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https://www.readbyqxmd.com/read/29606616/direct-conversion-of-mouse-fibroblasts-into-cholangiocyte-progenitor-cells
#1
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...
March 29, 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
#2
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
#3
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...
March 25, 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
#4
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
#5
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/29550924/role-of-lactoferrin-and-its-receptors-on-biliary-epithelium
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29459807/-primary-biliary-cholangitis-part-1-state-of-the-art-epidemiology-physiopathology-and-clinical-manifestations
#11
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...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/29419931/observations-on-the-ex-situ-perfusion-of-livers-for-transplantation
#12
Christopher J E Watson, Vasilis Kosmoliaptsis, Caitlin Pley, Lucy Randle, Corinna Fear, Keziah Crick, Alexander E Gimson, Michael Allison, Sara Upponi, Rebecca Brais, Ina Jochmans, Andrew J Butler
Normothermic ex situ liver perfusion might allow viability assessment of livers before transplantation. Perfusion characteristics were studied in 47 liver perfusions, of which 22 resulted in transplants. Hepatocellular damage was reflected in the perfusate transaminase concentrations, which correlated with post-transplant peak transaminase levels. Lactate clearance occurred within 3 hours in 46 of 47 perfusions, and glucose rose initially during perfusion in 44. Three livers required higher levels of bicarbonate support to maintain physiological pH, including one developing primary non-function...
February 8, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29419536/extracorporeal-membrane-oxygenation-in-a-patient-with-biliary-atresia-case-and-review-of-extracorporeal-life-support-organization-data
#13
Inna N Lobeck, Alexander Bondoc, Heather Nolan, Jason S Frischer, Kathleen M Campbell, Thomas D Ryan, Stuart L Goldstein, Jaimie D Nathan, Maria H Alonso, Greg M Tiao
Biliary atresia is a newborn cholangiopathy that may lead to portopulmonary hypertension and cirrhosis-induced cardiomyopathy while awaiting liver transplantation. Extracorporeal life support and hepatic toxin filtration are life-saving interventions that provide cardiopulmonary support and hepatic dialysis to allow resolution of a child's illness. We utilized a combination of these extreme measures to bridge an infant with biliary atresia to transplantation. We reviewed cases of extracorporeal life support utilization in transplantation recipients in the Extracorporeal Life Support Organization database and determined that ours was the only use of pretransplant extracorporeal life support in biliary atresia...
February 6, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29406331/reduced-immunohistochemical-expression-of-hnf1%C3%AE-and-foxa2-in-liver-tissue-can-discriminate-between-biliary-atresia-and-other-causes-of-neonatal-cholestasis
#14
Usama F Shaalan, Noha L Ibrahim, Nermine A Ehsan, Mervat M Sultan, Ghada M Naser, Mohamed O Abd El-Fatah
Biliary atresia (BA) is a necroinflammatory occlusive cholangiopathy that affects infants. Genetic and environmental factors has been proposed for its occurrence. The objectives of this study was to investigate the protein expression of 2 important genes regulating ductal plate remodeling, hepatocyte nuclear factor 1-beta (Hnf1β) and the fork head box protein A2 (FoxA2) in liver tissue from patients with BA and to compare their expression with other causes of neonatal cholestasis (NC). This retrospective study included 60 pediatric patients, 30 with BA and 30 with NC...
February 5, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29392752/increased-expression-of-senescence-associated-cell-cycle-regulators-in-the-progression-of-biliary-atresia-an-immunohistochemical-study
#15
Motoko Sasaki, Fang-Ying Kuo, Chao-Cheng Huang, Paul E Swanson, Chao-Long Chen, Jiin-Haur Chuang, Matthew M Yeh
AIMS: Cellular senescence plays a role in tumor suppression and in the pathogenesis of various non-neoplastic diseases, including primary biliary cholangitis and other adult cholangiopathies. Less is known about the role of cellular senescence in cholangiopathies in children. With that in mind, we examined the expression of senescence-associated cell cycle regulators in biliary atresia, the most common form of pediatric obliterative cholangiopathy. METHODS AND RESULTS: The expression of s senescence-associated cell cycle regulators (p16Ink4a and p21WAF1/Cip1 ) and a ductular reaction related marker (neural cell adhesion molecule - NCAM), was examined in bile ducts and bile ductules in liver samples taken from the patients with biliary atresia (n=80; including 23 samples at the time of Kasai-procedure (KP) and 63 obtained from the explanted liver (LT) [6 cases with samples at both surgical stages of disease]) and from appropriate controls (n=17)...
February 2, 2018: Histopathology
https://www.readbyqxmd.com/read/29314132/editorial-critical-care-cholangiopathy-rare-but-important
#16
EDITORIAL
A MacGilchrist
No abstract text is available yet for this article.
February 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29312707/rare-causes-of-hyperbilirubinemia-after-lung-transplantation-our-experience-at-a-single-center
#17
Su Hwan Lee, Moo Suk Park, Jin Gu Lee, Joo Han Song, Kyung Soo Chung, Ji Ye Jung, Eun Young Kim, Young Sam Kim, Se Kyu Kim, Joon Chang, Hyo Chae Paik, Song Yee Kim
Background: Lung transplantation is the last treatment option for end-stage lung disease, and the number of lung transplantations has been steadily increasing. Hyperbilirubinemia is a rare complication after lung transplantation. The aim of this study was to review rare causes of hyperbilirubinemia after lung transplantation at our center. Methods: In this single-center study, we retrospectively reviewed the records of 116 consecutive lung transplantation patients who underwent lung transplantation at Severance Hospital and Gangnam Severance Hospital of Yonsei University College of Medicine in South Korea between December 22, 2010 and January 1, 2016...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29248458/hepatic-stem-progenitor-cell-activation-differs-between-primary-sclerosing-and-primary-biliary-cholangitis
#18
Guido Carpino, Vincenzo Cardinale, Trine Folseraas, Diletta Overi, Annarosa Floreani, Antonio Franchitto, Paolo Onori, Nora Cazzagon, Pasquale B Berloco, Tom H Karlsen, Domenico Alvaro, Eugenio Gaudio
Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are human primary cholangiopathies characterized by the damage of mature cholangiocytes and by the appearance of ductular reaction (DR) as the results of hepatic progenitor cell activation. This study evaluated the differences in progenitor cell niche activation between these two cholangiopathies. Liver tissue was obtained from healthy liver donors (n = 5) and from patients with PSC (n = 20) or PBC (n = 20). DR, progenitor cell phenotype, and signaling pathways were investigated by IHC analysis and immunofluorescence...
March 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29234198/long-term-outcomes-of-patients-with-significant-biliary-obstruction-due-to-portal-cavernoma-cholangiopathy-and-extra-hepatic-portal-vein-obstruction-ehpvo-with-no-shuntable-veins
#19
Akash Shukla, Amit Gupte, Vedant Karvir, Prashant Dhore, Shobna Bhatia
Aims: The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC. Methods: Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years...
December 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29212771/establishment-of-a-surgical-bile-duct-injection-technique-giving-direct-access-to-the-bile-ducts-for-studies-of-the-murine-biliary-tree
#20
Natalie Lie Berntsen, Bjarte Fosby, Laura Valestrand, Corey Tan, Henrik M Reims, Elisabeth Schrumpf, Tom Hemming Karlsen, Pål-Dag Line, Espen Melum
Cholangiopathies are progressive disorders with largely unknown pathoetiology and limited treatment options. We aimed to develop a novel surgical technique with direct access to the bile ducts that would complement existing mouse models of cholestasis, biliary inflammation, and fibrosis and present a new route of administration for testing of potential treatment strategies. We developed a surgical technique to access the murine biliary tree by injection of different solvents through catheterization of the gall bladder with simultaneous clamping of the common bile duct...
March 1, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
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