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https://www.readbyqxmd.com/read/28526690/emerging-concepts-in-biliary-repair-and-fibrosis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
Benign biliary strictures (BBSs) are commonly caused by surgical injury, chronic pancreatitis, and inflammatory cholangiopathies. Although advanced imaging tests and tissue acquisition methods have been developed for evaluation of indeterminate biliary strictures, differentiation of BBSs from biliary malignancies remains a challenge to clinicians. The majority of BBSs have good response to nonsurgical treatment and surgical intervention mainly serves as a rescue when nonsurgical approaches fail. Endoscopic management is a safe, effective, and less-invasive treatment for BBSs compared with other approaches...
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
#14
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
https://www.readbyqxmd.com/read/28249274/the-emerging-role-of-soluble-adenylyl-cyclase-in-primary-biliary-cholangitis
#15
REVIEW
Jung-Chin Chang, Ulrich Beuers, Ronald P J Oude Elferink
BACKGROUND: Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic acid and reduced expression of anion exchanger 2 (AE2) on canalicular membranes and small bile ducts underline the importance of bicarbonate transportation in its disease mechanism...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28249268/bile-acids-in-polycystic-liver-diseases-triggers-of-disease-progression-and-potential-solution-for-treatment
#16
REVIEW
Maria J Perugorria, Ibone Labiano, Aitor Esparza-Baquer, Marco Marzioni, Jose J G Marin, Luis Bujanda, Jesús M Banales
Polycystic liver diseases (PLDs) are a group of genetic hereditary cholangiopathies characterized by the development and progressive growth of cysts in the liver, which are the main cause of morbidity. Current therapies are based on surgical procedures and pharmacological strategies, which show short-term and modest beneficial effects. Therefore, the determination of the molecular mechanisms of pathogenesis appears to be crucial in order to find new potential targets for pharmacological therapy. Ductal plate malformation during embryogenesis and abnormal cystic cholangiocyte growth and secretion are some of the key mechanisms involved in the pathogenesis of PLDs...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28201845/regenerative-medicine-and-the-biliary-tree
#17
Thiago M De Assuncao, Nidhi Jalan-Sakrikar, Robert C Huebert
Despite decades of basic research, biliary diseases remain prevalent, highly morbid, and notoriously difficult to treat. We have, however, dramatically increased our understanding of biliary developmental biology, cholangiocyte pathophysiology, and the endogenous mechanisms of biliary regeneration and repair. All of this complex and rapidly evolving knowledge coincides with an explosion of new technological advances in the area of regenerative medicine. New breakthroughs such as induced pluripotent stem cells and organoid culture are increasingly being applied to the biliary system; it is only a matter of time until new regenerative therapeutics for the cholangiopathies are unveiled...
February 2017: Seminars in Liver Disease
https://www.readbyqxmd.com/read/28184004/ets-proto-oncogene-1-transcriptionally-up-regulates-the-cholangiocyte-senescence-associated-protein-cyclin-dependent-kinase-inhibitor-2a
#18
Steven P O'Hara, Patrick L Splinter, Christy E Trussoni, Maria J Lorenzo Pisarello, Lorena Loarca, Noah S Splinter, Bryce F Schutte, Nicholas F LaRusso
Primary sclerosing cholangitis (PSC) is a chronic, fibroinflammatory cholangiopathy (disease of the bile ducts) of unknown pathogenesis. We reported that cholangiocyte senescence features prominently in PSC and that neuroblastoma RAS viral oncogene homolog (NRAS) is activated in PSC cholangiocytes. Additionally, persistent microbial insult (e.g. LPSs) induces cyclin-dependent kinase inhibitor 2A (CDKN2A/p16(INK4a)) expression and senescence in cultured cholangiocytes in an NRAS-dependent manner. However, the molecular mechanisms involved in LPS-induced cholangiocyte senescence and NRAS-dependent regulation of CDKN2A remain unclear...
March 24, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28141909/improving-the-outcomes-of-organs-obtained-from-controlled-donation-after-circulatory-death-donors-using-abdominal-normothermic-regional-perfusion
#19
Eduardo Miñambres, Borja Suberviola, Beatriz Dominguez-Gil, Emilio Rodrigo, Juan Carlos Ruiz-San Millan, Juan Carlos Rodríguez-San Juan, Maria Angeles Ballesteros
Donation after circulatory death (DCD) has experienced a significant expansion over the last decade. However, warm ischemia results in a greater risk for transplantation. Indeed, controlled DCD (cDCD) was associated with inferior outcomes compared to donation after brain death. The use of abdominal normothermic regional perfusion (nRP) to restore blood flow prior to organ recovery in cDCD has been proposed as a better alternative than rapid recovery to reverse the effect of ischemia and improve recipients' outcome...
January 31, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28121911/normothermic-perfusion-in-the-assessment-and-preservation-of-declined-livers-prior-to-transplantation-hyperoxia-and-vasoplegia-important-lessons-from-the-first-12-cases
#20
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 programme of normothermic ex situ liver perfusion (NESLiP) was developed to facilitate better assessment and use of marginal livers, while minimising cold ischaemia. METHODS: Declined marginal livers and those offered for research were evaluated. NESLiP was performed using an erythrocyte-based perfusate. Viability was assessed with reference to biochemical changes in the perfusate. RESULTS: 12 livers (9 from circulatory death (DCD) and 3 from brain-dead donors), median Donor Risk Index 2...
January 25, 2017: Transplantation
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