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Primary sclerosing cholangitis

E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leukocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged 3-15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011 and followed up for 4...
October 19, 2016: Acta Paediatrica
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Ashwani K Singal, Xiao Fang, Mohamed Kaif, Mohsen Hasanin, Brendan M Mcguire, Yong-Fang Kuo, Russell H Wiesner
MAIN PROBLEM: Patients with primary sclerosing cholangitis (PSC) have frequent episodes of cholangitis with potential for high mortality while waiting for liver transplantation. However, data on wait-list mortality specific to liver disease etiology are limited. RESULTS: Using UNOS database (2002-2013), of 81,592 listed patients, 11284 (13.8%) died waiting for transplant. Primary biliary cirrhosis (PBC) patients (N=3491) compared to PSC (N=4905) differed for age (56 vs...
October 18, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
Nurdan Tözün, Eser Vardareli
Gastrointestinal (GI) cancers are the leading cause of mortality worldwide. These cancers are the end result of a complex interplay between gene and environment. Bacteria, parasites, and viruses have been implicated in some cancers. Recent data have put at focus the gut microbiome as the key player firing tumorigenesis. Experimental and human studies have provided evidence on the role of microbiota in cancer development. Although subject to changes in different settings such as antibiotic treatment, diet or lifestyle, our microbiome is quite stable and is capable of increasing susceptibility to cancer or decrease and halt its progression...
November 2016: Journal of Clinical Gastroenterology
Cynthia Buness, Keith D Lindor, Tamir Miloh
Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin...
September 2016: Pediatric Gastroenterology, Hepatology & Nutrition
Rodrigo Liberal, Charlotte R Grant
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues...
October 8, 2016: World Journal of Hepatology
Hanno Ehlken, Raluca Wroblewski, Christophe Corpechot, Lionel Arrivé, Tim Rieger, Johannes Hartl, Susanne Lezius, Peter Hübener, Kornelius Schulze, Roman Zenouzi, Marcial Sebode, Moritz Peiseler, Ulrike W Denzer, Alexander Quaas, Christina Weiler-Normann, Ansgar W Lohse, Olivier Chazouilleres, Christoph Schramm
BACKGROUND: Patients with primary sclerosing cholangitis (PSC) develop progressive liver fibrosis and end-stage liver disease. Non-invasive and widely available parameters are urgently needed to assess disease stage and the risk of clinical progression. Transient elastography (TE) has been reported to predict fibrosis stage and disease progression. However, these results have not been confirmed in an independent cohort and comparison of TE measurement to other non-invasive means is missing...
2016: PloS One
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Sven H Loosen, Fabian Benz, Jennifer Niedeggen, Maximilian Schmeding, Florian Schüller, Alexander Koch, Mihael Vucur, Frank Tacke, Christian Trautwein, Christoph Roderburg, Ulf P Neumann, Tom Luedde
BACKGROUND: Elevated expression levels of S100A6, a calcium-binding low-molecular-weight protein, were demonstrated in various malignancies. Moreover, increased serum levels of S100A6 were suggested as a novel biomarker for various inflammatory and malignant diseases including lung and gastric cancer. However, up to now, serum concentrations of S100A6 have not been analyzed in patients with cholangiocarcinoma (CCA). METHODS: S100A6 mRNA expression levels were analyzed in human and murine CCA tumor samples, using semi-quantitative reverse transcriptase PCR...
December 2016: Clinical and Translational Medicine
S H Loosen, M Schmeding, C Roderburg, M Binnebösel, I Temizel, F M Mottaghy, J J W Tischendorf, F Tacke, N T Gaisa, K Hussein, U Lehmann, C Trautwein, U P Neumann, T Luedde
We report the case of a 53-year-old female patient who was transplanted with the liver of a 71-year-old male donor for advanced primary sclerosing cholangitis (PSC) and who additionally was diagnosed with a histologically non-classifiable colitis shortly before transplantation. Upon follow-up abdominal ultrasound 4 months after transplantation, a liver lesion measuring 16 × 23 mm was detected in the transplanted liver. This lesion had not been noticed immediately after transplantation and showed a pattern suspicious for malignancy in contrast-enhanced ultrasound...
October 5, 2016: Zeitschrift Für Gastroenterologie
Jarno Jokelainen, Marianne Udd, Leena Kylänpää, Harri Mustonen, Jorma Halttunen, Outi Lindström, Reino Pöyhiä
OBJECTIVE: Patient controlled sedation (PCS) has been shown to be a valid choice for sedation during endoscopic retrograde cholangiopancreatography (ERCP) in randomized studies. However, large scale studies are lacking. MATERIAL AND METHODS: A single center, prospective observational study to determine how sedation for ERCP is administered in clinical setting. All 956 patients undergoing 1196 ERCPs in the endoscopy unit of Helsinki University Central Hospital 2012-2013, methods of sedation and adverse events associated with different sedations were recorded...
October 5, 2016: Scandinavian Journal of Gastroenterology
B Njei, T R McCarty, S Varadarajulu, U Navaneethan
BACKGROUND: The accuracy of current endoscopic modalities for diagnosing cholangiocarcinoma in primary sclerosing cholangitis (PSC) is suboptimal. AIM: To evaluate the comparative effectiveness of endoscopic retrograde cholangiopancreatography (ERCP)-based modalities, independently or in combination, for the diagnosis of cholangiocarcinoma in patients with PSC-induced biliary strictures. METHODS: Searches of PubMed, EMBASE, Web of Science and the Cochrane Library databases were performed through December 2015...
October 3, 2016: Alimentary Pharmacology & Therapeutics
Serge J Zweers, Elisabeth M de Vries, Martin Lenicek, Dagmar Tolenaars, D Rudi de Waart, Kiran V K Koelfat, Albert K Groen, Steven W M Olde Damink, Ulrich Beuers, Cyriel Ponsioen, Peter L M Jansen, Frank G Schaap
BACKGROUND: Bile salts likely contribute to liver injury in patients with primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC). Fibroblast growth factor 19 (FGF19) is a bile salt-induced enterokine with hepatoprotective potential as it suppresses de novo bile salt synthesis. Here, we evaluated the bile salt receptor FXR/FGF19 gut-liver axis in PSC and PBC patients. METHODS: Fasted patients with PSC (n = 12) and PBC (n = 10), and healthy controls (HC; n = 10) were orally challenged with the natural FXR agonist chenodeoxycholic acid (CDCA 15 mg/kg)...
September 30, 2016: Hepatology International
Keisuke Hata, Soichiro Ishihara, Hiroaki Nozawa, Kazushige Kawai, Tomomichi Kiyomatsu, Toshiaki Tanaka, Junko Kishikawa, Hiroyuki Anzai, Toshiaki Watanabe
Restorative proctocolectomy with ileal pouch-anal anastomosis has been the surgical treatment of choice for patients with ulcerative colitis who require surgery. The quality of life after this procedure is satisfactory in most cases; however, pouchitis is a troublesome condition involving inflammation of the ileal pouch. When a patient presents with symptoms of pouchitis, such as increased bowel movements, mucous and/or bloody exudates, abdominal cramps, and fever, endoscopy is essential for a precise diagnosis...
September 28, 2016: Digestive Endoscopy: Official Journal of the Japan Gastroenterological Endoscopy Society
H Grønbaek, M Kreutzfeldt, K Kazankov, N Jessen, T Sandahl, S Hamilton-Dutoit, H Vilstrup, H J Møller
BACKGROUND: Autoimmune hepatitis (AIH) is characterised by liver inflammation with reversibility upon anti-inflammatory treatment. Soluble (s)CD163, a specific macrophage activation marker, is associated with inflammation in other liver diseases, but never investigated in AIH. AIM: To investigate sCD163 in patients with acute AIH and in complete and incomplete responders to standard anti-inflammatory pharmacotherapy, and during follow-up in treatment naive patients...
November 2016: Alimentary Pharmacology & Therapeutics
Xiongbo Wu, Min Xia, Dayang Chen, Fang Wu, Zhifa Lv, Qiang Zhan, Yang Jiao, Wenjie Wang, Guangxia Chen, Fangmei An
Altered microRNA (miRNA) expression plays a role in cholangiocarcinoma (CCA) development; thus, detection of blood-circulating miRNAs could be useful as CCA markers. This study profiled serum miRNA levels in patients with primary sclerosing cholangitis (PSC) and CCA and then assessed the role of miR-150-5p in CCA progression in vitro. Three samples were randomly selected from each of 50 sera of healthy controls, 30 PSC sera, and 28 CCA sera with matched bile samples for miRNA microarray profiling. The dysregulated miRNAs were confirmed using qRT-PCR, and miR-150-5p was selected for further in vitro and ex vivo studies...
September 22, 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
Eva Kristine Klemsdal Henriksen, Kristin Kaasen Jørgensen, Fatemeh Kaveh, Kristian Holm, David Hamm, Johanna Olweus, Espen Melum, Brian K Chung, Tor J Eide, Knut E A Lundin, Kirsten Muri Boberg, Tom H Karlsen, Gideon M Hirschfield, Evaggelia Liaskou
BACKGROUND & AIMS: Recruitment of gut-derived memory T cells to the liver is believed to drive hepatic inflammation in primary sclerosing cholangitis (PSC). However, whether gut-infiltrating and liver-infiltrating T cells share T-cell receptors (TCRs) and antigenic specificities is unknown. We used paired gut and liver samples from PSC patients with concurrent inflammatory bowel disease (PSC-IBD), and normal tissue samples from colon cancer controls, to assess potential T-cell clonotype overlap between the two compartments...
September 16, 2016: Journal of Hepatology
Melinda J Yeh, So Yeon Kim, Kartik S Jhaveri, Spencer C Behr, Nieun Seo, Benjamin M Yeh
Autoimmune biliary diseases are poorly understood but important to recognize. Initially, autoimmune biliary diseases are asymptomatic but may lead to progressive cholestasis with associated ductopenia, portal hypertension, cirrhosis, and eventually liver failure. The three main forms of autoimmune biliary disease are primary biliary cirrhosis, primary sclerosing cholangitis, and IgG4-associated cholangitis. Although some overlap may occur between the three main autoimmune diseases of the bile ducts, each disease typically affects a distinct demographic group and requires a disease-specific diagnostic workup...
September 17, 2016: Abdominal Radiology
Ho-Su Lee, Jaewon Choe, Seon-Ok Kim, Sun-Ho Lee, Hyo Jeong Lee, Hyungil Seo, Gwang-Un Kim, Myeongsook Seo, Eun Mi Song, Sung Wook Hwang, Sang Hyoung Park, Dong-Hoon Yang, Kyung-Jo Kim, Byong Duk Ye, Jeong-Sik Byeon, Seung-Jae Myung, Yong Sik Yoon, Chang Sik Yu, Jin-Ho Kim, Suk-Kyun Yang
BACKGROUND AND AIM: Limited data are available regarding mortality from inflammatory bowel disease in non-Caucasian populations. Herein, we evaluated overall and cause-specific mortality in a hospital-based cohort of Korean inflammatory bowel disease patients. METHODS: We determined mortality in 2,414 Crohn's disease patients and 2,798 ulcerative colitis patients diagnosed between 1977 and 2013. Standardized mortality ratios were calculated in several demographic and phenotypic subgroups...
September 16, 2016: Journal of Gastroenterology and Hepatology
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