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Primary sclerosing cholangitis

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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
#1
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
#2
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/28627935/interactions-between-primary-sclerosing-cholangitis-and-inflammatory-bowel-disease-implications-in-the-adult-liver-transplant-setting
#3
Ken Liu, Simone I Strasser, David J Koorey, Rupert W Leong, Michael Solomon, Geoffrey W McCaughan
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease which is associated with inflammatory bowel disease (IBD) in most cases. As there is currently no medical therapy which alters the natural history of PSC, liver transplantation may be required. Areas covered: We searched for articles in PubMed and critically reviewed current literature on the interrelationship between PSC and IBD with a specific focus on considerations for patients in the liver transplant setting. Expert commentary: PSC is an uncommon disease which limits available studies to be either retrospective or contain relatively small numbers of patients...
June 21, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28624107/recurrence-of-primary-sclerosing-cholangitis-primary-biliary-cholangitis-and-auto-immune-hepatitis-after-liver-transplantation
#4
REVIEW
T Visseren, S Darwish Murad
Liver transplantation is a well-accepted treatment for decompensated chronic liver disease due to primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC) and auto-immune hepatitis (AIH). Survival after liver transplantation is generally good with 1 and 5-year survival rates around 90% and 70-85%. After transplantation, however, these diseases recur in 8.6-27% (rPSC), 10.9-42.3% (rPBC) and 7-42% (rAIH), and this poses significant challenges in terms of management and graft outcome in these patients...
April 2017: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/28614231/incidence-prevalence-and-natural-history-of-primary-sclerosing-cholangitis-in-the-united-kingdom
#5
Huifang Liang, Sudhakar Manne, Jesse Shick, Trevor Lissoos, Paul Dolin
Primary sclerosing cholangitis (PSC) is a rare obliterative fibrotic condition of the bile ducts. We assessed PSC epidemiology and natural history within the UK Clinical Practice Research Datalink (CPRD).Incidence and natural history of PSC were evaluated in a retrospective cohort study using linkage of CPRD, Hospital Episode Statistics, and Office for National Statistics data. Data from age, sex, and general practice-matched population controls provided a context for the incident PSC patients. Liver disease other than PSC was defined as autoimmune hepatitis, hepatitis, hepatomegaly, liver failure, cirrhosis, portal hypertension, cholangiocarcinoma, or hepatobiliary cancer...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28611476/primary-sclerosing-cholangitis-ursodeoxycholic-acid-derivative-safe-and-effective
#6
Iain Dickson
No abstract text is available yet for this article.
June 14, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28611259/prevalence-risk-factors-and-survival-of-patients-with-intrahepatic-cholangiocarcinoma
#7
Paulina Chinchilla-López, Nancy Aguilar-Olivos, Jaime García-Gómez, Karen Hernández-Alejandro, Fredy Chablé-Montero, Daniel Motola-Kuba, Tushar Patel, Nahum Mendez-Sánchez
PURPOSE: To investigate the prevalence, related risk factors, and survival of intrahepatic cholangiocarcinoma in a Mexican population. MATERIAL AND METHODS: We conducted a cross-sectional study at Medica Sur Hospital in Mexico City with approval of the local research ethics committee. We found cases by reviewing all clinical records of in-patients between October 2005 and January 2016 who had been diagnosed with malignant liver tumors. Clinical characteristics and comorbidities were obtained to evaluate the probable risk factors and the Charlson index...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28602220/inhibition-of-microrna-24-increases-liver-fibrosis-by-enhanced-menin-expression-in-mdr2-mice
#8
Chad Hall, Laurent Ehrlich, Fanyin Meng, Pietro Invernizzi, Francesca Bernuzzi, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
BACKGROUND: Liver transplantation remains the primary treatment for primary sclerosing cholangitis (PSC). Mdr2(-/-) mice provide a reliable in vivo model of PSC and develop characteristic biliary inflammation and fibrosis. We tested the hypothesis that the tumor suppressor protein menin is implicated in the progression of liver fibrosis and that menin expression can be regulated in the liver via microRNA-24 (miR-24). MATERIALS AND METHODS: Menin expression was measured in human PSC and Mdr2(-/-) mice...
May 11, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28588885/elevated-trimethylamine-n-oxide-tmao-is-associated-with-poor-prognosis-in-primary-sclerosing-cholangitis-patients-with-normal-liver-function
#9
Martin Kummen, Mette Vesterhus, Marius Trøseid, Bjørn Moum, Asbjørn Svardal, Kirsten Muri Boberg, Pål Aukrust, Tom Hemming Karlsen, Rolf Kristian Berge, Johannes Roksund Hov
BACKGROUND: Trimethylamine-N-oxide (TMAO) is produced in the liver from trimethylamine, which is exclusively generated by gut bacteria. OBJECTIVE: The objective of this article is to investigate the relationship between TMAO and primary sclerosing cholangitis (PSC) and its clinical characteristics. METHODS: Serum TMAO was measured in 305 PSC patients, 90 ulcerative colitis patients and 99 healthy controls. RESULTS: In PSC patients with normal liver function (n = 197), TMAO was higher in patients reaching liver transplantation or death during follow-up than those who did not, with an optimal TMAO cut-off of 4...
June 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28588187/primary-sclerosing-cholangitis-in-a-young-female
#10
S Biswas, C S Bala, M N Ahasan, M K Chowdhury, M M Hassan, P K Sarkar, M M Sarkar, M Haque
Primary sclerosing cholangitis (PSC) is a liver disease characterized by inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts resulting in cholestasis. Due to nonspecific symptoms it is difficult to diagnose until complication arises. It is common in male and usually associated with other autoimmune diseases. Here, we report a case of PSC in a young female which was initially thought to be drug induced cholestasis without presence of any other autoimmunity.
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28583556/duct-to-duct-biliary-reconstruction-in-living-donor-liver-transplantation-for-primary-sclerosing-cholangitis-report-of-a-case
#11
T Motomura, T Yoshizumi, H Wang, A Nagatsu, S Itoh, N Harada, N Harimoto, T Ikegami, H Uchiyama, Y Soejima, Y Maehara
Although Roux-en Y hepaticojejunostomy was previously recommended for the biliary reconstruction in liver transplantation for primary sclerosing cholangitis (PSC), some recent reports showed no difference in the graft survival between Roux-en Y and duct-to-duct anastomosis in deceased-donor liver transplantation. On the other hand, considering the risk of recurrence and the short length of the bile duct of the graft, duct-to-duct biliary anastomosis has never been reported in a patient undergoing living-donor liver transplantation (LDLT) for PSC...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28580726/the-first-case-of-domino-split-liver-transplantation-in-maple-syrup-urine-disease
#12
Uta Herden, Jun Li, Lutz Fischer, Florian Brinkert, Martin Blohm, René Santer, Bjoern Nashan, Enke Grabhorn
The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet...
June 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28566881/inflammatory-bowel-disease-in-liver-transplanted-patients
#13
REVIEW
Tajana Filipec Kanizaj, Maja Mijic
Most common hepatobiliary manifestation of inflammatory bowel disease (IBD) are primary sclerosing cholangitis (PSC) and autoimmune hepatitis, ranking them as the main cause of liver transplantation (LT) in IBD setting. Course of pre-existing IBD after LT differs depending on many transplant related factors. Potential risk factors related to IBD deterioration after LT are tacrolimus-based immunosuppressive regimens, active IBD and cessation of 5-aminosalicylates at the time of LT. About 30% patients experience improvement of IBD after LT, while approximately the same percentage of patients worsens...
May 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28564724/epigenetics-in-the-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis
#14
Angela C Cheung, Nicholas F LaRusso, Gregory J Gores, Konstantinos N Lazaridis
No abstract text is available yet for this article.
May 2017: Seminars in Liver Disease
https://www.readbyqxmd.com/read/28561948/identification-of-fucosylated-fetuin-a-as-a-potential-biomarker-for-cholangiocarcinoma
#15
Lucy Betesh, Mary Ann Comunale, Mengjun Wang, Hongyan Liang, Julie Hafner, Aykan Karabudak, Nasra H Giama, Catherine D Moser, Eiji Miyoshi, Lewis R Roberts, Timothy M Block, Anand Mehta
CLINICAL RELEVANCE: Cholangiocarcinoma is a cancer of bile duct cells for which no effective biomarker exists. Tumor masses are often not identifiable by imaging modalities as a result of the location of the tumor in the hilum of the liver or within the bile ducts. Because of the problems with obtaining a definitive tissue diagnosis, treatment and management decisions for patients with biliary strictures that may be malignant are difficult. The work presented here highlights the potential role of fucosylated fetuin-A as a biomarker of cholangiocarcinoma and may help in the early detection of this deadly cancer...
May 31, 2017: Proteomics. Clinical Applications
https://www.readbyqxmd.com/read/28555945/recommendations-on-the-use-of-mri-in-psc-a-position-statement-from-the-international-psc-study-group
#16
REVIEW
Christoph Schramm, John Eaton, Kristina I Ringe, Sudhakar Venkatesh, Jin Yamamura
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder characterized by inflammation and fibrosis of the intra and/or extrahepatic bile ducts. Magnetic resonance imaging (MRI) is a noninvasive imaging modality that can be used to diagnose PSC and detect disease related complications. Quantitative MRI technologies also have the potential to provide valuable prognostic information. Despite the potential of this imaging technology, the clinical application of MRI in the care of PSC patients and imaging standards vary across institutions...
May 26, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28555885/serum-extracellular-vesicles-contain-protein-biomarkers-for-primary-sclerosing-cholangitis-and-cholangiocarcinoma
#17
Ander Arbelaiz, Mikel Azkargorta, Marcin Krawczyk, Alvaro Santos-Laso, Ainhoa Lapitz, Maria J Perugorria, Oihane Erice, Esperanza Gonzalez, Raul Jimenez-Agüero, Adelaida Lacasta, Cesar Ibarra, Alberto Sanchez-Campos, Juan P Jimeno, Frank Lammert, Piotr Milkiewicz, Marco Marzioni, Rocio I R Macias, Jose J G Marin, Tushar Patel, Gregory J Gores, Ibon Martinez, Félix Elortza, Juan M Falcon-Perez, Luis Bujanda, Jesus M Banales
Cholangiocarcinoma (CCA) includes a heterogeneous group of biliary cancers with poor prognosis. Several conditions such as primary sclerosing cholangitis (PSC) are risk factors. Non-invasive differential diagnosis between intrahepatic CCA (iCCA) and hepatocellular carcinoma (HCC) is sometimes difficult. Accurate non-invasive biomarkers for PSC, CCA or HCC are not available. In the search of novel biomarkers, serum extracellular vesicles (EV) were isolated from CCA (n=43), PSC (n=30) or HCC (n=29) patients, and healthy individuals (control, n=32), and their protein content was characterized...
May 26, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28547740/biliary-duodenal-anastomosis-using-magnetic-compression-following-massive-resection-of-small-intestine-due-to-strangulated-ileus-after-living-donor-liver-transplantation-a-case-report
#18
Ryusuke Saito, Hiroyuki Tahara, Seiichi Shimizu, Masahiro Ohira, Kentaro Ide, Kohei Ishiyama, Tsuyoshi Kobayashi, Hideki Ohdan
BACKGROUND: Despite the improvements of surgical techniques and postoperative management of patients with liver transplantation, biliary complications are one of the most common and important adverse events. We present a first case of choledochoduodenostomy using magnetic compression following a massive resection of the small intestine due to strangulated ileus after living donor liver transplantation. CASE PRESENTATION: The 54-year-old female patient had end-stage liver disease, secondary to liver cirrhosis, due to primary sclerosing cholangitis with ulcerative colitis...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28543841/role-of-fluorescence-in-situ-hybridization-in-diagnosing-cholangiocarcinoma-in-indeterminate-biliary-strictures
#19
Z H Liew, T J Z Loh, T K H Lim, T H Lim, C J L Khor, S J Mesenas, C S C Kong, W C Ong, D M Y Tan
BACKGROUND: Brush cytology, the conventional method to diagnose cholangiocarcinoma, has been plagued by low diagnostic sensitivity and false-negative results. We aim to study the clinical utility of fluorescence in situ hybridization (FISH) in enhancing identification of malignant biliary strictures. METHODS: Brush cytologic specimens collected from endoscopic retrograde cholangiopancreatography (ERCP) for biliary strictures in a tertiary hospital in Singapore from March 2013 to July 2015 were examined by FISH technique using UroVysion probe set in this study...
May 19, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28535810/fibrates-for-the-treatment-of-cholestatic-itch-fitch-study-protocol-for-a-randomized-controlled-trial
#20
Ruth Bolier, Elsemieke S de Vries, Albert Parés, Jeltje Helder, E Marleen Kemper, Koos Zwinderman, Ronald P Oude Elferink, Ulrich Beuers
BACKGROUND: Pruritus (itch) is a frequent, burdensome and difficult-to-treat symptom in patients with cholestasis. Fibrates are currently under investigation for the treatment of primary biliary cholangitis in patients with a suboptimal response to ursodeoxycholic acid. Moreover, there is empirical evidence for a possible antipruritic effect. We aim to prove this in a randomized controlled trial, including patients with cholestatic liver diseases other than primary biliary cholangitis that are accompanied by pruritus...
May 23, 2017: Trials
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