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https://www.readbyqxmd.com/read/28648950/mechanisms-of-cholangiocyte-responses-to-injury
#1
REVIEW
Keisaku Sato, Fanyin Meng, Thao Giang, Shannon Glaser, Gianfranco Alpini
Cholangiocytes, epithelial cells that line the biliary epithelium, are the primary target cells for cholangiopathies including primary sclerosing cholangitis and primary biliary cholangitis. Quiescent cholangiocytes respond to biliary damage and acquire an activated neuroendocrine phenotype to maintain the homeostasis of the liver. The typical response of cholangiocytes is proliferation leading to bile duct hyperplasia, which is a characteristic of cholestatic liver diseases. Current studies have identified various signaling pathways that are associated with cholangiocyte proliferation/loss and liver fibrosis in cholangiopathies using human samples and rodent models...
June 22, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28645651/post-transplant-cholangiopathy-classification-pathogenesis-and-preventive-strategies
#2
REVIEW
Yvonne de Vries, Fien A von Meijenfeldt, Robert J Porte
Biliary complications are the most frequent cause of morbidity, re-transplantation, and even mortality after liver transplantation. In general, biliary leakage and anastomotic and non-anastomotic biliary strictures (NAS) can be recognized. There is no consensus on the exact definition of NAS and different names and criteria have been used in literature. We propose to use the term post-transplant cholangiopathy for the spectrum of abnormalities of large donor bile ducts, that includes NAS, but also intraductal casts and intrahepatic biloma formation, in the presence of a patent hepatic artery...
June 20, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#3
Sittisak Honsawek, Wanvisa Udomsinprasert, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients. METHODS: One hundred post-Kasai BA patients and 40 controls were enrolled...
June 21, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28635176/genetic-loss-of-the-muscarinic-m3-receptor-m3-r-markedly-alters-bile-formation-and-cholestatic-liver-injury-in-mice
#4
Franziska Durchschein, Elisabeth Krones, Marion J Pollheimer, Gernot Zollner, Martin Wagner, Jean-Pierre Raufman, Peter Fickert
Hepatic innervation represents a potentially underestimated regulator of liver function and regeneration. The muscarinic 3 receptor (M3 -R) is the primary cholangiocyte receptor for the afferent parasympathetic innervation of bile ducts. We aimed to determine the specific role of the M3 -R in bile formation and models for cholestatic liver disease in mice. We compared bile flow and composition in M3 -R knock-out mice (M3 -R(-/-) ) and wild type littermates (WT). M3 -R(-/-) mice showed significantly reduced bile flow compared to WT mice, most likely due to decreased biliary HCO3(-) secretion...
June 21, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28634212/prolonged-darkness-reduces-liver-fibrosis-in-a-mouse-model-of-primary-sclerosing-cholangitis-by-mir-200b-down-regulation
#5
Nan Wu, Fanyin Meng, Tianhao Zhou, Yuyan Han, Lindsey Kennedy, Julie Venter, Heather Francis, Sharon DeMorrow, Paolo Onori, Pietro Invernizzi, Francesca Bernuzzi, Romina Mancinelli, Eugenio Gaudio, Antonio Franchitto, Shannon Glaser, Gianfranco Alpini
Melatonin therapy or prolonged exposure to complete darkness reduces biliary hyperplasia and liver fibrosis in bile-duct-ligated (BDL) rats; however, no information exists in primary sclerosing cholangitis (PSC). Thus, we aimed to determine the therapeutic effects of prolonged dark therapy or melatonin administration on hepatic fibrosis in the Mdr2(-/-) mouse model of PSC. Melatonin levels, biliary mass, liver fibrosis, angiogenesis and miR-200b expression were evaluated in wild-type and Mdr2(-/-) mice exposed to darkness or melatonin treatment or in male PSC patient samples and healthy controls...
June 20, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28556380/waitlist-outcomes-for-patients-relisted-following-failed-donation-after-cardiac-death-liver-transplant-implications-for-awarding-meld-exception-scores
#6
Kristopher P Croome, David D Lee, Justin H Nguyen, Andrew P Keaveny, C Burcin Taner
Understanding of outcomes for patients re-listed for ischemic cholangiopathy (IC) following a donation after cardiac death (DCD) liver transplant (LT) will help standardization of MELD exception scheme for re-transplantation. Early relisting (E-RL) for DCD graft failure due to PNF or HAT was defined as re-listing ≤ 14 days after DCD-LT, late relisting (L-RL) due to biliary complications was defined as re-listing 14 days- 3 years after DCD-LT. Of 3908 DCD-LT performed nationally between 2002-2016, 540 (13...
May 29, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28526690/emerging-concepts-in-biliary-repair-and-fibrosis
#7
Luca Fabris, Carlo Spirli, Massimiliano Cadamuro, Romina Fiorotto, Mario Strazzabosco
Chronic diseases of the biliary tree (cholangiopathies) represent one of the major unmet needs in clinical hepatology and a significant knowledge gap in liver pathophysiology. The common theme in cholangiopathies is that the target of the disease is the biliary tree. After damage to the biliary epithelium, inflammatory changes stimulate a reparative response with proliferation of cholangiocytes and restoration of the biliary architecture, owing to the re-activation of a variety of morphogenetic signals. Chronic damage and inflammation, will ultimately result in pathologic repair, with generation of biliary fibrosis and clinical progression of the disease...
May 19, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28523071/forsaken-foregut-case-report-of-simultaneous-black-esophagus-and-ischemic-cholangiopathy
#8
Paul A Cameron, Franzjosef Schweiger
Black esophagus or acute esophageal necrosis rarely occurs after severe hemodynamic compromise or low-flow states. Other contributing factors may include corrosive injury from gastric contents and diminished mucosal repair mechanisms. Ischemic cholangitis, another rare clinical entity, is also usually the result of a significant vascular and/or hypotensive insult to the biliary tree. We describe the first case of combined acute esophageal necrosis and ischemic cholangiopathy in a 62-year-old male who completely recovered from the esophageal injury but developed progressive liver failure from ischemic cholangiopathy...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28497003/achondroplasia-and-biliary-atresia-a-rare-association-and-review-of-literature
#9
Ranjit I Kylat
Achondroplasia (ACH) occurs in most cases as de novo mutations of the gene-encoding fibroblast growth factor receptor 3 (FGFR3). Biliary atresia (BA) is a progressive neonatal inflammatory and fibro-obliterative cholangiopathy affecting the extra- and intrahepatic biliary tree to varying degrees, and it results in obstruction to bile flow and cholestatic jaundice in neonates. BA is thought to be a multifactorial disease, genome association studies have shown abnormalities in susceptibility genes, and levels of fibroblast growth factor 21 (FGF21) and fibroblast growth factor 23 (FGF23) have been noted to be increased...
June 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28478841/somatic-mutation-a-cause-of-biliary-atresia-a-hypothesis
#10
Alexandre Fabre, Céline Roman, Bertrand Roquelaure
Despite many years of research, the causes of biliary atresia still remain elusive. Infection, immune disorder, toxins or maternal microchimerism have been cited as potential triggers of biliary atresia. This is a rare disease with a stable incidence over the years although with sizeable ethnic variations. This stability suggests that environmental factors have in fact only a slight influence. During the search for etiologies, twin studies have often helped disentangle the genetic from the environmental. For this condition, twin studies have mainly demonstrated a lack of concordance between twins (either monozygotic or dizygotic), ruling out Mendelian, infectious or toxic causes...
May 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28437389/normothermic-perfusion-in-the-assessment-and-preservation-of-declined-livers-before-transplantation-hyperoxia-and-vasoplegia-important-lessons-from-the-first-12-cases
#11
Christopher J E Watson, Vasilis Kosmoliaptsis, Lucy V Randle, Alexander E Gimson, Rebecca Brais, John R Klinck, Mazin Hamed, Anastasia Tsyben, Andrew J Butler
BACKGROUND: A program of normothermic ex situ liver perfusion (NESLiP) was developed to facilitate better assessment and use of marginal livers, while minimizing cold ischemia. METHODS: Declined marginal livers and those offered for research were evaluated. Normothermic ex situ liver perfusion was performed using an erythrocyte-based perfusate. Viability was assessed with reference to biochemical changes in the perfusate. RESULTS: Twelve livers (9 donation after circulatory death [DCD] and 3 from brain-dead donors), median Donor Risk Index 2...
May 2017: Transplantation
https://www.readbyqxmd.com/read/28404115/advances-in-primary-sclerosing-cholangitis
#12
REVIEW
Jennifer L Horsley-Silva, Elizabeth J Carey, Keith D Lindor
Primary sclerosing cholangitis is a chronic, progressive cholangiopathy that frequently affects men and is associated with inflammatory bowel disease. Although the cause of the disease is still debated, a genetic association and link to immune-mediated disease triggered by environmental factors are thought to contribute. The disease can present as isolated imaging abnormalities, biochemical changes, cholangiocarcinoma, or end-stage complications such as cirrhosis. Symptoms of primary sclerosing cholangitis include fatigue, jaundice, pruritus, or steatorrhoea...
September 2016: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28381144/varicella-zoster-virus-gastritis
#13
Erik W Nohr, Doha M Itani, Christopher N Andrews, Margaret M Kelly
We report varicella-zoster virus (VZV) gastritis in a 70-year-old woman postchemotherapy for lymphoma, presenting with abdominal pain, vomiting, and delirium without rash. A gastric biopsy demonstrated viral inclusions but posed a diagnostic challenge as immunohistochemistry for cytomegalovirus and herpes simplex virus were negative, and VZV immunohistochemistry was not available. The patient developed a vesicular rash 7 days after her symptoms began. Molecular testing of the gastric biopsy and a skin swab both confirmed VZV infection...
March 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28343205/-biliary-atresia-signs-and-symptoms-diagnosis-clinical-management
#14
Ewa Orłowska, Piotr Czubkowski, Piotr Socha
Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28333915/directed-differentiation-of-human-induced-pluripotent-stem-cells-into-functional-cholangiocyte-like-cells
#15
Fotios Sampaziotis, Miguel Cardoso de Brito, Imbisaat Geti, Alessandro Bertero, Nicholas Rf Hannan, Ludovic Vallier
The difficulty in isolating and propagating functional primary cholangiocytes is a major limitation in the study of biliary disorders and the testing of novel therapeutic agents. To overcome this problem, we have developed a platform for the differentiation of human pluripotent stem cells (hPSCs) into functional cholangiocyte-like cells (CLCs). We have previously reported that our 26-d protocol closely recapitulates key stages of biliary development, starting with the differentiation of hPSCs into endoderm and subsequently into foregut progenitor (FP) cells, followed by the generation of hepatoblasts (HBs), cholangiocyte progenitors (CPs) expressing early biliary markers and mature CLCs displaying cholangiocyte functionality...
April 2017: Nature Protocols
https://www.readbyqxmd.com/read/28317394/an-update-on-the-pathophysiology-and-management-of-polycystic-liver-disease
#16
May Yw Wong, Geoffrey W McCaughan, Simone I Strasser
Polycystic liver disease (PLD) is characterized by the presence of multiple cholangiocyte-derived hepatic cysts that progressively replace liver tissue. They are classified as an inherited ciliopathy /cholangiopathy as pathology exists at the level of the primary cilia of cholangiocytes. Aberrant expression of the proteins in primary cilia can impair their structures and functions, thereby promoting cystogenesis. Areas covered: This review begins by looking at the epidemiology of PLD and its natural history...
March 28, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28316853/primary-hepatic-neuroendocrine-tumor-with-unusual-thyroid-follicular-like-morphologic-characteristics
#17
Mohd Elmugtaba Ibrahim, Kerolos Abadeer, Qihui Jim Zhai, Aziza Nassar
We describe a primary hepatic neuroendocrine tumor of a 57-year-old Thai woman who presented in 2004 with a suspicious mass in the left hepatic lobe. She underwent left hepatectomy for the 10.5-cm mass, called intermediate grade neuroendocrine carcinoma of unknown origin, likely metastatic. The tumor recurred in 2007, then called recurrent primary hepatic neuroendocrine tumor (PHNET), and the patient underwent liver transplant. Because of similarity between the neuroendocrine tumor and a thyroid tumor-specifically, follicular-like characteristics-immunohistochemical stains for thyroglobulin, TTF1, and calcitonin were performed...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28289704/preferential-tnf%C3%AE-signaling-via-tnfr2-regulates-epithelial-injury-and-duct-obstruction-in-experimental-biliary-atresia
#18
Pranavkumar Shivakumar, Tatsuki Mizuochi, Reena Mourya, Sridevi Gutta, Li Yang, Zhenhua Luo, Jorge A Bezerra
Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNFA and its receptors TNFR1 and TNFR2 in human livers and found an increased expression of the receptors at the time of diagnosis...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28283322/asia-pacific-consensus-guidelines-for-endoscopic-management-of-benign-biliary-strictures
#19
REVIEW
Bing Hu, Bo Sun, Qiang Cai, James Yun Wong Lau, Shuren Ma, Takao Itoi, Jong Ho Moon, Ichiro Yasuda, Xiaofeng Zhang, Hsiu-Po Wang, Shomei Ryozawa, Rungsun Rerknimitr, Wen Li, Hiromu Kutsumi, Sundeep Lakhtakia, Hideyuki Shiomi, Ming Ji, Xun Li, Dongmei Qian, Zhuo Yang, Xiao Zheng
No abstract text is available yet for this article.
March 7, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28271527/the-role-of-lncrna-h19-in-gender-disparity-of-cholestatic-liver-injury-in-mdr2-mice
#20
Xiaojiaoyang Li, Runping Liu, Jing Yang, Lixin Sun, Luyong Zhang, Zhenzhou Jiang, Puneet Puri, Emily C Gurley, Guanhua Lai, Yuping Tang, Zhiming Huang, William M Pandak, Phillip B Hylemon, Huiping Zhou
The multi-drug resistance 2 knockout (Mdr2(-/-) ) mouse is a well-established model of cholestatic cholangiopathies. Female Mdr2(-/-) mice develop more severe hepatobiliary damage than male Mdr2(-/-) mice, which is correlated with a higher proportion of taurocholate (TCA) in bile. Although estrogen has been identified as an important player in intrahepatic cholestasis, the underlying molecular mechanisms of gender-based disparity of cholestatic injury remain unclear. The long non-coding RNA H19 is an imprinted, maternally expressed and estrogen-targeted gene, which is significantly induced in human fibrotic/cirrhotic liver and bile duct ligated mouse liver...
March 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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