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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28650098/disparities-in-eurotransplant-liver-transplantation-waitlist-outcome-between-patients-with-and-without-exceptional-meld
#1
Andreas Umgelter, Alexander Hapfelmeier, Wouter Kopp, Marieke van Rosmalen, Xavier Rogiers, Markus Guba
BACKGROUND: The sickest-first principle in donor-liver allocation can be implemented by allocating organs to cirrhotic patients with the highest Model of End-stage Liver Disease (MELD) scores. For patients with other risk factors, standard (SE) and non-standard exceptions (NSE) have been developed. We investigated whether this system of matched MELD scores achieves similar outcome on the liver transplant waiting-list for various diagnostic groups in Eurotransplant (ET) countries with MELD-based individual allocation (Belgium, The Netherlands and Germany)...
June 26, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28648950/mechanisms-of-cholangiocyte-responses-to-injury
#2
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/28646549/inhibition-of-jnk-signaling-mediates-ppar%C3%AE-dependent-protection-against-intrahepatic-cholestasis-by-fenofibrate
#3
Manyun Dai, Julin Yang, Minzhu Xie, Jiao Lin, Min Luo, Huiying Hua, Gangming Xu, Hante Lin, Danjun Song, Yuqing Cheng, Bin Guo, Jinshun Zhao, Frank J Gonzalez, Aiming Liu
BACKGROUND AND PURPOSE: Fenofibrate, a PPARα agonist, is the most widely prescribed drug for the treatment of hyperlipidemia. Although fibrate drugs were reported to be beneficial for cholestasis, the underlying mechanism has not been determined. EXPERIMENTAL APPROACH: Wild-type mice and Pparα-null mice were orally pretreated with fenofibrate for three days, following which ANIT was administered to induce cholestasis. The potent experimental PPARα agonist WY14643 was used to validate the role of PPARα, and the JNK inhibitor SP600125 was employed to explore the role of the JNK pathway in cholestatic liver injury...
June 23, 2017: British Journal of Pharmacology
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/28627935/interactions-between-primary-sclerosing-cholangitis-and-inflammatory-bowel-disease-implications-in-the-adult-liver-transplant-setting
#6
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/28625770/secondary-sclerosing-cholangitis-following-cardiac-surgery-an-uncommon-but-deadly-gastrointestinal-complication
#7
EDITORIAL
Kevin Landolfo, Erol Belli
No abstract text is available yet for this article.
May 18, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28624107/recurrence-of-primary-sclerosing-cholangitis-primary-biliary-cholangitis-and-auto-immune-hepatitis-after-liver-transplantation
#8
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/28620623/resolution-of-diffuse-intrahepatic-biliary-strictures-after-chemotherapy-for-metastatic-ovarian-cancer
#9
Daniel Lew, Vinay Sundaram, Brad D Barrows, Simon K Lo, Srinivas Gaddam
Sclerosing cholangitis and cholestatic jaundice secondary to metastatic disease is a rare complication. We report a rare case of secondary sclerosing cholangitis (SSC) due to lymphatic spread from ovarian cancer with complete resolution after chemotherapy. The diagnosis of SSC from metastatic ovarian cancer was clinically challenging, as endoscopic retrograde cholangiopancreatography revealed irregular hepatic ducts consistent with sclerosing cholangitis, but it did not identify any malignant cells. The final diagnosis was made with liver biopsy revealing high-grade metastatic Mullerian carcinoma...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28614231/incidence-prevalence-and-natural-history-of-primary-sclerosing-cholangitis-in-the-united-kingdom
#10
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
#11
Iain Dickson
No abstract text is available yet for this article.
July 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28611259/prevalence-risk-factors-and-survival-of-patients-with-intrahepatic-cholangiocarcinoma
#12
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/28607292/laparoscopic-total-colectomy-for-ulcerative-colitis-after-liver-transplantation-is-feasible
#13
Benjamin Darnis, Gilles Poncet, Maud Robert
Ulcero-haemorrhagic rectocolitis can occur after liver transplantation for sclerosing cholangitis. Total colectomy with or without proctectomy may be indicated in case of chronic drug-resistant colitis, dysplasia or cancer. Today, laparoscopic approach is the standard for such procedure in non-operated patients. We performed a completely laparoscopic total colectomy 5 years after a liver transplantation. There were a few peritoneal adherences, and we could safely perform the procedure almost as usual. It provided all the advantages of the laparoscopic approach in the post-operative course...
July 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28602220/inhibition-of-microrna-24-increases-liver-fibrosis-by-enhanced-menin-expression-in-mdr2-mice
#14
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/28597951/treg-conditioning-endows-activated-teff-with-suppressor-function-in-autoimmune-hepatitis-autoimmune-sclerosing-cholangitis
#15
Rodrigo Liberal, Charlotte R Grant, Muhammed Yuksel, Jonathon Graham, Alireza Kalbasi, Yun Ma, Michael A Heneghan, Giorgina Mieli-Vergani, Diego Vergani, Maria Serena Longhi
Imbalance between T regulatory (Treg) and T effector (Teff) cells is likely to contribute to induction and perpetuation of liver damage in autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC) either through inability of Tregs to restrain proliferation and effector cytokine production by responders or through conversion of Tregs into T helper type 1 (Th1) or type 17 (Th17) effector lymphocytes. We investigated the effect of Treg skewing on the phenotypic and functional properties of CD4(+) CD127(+) CD25(high) cells, an activated subset of Teff, in 32 patients with AIH, 20 with AISC and in 36 healthy subjects (HS)...
June 9, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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
#16
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
#17
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
#18
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
#19
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
#20
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
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