-report
#9
Weijie Tao, Xiaoju Shi, Guangyi Wang
Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) is a novel approach for performing liver resection, and the number of clinical applications of this technique has rapidly increased within recent years. ALPPS is important in patients who have insufficient residual liver volume and cannot undergo radical hepatic resection. The most common postoperative complications of ALPPS include biliary fistula and infection. To date, postoperative acute kidney injury following ALPPS has not been reported...
March 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29593060/the-british-society-of-gastroenterology-uk-pbc-primary-biliary-cholangitis-treatment-and-management-guidelines
#10
Gideon M Hirschfield, Jessica K Dyson, Graeme J M Alexander, Michael H Chapman, Jane Collier, Stefan Hübscher, Imran Patanwala, Stephen P Pereira, Collette Thain, Douglas Thorburn, Dina Tiniakos, Martine Walmsley, George Webster, David E J Jones
Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. Both genetic and environmental influences are presumed relevant to disease initiation. PBC is most prevalent in women and those over the age of 50, but a spectrum of disease is recognised in adult patients globally; male sex, younger age at onset (<45) and advanced disease at presentation are baseline predictors of poorer outcome...
March 28, 2018: Gut
https://www.readbyqxmd.com/read/29579832/characteristics-of-liver-transplantation-in-argentina-a-multicenter-study
#11
L Haddad, S Marciano, M Cleres, A Zerega, F Piñero, F Orozco, G Braslavsky, M Mendizabal, G Gondolesi, O Gil, M Silva, R Mastai, O Imventarza, V Descalzi, A Gadano
INTRODUCTION: There is a lack of information regarding outcomes after liver transplant in Latin America. OBJECTIVES: This study sought to describe outcomes after liver transplant in adult patients from Argentina. METHODS: We performed an ambispective cohort study of adult patients transplanted between June 2010 and October 2012 in 6 centers from Argentina. Only patients who survived after the first 48 hours postransplantation were included...
March 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29577030/eus-guided-gall-bladder-drainage-in-severe-liver-disease-a-single-center-experience-in-critically-ill-cirrhotics
#12
Kapil Dev Jamwal, Manoj Kumar Sharma, Rakhi Maiwall, Barjesh Kumar Sharma, Shiv Kumar Sarin
Background and Aims : Acute calculous cholecystitis with impending gall bladder perforation in severe liver diseases including decompensated cirrhosis and acute-on-chronic liver failure (ACLF) is difficult to manage, due to the procedures such as cholecystectomy and per cutaneous cholecystostomy being associated with high risk and complications in these patients. Methods : Four cases of severe liver disease with acute calculous cholecystitis who presented to the Institute of Liver and Biliary Sciences (New Delhi, India) for further management were included in the study if they underwent endoscopic ultrasound-guided gall bladder drainage (EUS-GBD)...
March 28, 2018: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29574435/autoimmune-pancreatitis-with-concomitant-autoimmune-haemolytic-anaemia
#13
Lauren Passby, Matthew Harris, Ahmed Al-Mukhtar
Autoimmune pancreatitis (AIP) is an infrequent cause of acute pancreatitis, being more commonly associated with chronic pancreatitis. AIP can be associated with other autoimmune manifestations, including Sjögren's, inflammatory bowel disease, primary biliary cirrhosis, rheumatoid arthritis, hypothyroidism and sarcoidosis. Rarely, concurrent autoimmune haemolytic anaemia (AIHA) is observed, as seen in our case report of a 33-year-old postpartum woman.
March 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29554146/evaluation-of-classical-and-novel-autoantibodies-for-the-diagnosis-of-primary-biliary-cholangitis-autoimmune-hepatitis-overlap-syndrome-pbc-aih-os
#14
Henry H Nguyen, Abdel Aziz Shaheen, Natalia Baeza, Ellina Lytvyak, Stefan J Urbanski, Andrew L Mason, Gary L Norman, Marvin J Fritzler, Mark G Swain
BACKGROUND AND AIMS: Up to 20% of Primary Biliary Cholangitis (PBC) patients are estimated to have features that overlap with Autoimmune Hepatitis (AIH). Patients with PBC-AIH overlap syndrome (PBC-AIH OS) have been reported to exhibit suboptimal responses to ursodeoxycholic acid therapy, and are more likely to progress to cirrhosis. Anti-double stranded DNA (anti-dsDNA) and anti-p53 have been previously suggested to be potential autoantibodies for identifying patients with PBC-AIH OS...
2018: PloS One
https://www.readbyqxmd.com/read/29552242/congestive-cirrhosis-in-osler-weber-rendu-syndrome-a-rare-case-report
#15
Patrícia Leitão, André Carvalho, Conceição Guerra, José Gonçalves, Isabel Ramos
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous)...
February 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29551711/magnetic-resonance-cholangiogram-patterns-and-clinical-profiles-of-ketamine-related-cholangiopathy-in-drug-users
#16
Wai-Kay Seto, Siu-King Mak, Keith Chiu, Varut Vardhanabhuti, Ho-Fai Wong, Heng-Tat Leong, Paul S F Lee, Y C Ho, Chi-Kei Lee, Ka-Shing Cheung, Man-Fung Yuen, Wai K Leung
BACKGROUND: Recreational ketamine use has emerged as an important health and social issue worldwide. Although ketamine is associated with biliary tract damage, the clinical and radiological profiles of ketamine-related cholangiopathy have not been well-described. METHODS: We prospectively recruited consecutive Chinese individuals who used ketamine recreationally at least twice per month over 6 months in the past 2 years via a territory-wide community network of charitable organizations tackling substance abuse...
March 15, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29545925/interferon-free-treatment-for-patients-with-chronic-hepatitis-c-and-autoimmune-liver-disease-higher-svr-rates-with-special-precautions-for-deterioration-of-autoimmune-hepatitis
#17
Tatsuo Kanda, Shin Yasui, Masato Nakamura, Shingo Nakamoto, Koji Takahashi, Shuang Wu, Reina Sasaki, Yuki Haga, Sadahisa Ogasawara, Tomoko Saito, Kazufumi Kobayashi, Soichiro Kiyono, Yoshihiko Ooka, Eiichiro Suzuki, Tetsuhiro Chiba, Hitoshi Maruyama, Fumio Imazeki, Mitsuhiko Moriyama, Naoya Kato
Background: Interferon-free treatment can achieve higher sustained virological response (SVR) rates, even in patients in whom hepatitis C virus (HCV) could not be eradicated in the interferon treatment era. Immune restoration in the liver is occasionally associated with HCV infection. We examined the safety and effects of interferon-free regimens on HCV patients with autoimmune liver diseases. Results: All 7 HCV patients with autoimmune hepatitis (AIH) completed treatment and achieved SVR...
February 20, 2018: Oncotarget
https://www.readbyqxmd.com/read/29543880/increased-risk-of-osteoporosis-in-patients-with-primary-biliary-cirrhosis
#18
Chen-Yi Liao, Chi-Hsiang Chung, Pauling Chu, Kuang-Yu Wei, Tseng-Min Feng, Fu-Huang Lin, Chang-Huei Tsao, Chia-Chao Wu, Wu-Chien Chien
BACKGROUND: We evaluated the risk of osteoporosis in patients with primary biliary cirrhosis (PBC) using a nationwide population-based dataset. METHODS: In a cohort study of 986,713 individuals, we selected 2,493 PBC patients who were aged 18 years or older and had been diagnosed with PBC, based on the International Classification of Disease (ICD-9-CM) codes 571.6, during 20002010. The control cohort comprised 9,972 randomly selected, propensity matched patients (by age, gender, and index date), without PBC...
2018: PloS One
https://www.readbyqxmd.com/read/29543694/systematic-review-the-epidemiology-natural-history-and-burden-of-alagille-syndrome
#19
B M Kamath, A Baker, R Houwen, L Todorova, N Kerkar
BACKGROUND: Alagille syndrome (ALGS) is an inherited multisystem disorder typically manifesting as cholestasis, and potentially leading to end-stage liver disease and death. AIM: To perform the first systematic review of the epidemiology, natural history and burden of ALGS with a focus on the liver component. METHODS: Electronic databases and proceedings from key congresses were searched in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 guidelines...
March 14, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29536837/-new-name-and-new-treatments-for-primary-biliary-cholangitits
#20
Lars Bossen, Henriette Ytting, Peter Jepsen, Ole Hamberg, Peter Ott, Henning Grønbæk
The name of chronic liver disease: primary biliary cirrhosis, has been changed to: primary biliary cholangitis, primarily because of the stigma associated with the word "cirrhosis", as only a minority of the patients develop cirrhosis. In this review we present data on epidemiology and discuss the current treatments with focus on ursodeoxycholic acid and the newly described effects of the farnesoid receptor agonist obeticholic acid.
March 5, 2018: Ugeskrift for Laeger
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