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https://www.readbyqxmd.com/read/29017709/prognostic-scores-and-non-invasive-markers-in-primary-sclerosing-cholangitis-good-for-patients-or-for-papers
#1
Douglas Thorburn
No abstract text is available yet for this article.
November 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28993983/skin-manifestations-associated-with-autoimmune-liver-diseases-a-systematic-review
#2
REVIEW
Benedetta Terziroli Beretta-Piccoli, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum...
October 9, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28993258/twenty-year-comparative-analysis-of-patients-with-autoimmune-liver-diseases-on-transplant-waitlists
#3
G J Webb, A Rana, J Hodson, M Z Akhtar, J W Ferguson, J M Neuberger, J M Vierling, G M Hirschfield
BACKGROUND & AIMS: The rarity of autoimmune liver disease poses challenges to epidemiology studies. However, waitlists for liver transplantation can be used to study patients with end-stage liver diseases. We used these waitlists to assess trends in numbers and demographics patients awaiting liver transplant for primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), or autoimmune hepatitis (AIH). METHODS: We collected data from United Kingdom (UK) and United States (US) national registries for all adults on liver transplant waitlists, from January 1, 1995 through December 31, 2014...
October 6, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28990806/optimizing-the-detection-of-biliary-dysplasia-in-primary-sclerosing-cholangitis-before-liver-transplantation
#4
Ammar Majeed, Maria Castedal, Urban Arnelo, Gunnar Söderdahl, Annika Bergquist, Karouk Said
BACKGROUND: Patients with primary sclerosing cholangitis (PSC) have increased risk of cholangiocarcinoma (CCA). We evaluated pre-transplant work-up in PSC patients, to search for the most effective strategy for the detection of biliary dysplasia or early CCA. METHODS: Two hundred and twenty five consecutive PSC patients undergoing liver transplantation (LTx) in Sweden between 1999 and 2013 were studied. Patients with CCA or dysplasia in the explanted liver were compared with those with benign histopathology...
October 9, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28987259/primary-sclerosing-cholangitis-what-the-gastroenterologist-and-hepatologist-needs-to-know
#5
REVIEW
Andrea A Gossard, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic biliary tract disease characterized by segmental strictures. The disease is progressive with no proven treatments and may eventually lead to cirrhosis and end-stage liver disease. Abrupt changes in liver biochemistries, pain, and/or cholangitis may suggest a dominant stricture amenable to endoscopic therapy or the development of cholangiocarcinoma. Patients with PSC are at increased risk of cholangiocarcinoma. There is a strong association with inflammatory bowel disease, and an associated increased risk of colorectal cancer...
November 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28981638/long-term-risk-of-cancer-following-ileal-pouch-anal-anastomosis-for-ulcerative-colitis
#6
Anders Mark-Christensen, Rune Erichsen, Søren Brandsborg, Jacob Rosenberg, Niels Qvist, Ole Thorlacius-Ussing, Jens Hillingsø, Jørn Helmut Pachler, Erica Gould Christiansen, Søren Laurberg
Background: The overall risk of cancer following ileal pouch-anal anastomosis [IPAA] is unknown, and pouch cancer surveillance is controversial. We evaluated long-term risk of cancer in a national cohort of patients with ulcerative colitis and IPAA, with emphasis on pouch cancer. Methods: Data on incident cancers were extracted from the national Danish Cancer Registry. Incidence rates for all site-specific cancers were compared between patients with IPAA and a gender- and age-matched comparison cohort from the background population to obtain incidence rate ratios [IRRs]...
August 3, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#7
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28970730/suspicious-brush-cytology-is-an-indication-for-liver-transplantation-evaluation-in-primary-sclerosing-cholangitis
#8
Sonja Boyd, Marko Vannas, Kalle Jokelainen, Helena Isoniemi, Heikki Mäkisalo, Martti A Färkkilä, Johanna Arola
AIM: To investigate markers for high-grade dysplasia for the optimal timing of liver transplantation in patients with primary sclerosing cholangitis (PSC). METHODS: Earlier data support a dysplasia-carcinoma sequence, even low- to high-grade dysplasia, in PSC-associated cholangiocarcinoma (CCA). Surveillance using endoscopic retrograde cholangiography (ERC) and brush cytology aims to detect cases of biliary dysplasia, and liver transplantation is an option in cases with suspicion of malignancy in brushing...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28970729/patients-with-inflammatory-bowel-disease-have-increased-risk-of-autoimmune-and-inflammatory-diseases
#9
Morten L Halling, Jens Kjeldsen, Torben Knudsen, Jan Nielsen, Lars Koch Hansen
AIM: To investigate whether immune mediated diseases (IMD) are more frequent in patients with inflammatory bowel disease (IBD). METHODS: In this population based registry study, a total of 47325 patients with IBD were alive and registered in the Danish National Patient Registry on December 16, 2013. Controls were randomly selected from the Danish Civil Registration System (CRS) and matched for sex, age, and municipality. We used ICD 10 codes to identify the diagnoses of the included patients...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28970141/primary-sclerosing-cholangitis-is-protective-against-non-alcoholic-fatty-liver-disease-in-inflammatory-bowel-disease
#10
Dustin E Bosch, Matthew M Yeh
Non-alcoholic fatty liver disease (NAFLD) occurs with higher prevalence in patients with inflammatory bowel disease (IBD) relative to the general population, and susceptibility is related to the metabolic syndrome, as well as higher prevalence of bowel resection and gut microbiotal factors. Liver complications, including NAFLD and primary sclerosing cholangitis (PSC) contribute to treatment and prognosis of patients with IBD. However, the potential interplay of NAFLD and PSC is not well understood. We retrospectively assessed severity of steatosis and steatohepatitis in liver specimens from 49 patients with IBD only, 44 with IBD and co-morbid PSC, and 30 with IBD and PSC after liver transplantation...
September 29, 2017: Human Pathology
https://www.readbyqxmd.com/read/28965148/heterogeneity-of-outcomes-following-liver-transplantation-for-primary-sclerosing-cholangitis-age-matters
#11
EDITORIAL
Paul Martin, Keith D Lindor
No abstract text is available yet for this article.
September 30, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28960437/safety-and-efficacy-of-biologic-agents-for-the-management-of-inflammatory-bowel-disease-after-liver-transplantation
#12
Suhail A Shaikh, Linda Stuckey, Sarah Tischer
Primary sclerosing cholangitis (PSC) frequently progresses to end-stage liver disease and cirrhosis, requiring liver transplantation. Approximately 70% of patients with PSC have concomitant inflammatory bowel disease (IBD) during their clinical course. After liver transplantation for PSC, corticosteroids and other high-intensity immunosuppressants are initiated to keep IBD in remission. Patients with IBD that is refractory to these agents may need to be managed with biologic therapies. Biologic agents, however, may further increase the risks for malignancy and infection due their immunosuppressive effects...
September 27, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28951947/cholangiocarcinoma-and-its-mimickers-in-primary-sclerosing-cholangitis
#13
REVIEW
Jonghun John Lee, Sebastian T Schindera, Hyun-Jung Jang, Scott Fung, Tae Kyoung Kim
Cholangiocarcinoma (CCA) is the most common malignancy in primary sclerosing cholangitis (PSC). Approximately half of CCA are diagnosed within two years of initial diagnosis and often have a poor prognosis because of advanced tumor stage at the time of diagnosis. Thus, rigorous initial imaging evaluation for detecting CCA is important. CCA in PSC usually manifests as intrahepatic mass-forming or perihilar periductal-infiltrating type. Imaging diagnosis is often challenging due to pre-existing biliary strictures and heterogeneous liver...
September 26, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28951512/efficacy-of-oral-vancomycin-in-recurrent-primary-sclerosing-cholangitis-following-liver-transplantation
#14
Penelope Hey, Julie Lokan, Paul Johnson, Paul Gow
Primary sclerosing cholangitis (PSC) is a liver disease that leads to progressive destruction and stricturing of the biliary tree. Unfortunately, apart from orthotopic liver transplantation (OLT), there are no universally accepted therapies to treat this disease. Even following transplantation, recurrence of PSC is seen in approximately one quarter of patients and leads to high rates of graft failure. Oral vancomycin, through possible immunomodulatory and anti-inflammatory mechanisms, has been shown in small-scale studies to be successful in improving liver function tests in patients with pretransplant PSC...
September 25, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28951510/increased-cholestatic-enzymes-in-two-patients-with-long-term-history-of-ulcerative-colitis-consider-primary-biliary-cholangitis-not-always-primary-sclerosing-cholangitis
#15
Erietta Polychronopoulou, Vasiliki Lygoura, Nikolaos K Gatselis, George N Dalekos
Several hepatobiliary disorders have been reported in ulcerative colitis (UC) patients with primary sclerosing cholangitis (PSC) being the most specific. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, rarely occurs in UC. We present two PBC cases of 67 and 71 years who suffered from long-standing UC. Both patients were asymptomatic but they had increased cholestatic enzymes and high titres of antimitochondrial antibodies (AMA)-the laboratory hallmark of PBC. After careful exclusion of other causes of cholestasis by MRI/magnetic resonance cholangiopancreatography (MRCP), virological and microbiological investigations, a diagnosis of PBC associated with UC was established...
September 25, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28943450/pathological-features-of-primary-sclerosing-cholangitis-identified-by-bile-proteomic-analysis
#16
C Rupp, K A Bode, Y Leopold, P Sauer, D N Gotthardt
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Previous bile proteomic analyses in patients with PSC have revealed changes in disease activity specific to malignant transformation. In this study, we established a reference bile duct-derived bile proteome for PSC that can be used to evaluate biliary pathophysiology. Samples were collected from patients with PSC or with choledocholithiasis (control) (n=6 each). Furthermore, patients with PSC-associated cholangiocarcinoma (CC) and with CC without concomitant PSC were analyzed...
September 22, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28926118/chop-induced-loss-of-intestinal-epithelial-stemness-contributes-to-bile-duct-ligation-induced-cholestatic-liver-injury
#17
Runping Liu, Xiaojiaoyang Li, Zhiming Huang, Derrick Zhao, Bhagyalaxmi Sukka Ganesh, Guanhua Lai, William M Pandak, Phillip B Hylemon, Jasmohan S Bajaj, Arun J Sanyal, Huiping Zhou
Impaired intestinal barrier function promotes the progression of various liver diseases including cholestatic liver disease. The close association of primary sclerosing cholangitis (PSC) with inflammatory bowel disease highlights the importance of the gut-liver axis. It has been reported that bile duct ligation (BDL)-induced liver fibrosis is significantly reduced in C/EBP homologous protein knock out (CHOP(-/-) ) mice. However, the underlying mechanisms remain unclear. In the current study, we demonstrate that BDL induces striking and acute hepatic ER stress responses after 1 day, which return to normal after 3 days...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28921801/complications-symptoms-quality-of-life-and-pregnancy-in-cholestatic-liver-disease
#18
Kais Zakharia, Anilga Tabibian, Keith D Lindor, James H Tabibian
Cholestatic liver diseases (CLDs) encompass a variety of disorders of bile formation and/or flow which generally result in progressive hepatobiliary injury and ultimately end-stage liver disease. Many patients with CLD are diagnosed between the ages of 20-50 years, a particularly productive period of life professionally, biologically, and in other respects; it is not surprising, thus, that CLD is often associated with impaired health-related quality of life (HRQOL) and uncertainty regarding implications for and outcomes of pregnancy...
September 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#19
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#20
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
November 2017: Clinical Rheumatology
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