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https://www.readbyqxmd.com/read/29909604/upregulation-of-high-mobility-group-box-1-may-contribute-to-the-pathogenesis-of-biliary-atresia
#1
Chun Jing Ye, Jiang Wang, Yi Fan Yang, Zhen Shen, Gong Chen, Yan Lei Huang, Yi Jie Zheng, Rui Dong, Shan Zheng
OBJECTIVES:  Biliary atresia (BA) is a progressive inflammatory obstructive cholangiopathy in infants. High mobility group box 1 (HMGB1) is known to play an important role as a late mediator of inflammation. However, it is not clear whether HMGB1 levels are of clinical significance in patients with BA. The aim of this study was to determine correlations between serum HMGB1 levels and the clinicopathologic features of BA. MATERIALS AND METHODS:  Serum samples were collected from 19 infants with BA, 7 infants with anicteric choledochal cysts (CC) and normal liver function, and 8 healthy controls...
June 17, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29791424/-primary-biliary-cholangitis-part-2-state-of-the-art-diagnosis-associated-diseases-treatment-and-prognosis
#2
Diego Andrés Rodríguez Lugo, Jorge Julián Coronado Tovar, Giovana Alejandra Solano Villamarin, Wiliam Otero Regino
Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others...
January 2018: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/29751790/obliterative-cholangiopathy-in-acquired-cystic-biliary-atresia-type-iii-after-cyst-perforation-a-case-report
#3
Tsugumichi Koshinaga, Kensuke Ohashi, Kakou Ono, Hide Kaneda, Takeshi Furuya
BACKGROUND: In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration. This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth. CASE PRESENTATION: An 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage...
May 11, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29742944/subcentimetric-incidental-intrahepatic-cholangiocarcinoma-in-an-explant-liver-diagnostic-difficulty-of-a-rare-entity
#4
Suvradeep Mitra, Gude Geethanjali, Sahaj Rathi, Arunanshu Behera, Ashim Das
Incidental intrahepatic cholangiocarcinoma (iCCA) is a rare neoplastic lesion in the explant liver specimens with an approximate incidence of 0.7%. The detection of iCCA is associated with poor prognosis in the posttransplant setting. The occurrence of a subcentimetric iCCA is very rare and poses a major diagnostic challenge to the pathologist. This article presents a rare case of subcentimetric iCCA in a young male in the background of advanced stage chronic liver disease resulting from autoimmune hepatitis possibly with chronic cholangiopathy along with the histomorphological differentials...
May 1, 2018: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29709678/lysyl-oxidase-like-protein-2-loxl2-modulates-barrier-function-in-cholangiocytes-in-cholestasis
#5
Marion J Pollheimer, Silvia Racedo, Amanda Mikels-Vigdal, Derek Marshall, Christopher Bowlus, Carolin Lackner, Tobias Madl, Tom H Karlsen, Johannes R Hov, Susan K Lyman, Joanne Adamkewicz, Victoria Smith, Emmanuel Moreau, Gernot Zollner, Tor Jacob Eide, Tatjana Stojakovic, Hubert Scharnagl, Hans-Jürgen Gruber, Rudolf E Stauber, Michael Trauner, Peter Fickert
BACKGROUND & AIMS: The lysyl oxidase-like protein 2 (LOXL2) promotes stabilization of the extracellular matrix, chemotaxis, cell growth and cell mobility. We aimed to (i) identify stimuli of LOXL2 in cholangiopathies, (ii) characterize the effects of LOXL2 on biliary epithelial cells' (BECs) barrier function, (iii) compare LOXL2 expression in primary sclerosing cholangitis (PSC), primary biliary cholangitis, and disease controls, and (iv) to determine LOXL2 expression and its cellular sources in four mouse models of cholangiopathies...
April 28, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29707809/hepatobiliary-and-pancreatic-nivolumab-related-cholangiopathy
#6
C Hamoir, M de Vos, F Clinckart, G Nicaise, M Komuta, N Lanthier
No abstract text is available yet for this article.
April 29, 2018: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29707407/variants-associated-with-infantile-cholestatic-syndromes-detected-in-extrahepatic-biliary-atresia-by-whole-exome-studies-a-20-case-series-from-thailand
#7
Surasak Sangkhathat, Wison Laochareonsuk, Wanwisa Maneechay, Kanita Kayasut, Piyawan Chiengkriwate
Biliary atresia (BA) is the most severe form of obstructive cholangiopathy occurring in infants. Definitive diagnosis of BA usually relies on operative findings together with supporting pathological patterns found in the extrahepatic bile duct. In infancy, overlapping clinical patterns of cholestasis can be found in other diseases including biliary hypoplasia and progressive familial intrahepatic cholestasis. In addition, BA has been reported as a phenotype in some rare genetic syndromes. Unlike BA, other cholangiopathic phenotypes have their own established genetic markers...
June 2018: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/29707074/hepatobiliary-manifestations-and-complications-in-inflammatory-bowel-disease-a-review
#8
REVIEW
Fotios S Fousekis, Vasileios I Theopistos, Konstantinos H Katsanos, Epameinondas V Tsianos, Dimitrios K Christodoulou
Liver and biliary track diseases are common extraintestinal manifestations of inflammatory bowel disease (IBD), reported both in Crohn's disease and ulcerative colitis, and may occur at any time during the natural course of the disease. Their etiology is mainly related to pathophysiological changes induced by IBD, and secondary, due to drugs used in IBD. Fatty liver is considered as the most frequent hepatobiliary manifestation in IBD, while primary sclerosing cholangitis (PSC) is the most correlated hepatobiliary disorder and is more prevalent in patients with ulcerative colitis...
April 2018: Gastroenterology Research
https://www.readbyqxmd.com/read/29686325/pivotal-roles-of-kupffer-cells-in-the-progression-and-regression-of-ddc-induced-chronic-cholangiopathy
#9
Leila Jemail, Masashi Miyao, Hirokazu Kotani, Chihiro Kawai, Hirozo Minami, Hitoshi Abiru, Keiji Tamaki
Kupffer cells (KCs) are key players in maintaining tissue homeostasis and are involved in various liver diseases. However, the roles of KCs in the pathogenesis of cholangiopathy are largely unknown. We aimed to investigate the precise roles of KCs in both the progression and regression phases of the 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC)-induced cholangiopathy model. In the early phase of DDC-induced cholangiopathy, the number of KCs significantly increased over time. Moreover, KCs were associated with abnormal phenotypic changes in other liver cells, such as hepatocytes, biliary epithelial cells, liver sinusoidal endothelial cells, and hepatic stellate cells...
April 23, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29679373/cytokine-producing-b-cells-promote-immune-mediated-bile-duct-injury-in-murine-biliary-atresia
#10
Joseph Bednarek, Brianna Traxinger, Dania Brigham, Jonathan Roach, David Orlicky, Dong Wang, Roberta Pelanda, Cara L Mack
Biliary atresia (BA) is a neonatal T cell-mediated inflammatory, sclerosing cholangiopathy. In the Rhesus rotavirus (RRV)-induced neonatal mouse model of BA (murine BA), mice lacking B cells do not develop BA, and the lack of B cells is associated with loss of T cell and macrophage activation. The aim of this study was to determine the mechanism of B cell-mediated immune activation (antigen presentation versus cytokine production) in murine BA. RESULTS: Normal neonatal B cells in the liver are predominantly at pro-B and pre-B cellular development...
April 20, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29606616/direct-conversion-of-mouse-fibroblasts-into-cholangiocyte-progenitor-cells
#11
Kyung Tae Lim, Jonghun Kim, Seon In Hwang, Ludi Zhang, Heonjong Han, Dasom Bae, Kee-Pyo Kim, Yi-Ping Hu, Hans R Schöler, Insuk Lee, Lijian Hui, Dong Wook Han
Disorders of the biliary epithelium, known as cholangiopathies, cause severe and irreversible liver diseases. The limited accessibility of bile duct precludes modeling of several cholangiocyte-mediated diseases. Therefore, novel approaches for obtaining functional cholangiocytes with high purity are needed. Previous work has shown that the combination of Hnf1β and Foxa3 could directly convert mouse fibroblasts into bipotential hepatic stem cell-like cells, termed iHepSCs. However, the efficiency of converting fibroblasts into iHepSCs is low, and these iHepSCs exhibit extremely low differentiation potential into cholangiocytes, thus hindering the translation of iHepSCs to the clinic...
May 8, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/29579871/analysis-of-ischemic-cholangiopathy-after-treatment-of-arterial-thrombosis-in-liver-transplantation-in-our-series
#12
M Dominguez Bastante, A Molina Raya, A Vilchez Rabelo, J Villar Del Moral, M Exposito Ruiz, Y Fundora Suarez
Liver transplantation offers patients with terminal liver disease an effective treatment accompanied by excellent quality of life, but it also has complications, such as hepatic artery thrombosis and development of ischemic cholangiopathy, described in 3%-17% of patients. It is a very important cause of morbidity and mortality. The objective of this report was to analyze the efficacy of the treatment they received in relation to the development of ischemic cholangiopathy and a comparative survival analysis and to propose prophylactic measures for high-risk patients...
March 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29575286/planar-xanthomas-secondary-to-post-transplantation-cholangiopathy-in-a-16-month-old-boy
#13
Nirali Patel, Adam Norberg, Marcia Hogeling
Planar xanthomas in children represent rare dermatologic findings associated with abnormalities in lipid metabolism. While planar xanthomas in Alagille's syndrome have been well described in the literature, there have been no cases reported of eruptive xanthomas in pediatric liver transplant patients. Herein we report a case of a 16-month-old boy status post-liver transplantation who presents with planar xanthomas secondary to cholangiopathy. A brief review of xanthomas and the related literature is also provided...
May 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29564205/from-gut-feeling-to-objectivity-machine-preservation-of-the-liver-as-a-tool-to-assess-organ-viability
#14
REVIEW
Christopher J E Watson, Ina Jochmans
Purpose of Review: The purpose of this review was to summarise how machine perfusion could contribute to viability assessment of donor livers. Recent Findings: In both hypothermic and normothermic machine perfusion, perfusate transaminase measurement has allowed pretransplant assessment of hepatocellular damage. Hypothermic perfusion permits transplantation of marginal grafts but as yet has not permitted formal viability assessment. Livers undergoing normothermic perfusion have been investigated using parameters similar to those used to evaluate the liver in vivo...
2018: Current Transplantation Reports
https://www.readbyqxmd.com/read/29551711/magnetic-resonance-cholangiogram-patterns-and-clinical-profiles-of-ketamine-related-cholangiopathy-in-drug-users
#15
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 & AIMS: 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: Chinese individuals who had used ketamine recreationally at least twice per month for six months in the previous two years via a territory-wide community network of charitable organizations tackling substance abuse were recruited...
March 16, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29550924/role-of-lactoferrin-and-its-receptors-on-biliary-epithelium
#16
Romina Mancinelli, Francesca Olivero, Guido Carpino, Diletta Overi, Luigi Rosa, Maria Stefania Lepanto, Antimo Cutone, Antonio Franchitto, Gianfranco Alpini, Paolo Onori, Piera Valenti, Eugenio Gaudio
Human lactoferrin is an iron-binding glycoprotein present at high concentrations in breast milk and colostrum. It is produced by many exocrine glands and widely distributed in a variety of body fluids. This protein has antimicrobial, immunomodulatory, antioxidant, and anticancer properties. Two important hLf receptors have been identified: LDL receptor related protein (LRP1), a low specificity receptor, and intelectin-1 (ITLN1), a high specificity receptor. No data are present on the role of hLf on the biliary epithelium...
March 17, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/29523787/paracrine-cellular-senescence-exacerbates-biliary-injury-and-impairs-regeneration
#17
Sofia Ferreira-Gonzalez, Wei-Yu Lu, Alexander Raven, Benjamin Dwyer, Tak Yung Man, Eoghan O'Duibhir, Philip J Starkey Lewis, Lara Campana, Tim J Kendall, Thomas G Bird, Nuria Tarrats, Juan-Carlos Acosta, Luke Boulter, Stuart J Forbes
Cellular senescence is a mechanism that provides an irreversible barrier to cell cycle progression to prevent undesired proliferation. However, under pathological circumstances, senescence can adversely affect organ function, viability and regeneration. We have developed a mouse model of biliary senescence, based on the conditional deletion of Mdm2 in bile ducts under the control of the Krt19 promoter, that exhibits features of biliary disease. Here we report that senescent cholangiocytes induce profound alterations in the cellular and signalling microenvironment, with recruitment of myofibroblasts and macrophages causing collagen deposition, TGFβ production and induction of senescence in surrounding cholangiocytes and hepatocytes...
March 9, 2018: Nature Communications
https://www.readbyqxmd.com/read/29467548/epidemiology-determinants-and-management-of-aids-cholangiopathy-a-review
#18
REVIEW
Maliha Naseer, Francis E Dailey, Alhareth Al Juboori, Sami Samiullah, Veysel Tahan
Diseases of the liver and biliary tree have been described with significant frequency among patients with human immunodeficiency virus (HIV), and its advanced state, acquired immunodeficiency syndrome (AIDS). Through a variety of mechanisms, HIV/AIDS has been shown to affect the hepatic parenchyma and biliary tree, leading to liver inflammation and biliary strictures. One of the potential hepatobiliary complications of this viral infection is AIDS cholangiopathy, a syndrome of biliary obstruction and liver damage due to infection-related strictures of the biliary tract...
February 21, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29464971/inflammatory-nodules-identify-steroid-responsive-primary-sclerosing-cholangitis
#19
Jonathan H Chen, Vikram Deshpande
Primary sclerosing cholangitis (PSC) is a cholangiopathy-usually associated with inflammatory bowel disease-that leads to cirrhosis and liver failure. Based on a multitude of clinical trials, there is general consensus that PSC progression is unchanged by current therapies, including steroids. However, there are scattered reports in the literature of PSC patients responsive to steroids. Recently, several steroid-responsive PSC mimics have been described, most notably immunoglobulin G4-related sclerosing cholangitis...
February 1, 2018: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29464203/rituximab-induction-to-prevent-the-recurrence-of-psc-after-liver-transplantation-the-lessons-learned-from-abo-incompatible-living-donor-liver-transplantation
#20
Yohei Yamada, Ken Hoshino, Yasushi Fuchimoto, Kentaro Matsubara, Taizo Hibi, Hiroshi Yagi, Yuta Abe, Masahiro Shinoda, Minoru Kitago, Hideaki Obara, Takahito Yagi, Hideaki Okajima, Toshimi Kaido, Shinji Uemoto, Tatsuya Suzuki, Keiichi Kubota, Tomoharu Yoshizumi, Yoshihiko Maehara, Yukihiro Inomata, Yuko Kitagawa, Hiroto Egawa, Tatsuo Kuroda
Background: Multiple studies have failed to reveal an effective method for preventing the recurrence of primary sclerosing cholangitis (PSC) after liver transplantation (LTx). A national study conducted in Japan revealed several risk factors for the recurrence after living donor LTx (LDLTx); however, recipients of ABO-blood type incompatible (ABO-I) LTx were excluded from the previous analysis. In the present study, we investigated the efficacy of an immunosuppressive protocol in ABO-I LTx on the recurrence of PSC after LDLTx...
February 2018: Transplantation Direct
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