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https://www.readbyqxmd.com/read/29419931/observations-on-the-ex-situ-perfusion-of-livers-for-transplantation
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
Guido Carpino, Vincenzo Cardinale, Trine Folseraas, Diletta Overi, Annarosa Floreani, Antonio Franchitto, Paolo Onori, Nora Cazzagon, Pasquale Bartolomeo Berloco, Tom Hemming Karlsen, Domenico Alvaro, Eugenio Gaudio
Primary sclerosing cholangitis and primary biliary cholangitis are human primary cholangiopathies; these diseases are characterized by the damage of mature cholangiocytes and by the appearance of ductular reaction as the results of hepatic progenitor cell activation. The aims of this study were to evaluate differences in progenitor cell niche activation between these two human cholangiopathies. Human liver tissue was obtained from normal liver donors (n=5), primary sclerosing (n=20), and primary biliary cholangitis (n=20)...
December 14, 2017: 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
#8
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
#9
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
#10
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
#11
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
#12
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-diabetic-genetic-background-to-cause-autoimmune-cholangitis
#13
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...
January 1, 2018: 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
#14
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 most common form 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...
February 2018: 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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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