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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
#1
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
#2
Natalie Lie Berntsen, Bjarte Fosby, Laura Valestrand, Corey Tan, Henrik Reims, Elisabeth Schrumpf, Tom H 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...
December 6, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/29167395/large-scale-proteomics-identifies-mmp-7-as-a-sentinel-of-epithelial-injury-and-of-biliary-atresia
#3
Chatmanee Lertudomphonwanit, Reena Mourya, Lin Fei, Yue Zhang, Sridevi Gutta, Li Yang, Kevin E Bove, Pranavkumar Shivakumar, Jorge A Bezerra
Biliary atresia is a progressive infantile cholangiopathy of complex pathogenesis. Although early diagnosis and surgery are the best predictors of treatment response, current diagnostic approaches are imprecise and time-consuming. We used large-scale, quantitative serum proteomics at the time of diagnosis of biliary atresia and other cholestatic syndromes (serving as disease controls) to identify biomarkers of disease. In a discovery cohort of 70 subjects, the lead biomarker was matrix metalloproteinase-7 (MMP-7), which retained high distinguishing features for biliary atresia in two validation cohorts...
November 22, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29167218/concomitant-aids-cholangiopathy-and-fanconi-syndrome-as-complications-of-hiv-in-a-single-patient
#4
Robert Maweni, Jins Kallampallil, Szewai Leong, Srikanth Akunuri
We describe the case of a 50-year-old woman presenting to our acute medicine department with generalised non-specific symptoms on a background of HIV managed on triple therapy (tenofovir, lamivudine and zidovudine). On admission, she was noted to be acidotic with proteinuria, glycosuria, hypophosphataemia and generalised body pain, and was diagnosed with Fanconi's renotubular syndrome secondary to tenofovir. It was also noted that she had elevated liver dysfunction markers, and an MRI of the liver revealed a focal stricture near the ampulla of Vater, resulting in a diagnosis of AIDS cholangiopathy...
November 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#5
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/29158418/a-novel-pkhd1-mutation-interacts-with-the-nonobese-1diabetic-genetic-background-to-cause-autoimmune-cholangitis
#6
Wenting Huang, Daniel B Rainbow, Yuehong Wu, David Adams, Pranavkumar Shivakumar, Leah Kottyan, Rebekah Karns, Bruce Aronow, Jorge Bezerra, M Eric Gershwin, Laurence B Peterson, Linda S Wicker, William M Ridgway
We previously reported that NOD.c3c4 mice develop spontaneous autoimmune biliary disease (ABD) with anti-mitochondrial Abs, histopathological lesions, and autoimmune T lymphocytes similar to human primary biliary cholangitis. In this article, we demonstrate that ABD in NOD.c3c4 and related NOD ABD strains is caused by a chromosome 1 region that includes a novel mutation in polycystic kidney and hepatic disease 1 (Pkhd1). We show that a long terminal repeat element inserted into intron 35 exposes an alternative polyadenylation site, resulting in a truncated Pkhd1 transcript...
November 20, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29156507/liver-transplantation-in-adults-with-liver-disease-due-to-common-variable-immunodeficiency-leads-to-early-recurrent-disease-and-poor-outcome
#7
Vian Azzu, Joshua E Elias, Adam Duckworth, Susan Davies, Rebecca Brais, Dinakantha S Kumararatne, Alexander E S Gimson, William J H Griffiths
Common variable immunodeficiency (CVID) is the commonest from of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features such as nodular regenerative hyperplasia, inflammation, fibrosis and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence...
November 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29155020/the-uk-dcd-risk-score-a-new-proposal-to-define-futility-in-donation-after-circulatory-death-liver-transplantation
#8
Andrea Schlegel, Marit Kalisvaart, Irene Scalera, Richard W Laing, Hynek Mergental, Darius F Mirza, Thamara Perera, John Isaac, Philipp Dutkowski, Paolo Muiesan
OBJECTIVES: To design a new score on risk assessment in liver transplantation donated after circulatory death (DCD) based on donor and recipient parameters. BACKGROUND: Primary-non-function (PNF) and ischemic cholangiopathy (IC) are the most feared complications following DCD liver transplantation. METHODS: Using the United Kingdom (UK) national DCD database, a risk analysis was performed in adult recipients of DCD liver grafts in UK between 2000 and 2015 (n=1153)...
November 15, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29140985/notch-signaling-and-progenitor-ductular-reaction-in-steatohepatitis
#9
Carola M Morell, Romina Fiorotto, Marica Meroni, Aileen Raizner, Barbara Torsello, Massimiliano Cadamuro, Gaia Spagnuolo, Eleanna Kaffe, Salvatore Sutti, Emanuele Albano, Mario Strazzabosco
BACKGROUND AND OBJECTIVE: Persistent hepatic progenitor cells (HPC) activation resulting in ductular reaction (DR) is responsible for pathologic liver repair in cholangiopathies. Also, HPC/DR expansion correlates with fibrosis in several chronic liver diseases, including steatohepatitis. Increasing evidence indicates Notch signaling as a key regulator of HPC/DR response in biliary and more in general liver injuries. Therefore, we aimed to investigate the role of Notch during HPC/DR activation in a mouse model of steatohepatitis...
2017: PloS One
https://www.readbyqxmd.com/read/29135531/recurrent-subanesthetic-ketamine-infusions-for-complex-regional-pain-syndrome-leading-to-biliary-dilation-jaundice-and-cholangitis-a-case-report
#10
Nathan A Hewitt, Peter Cox
Complex regional pain syndrome is a chronic pain condition that may be resistant to many treatment modalities. Ketamine infusions have demonstrated some promising results, though their use may be associated with a number of adverse effects limiting their widespread applicability. Hepatotoxicity and cholangiopathy have been described in chronic ketamine abuse, though rarely in therapeutic use. We report the impact of recurrent short subanesthetic ketamine infusions for the treatment of complex regional pain syndrome resulting in biliary dilation, jaundice, and cholangitis...
November 9, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/29108514/hypothermic-oxygenated-machine-perfusion-for-liver-transplantation-an-initial-experience
#11
Damiano Patrono, Bruna Lavezzo, Luca Molinaro, Giorgia Rizza, Giorgia Catalano, Federica Gonella, Mauro Salizzoni, Renato Romagnoli
OBJECTIVES: Due to widespread exploitation of extended criteria donors, machine perfusion is emerging as an alternative to static cold storage for organ preservation. Hypothermic oxygenated machine perfusion has been associated with improved outcomes after liver transplant, both in laboratory and clinical settings. Here, we present our initial experience with hypothermic oxygenated machine perfusion, evaluating incidence of postreperfusion syndrome, early allograft dysfunction, and long-term biliary complications...
October 31, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29098121/using-zebrafish-to-model-liver-diseases-where-do-we-stand
#12
Duc-Hung Pham, Changwen Zhang, Chunyue Yin
Purpose of Review: The liver is the largest internal organ and performs both exocrine and endocrine function that is necessary for survival. Liver failure is among the leading causes of death and represents a major global health burden. Liver transplantation is the only effective treatment for end-stage liver diseases. Animal models advance our understanding of liver disease etiology and hold promise for the development of alternative therapies. Zebrafish has become an increasingly popular system for modeling liver diseases and complements the rodent models...
June 2017: Current Pathobiology Reports
https://www.readbyqxmd.com/read/29097260/advances-in-the-generation-of-bioengineered-bile-ducts
#13
REVIEW
Alexander W Justin, Kourosh Saeb-Parsy, Athina E Markaki, Ludovic Vallier, Fotios Sampaziotis
The generation of bioengineered biliary tissue could contribute to the management of some of the most impactful cholangiopathies associated with liver transplantation, such as biliary atresia or ischemic cholangiopathy. Recent advances in tissue engineering and in vitro cholangiocyte culture have made the achievement of this goal possible. Here we provide an overview of these developments and review the progress towards the generation and transplantation of bioengineered bile ducts.
October 30, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29070776/ccl5-is-induced-by-tlr-3-signaling-in-hucct1-human-biliary-epithelial-cells-possible-involvement-in-the-pathogenesis-of-biliary-atresia
#14
Taku Shimada, Tadaatsu Imaizumi, Kyogo Shirai, Tetsuya Tatsuta, Toshiro Kimura, Ryo Hayakari, Hidemi Yoshida, Tomoh Matsumiya, Hiroshi Kijima, Hiroki Mizukami, Kenichi Hakamada
Biliary atresia (BA) is a disease of the newborn that is characterized by progressive, inflammatory and sclerosing cholangiopathy. Innate immune responses to viral components are thought to be involved in the pathogenesis of BA. It is also reported that some chemokines, such as CCL5, are possibly involved in the pathogenesis of experimental animal model of BA. We treated human biliary epithelial HuCCT1 cells with polyinosinic-polycytidylic acid (poly IC), an authentic double-stranded RNA (dsRNA) which mimics viral RNA, and analyzed the CCL5 expression by quantitative reverse transcription-PCR (qRT-PCR) and enzyme-linked immunosorbent assay (ELISA)...
2017: Biomedical Research
https://www.readbyqxmd.com/read/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#15
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end-points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29039048/sclerosing-and-obstructive-cholangiopathy-in-biliary-atresia-mechanisms-and-association-with-biliary-innate-immunity
#16
REVIEW
Kenichi Harada
Biliary atresia (BA) is histologically characterized by a progressive, sclerosing cholangitis and the obstruction of extrahepatic bile ducts. In terms of the etiology and pathogenesis of BA, several viral infections consisting of dsRNA, including Reoviridae, have been implicated. Human biliary epithelial cells (BECs) possess an innate immune system consisting of Toll-like receptors (TLRs). BECs have negative regulatory mechanisms of TLR tolerance to avoid an excessive inflammatory response to lipopolysaccharide (LPS), a TLR4 ligand; however, they lack the tolerance to poly(I:C) (a synthetic analog of viral dsRNA), a TLR3 ligand...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29026064/the-effect-of-recipient-body-mass-index-and-its-extremes-on-survival-and-graft-vascular-and-biliary-complications-after-liver-transplantation-a-single-center-retrospective-study
#17
Emmanouil Giorgakis, Michele Tedeschi, Eliano Bonaccorsi-Riani, Shirin Elizabeth Khorsandi, Krishna Menon, Hector Vilca-Melendez, Wayel Jassem, Parthi Srinivasan, Andreas Prachalias, Nigel Heaton
BACKGROUND This is the largest UK-based study on the effect of recipient body mass index (BMI) and its extremes (BMI <18.5 and BMI ≥35 kg/m²) on liver transplant (LT) outcomes. Its purpose was to analyze the BMI effect on post-LT mortality, graft loss, primary non-function (PNF), and graft vascular and biliary complications. MATERIAL AND METHODS Data were retrieved from a single-center LT database of 2,115 consecutive patients receiving first LT during period February 2004 to September 2015. Survivals were compared across the BMI groups; the effects of recipient BMI on survival, PNF, and graft vascular and biliary complications were analyzed via regression...
October 13, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/29023905/cholangiopathy-in-critically-ill-patients-surviving-beyond-the-intensive-care-period-a-multicentre-survey-in-liver-units
#18
L Laurent, C Lemaitre, A Minello, A Plessier, G Lamblin, A Poujol-Robert, A Gervais-Hasenknopf, E-A Pariente, P Belenotti, N Mostefa-Kara, P Sogni, M Legrand, J-M Cournac, F Tamion, G Savoye, P Bedossa, D-C Valla, V Vilgrain, O Goria
BACKGROUND: The outcome of cholangiopathy developing in intensive care unit (ICU) is not known in patients surviving their ICU stay. AIM: To perform a survey in liver units, in order to clarify the course of cholangiopathy after surviving ICU stay. METHODS: The files of the liver units affiliated to the French network for vascular liver disease were screened for cases of ICU cholangiopathy developing in patients with normal liver function tests on ICU admission, and no prior history of liver disease...
December 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29022092/unique-manifestations-of-biliary-atresia-provide-new-immunological-insight-into-its-etiopathogenesis
#19
REVIEW
Toshihiro Muraji, Haruo Ohtani, Satoshi Ieiri
Biliary atresia (BA) is a unique cholestatic disease of newborns with a background of exaggerated immune response in the liver of unknown mechanism. Three hypotheses have been proposed; autoimmune type of cholangiopathy triggered by virus infection, graft-versus-host disease type of immune-mediated disease associated with maternal microchimerism and ductal plate malformation theory. Researchers on virus infection theory have experimentally explored immune process causing cholangiopathy on murine models of this disease, while in maternal microchimerism hypothesis were detected maternal cells in the BA patients' liver, of which roles are yet to be determined...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983725/liver-transplantation-for-biliary-atresia-a-systematic-review
#20
REVIEW
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shinji Uemoto
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan...
December 2017: Pediatric Surgery International
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