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primary cholangitis

Annarosa Floreani, Chiara Mangini, Anna Reig, Irene Franceschet, Nora Cazzagon, Lisa Perini, Llorenç Caballería, Silvia Cocchio, Vincenzo Baldo, Albert Parés
OBJECTIVES: Primary biliary cholangitis (PBC) is often associated with other autoimmune diseases, but little is known about the influence of thyroid disease (TD) on the natural history of PBC. Our aim is to analyze the association between PBC and TD, and the latter's impact on the natural history of PBC at two European centers. METHODS: The study involved 921 PBC patients enrolled between 1975 and 2015 in Padova (376 patients) and Barcelona (545 patients), with a mean follow-up of 126...
October 25, 2016: American Journal of Gastroenterology
Marcin Krawczyk, Roman Liebe, Michał Wasilewicz, Ewa Wunsch, Joanna Raszeja-Wyszomirska, Piotr Milkiewicz
BACKGROUND & AIMS: The amelioration of refractory cholestatic pruritus after plasmapheresis has been reported in single patients. Here we analyse the efficacy of plasmapheresis in a cohort of patients with primary biliary cholangitis (PBC). METHODS: Seventeen consecutive patients with PBC (age range 39-85 years, 16 females, 9 with cirrhosis) with refractory pruritus underwent 129 plasmapheresis procedures during 40 admissions. Pruritus was quantified by the 10 point numeric rating scale (NRS) before and after plasmapheresis, as well as ~30 and ~90 days later...
October 25, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
B Eksteen, J Heatherington, J Oshiomogo, R Panaccione, G Kaplan, S Ghosh
This article has been retracted: please see Elsevier Policy on Article Withdrawal ( The abstract authors and presenters of PS124 - EFFICACY AND SAFETY OF INDUCTION DOSING OF VEDOLIZUMAB FOR REDUCING BILIARY INFLAMMATION IN PRIMARY SCLEROSING CHOLANGITIS (PSC) IN INDIVIDUALS WITH INFLAMMATORY BOWEL DISEASE submitted and presented at ILC 2016 have raised concerns that the source data in some cases are inconsistent and requires further evaluation to determine the true magnitude of effect...
2016: Journal of Hepatology
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
Michele B Kaufman
Obeticholic acid (Ocaliva) for primary biliary cholangitis; sofosbuvir 400 mg/velpatasvir 100 mg (Epclusa) for chronic hepatitis C virus infection; and daclizumab (Zinbryta) for relapsing multiple sclerosis.
October 2016: P & T: a Peer-reviewed Journal for Formulary Management
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
Tadashi Namisaki, Kei Moriya, Mitsuteru Kitade, Hideto Kawaratani, Kosuke Takeda, Yasushi Okura, Hiroaki Takaya, Norihisa Nishimura, Kenichiro Seki, Kosuke Kaji, Shinya Sato, Yasuhiko Sawada, Junichi Yamao, Akira Mitoro, Masakazu Uejima, Tsuyoshi Mashitani, Naotaka Shimozato, Keisuke Nakanishi, Masanori Furukawa, Soichiro Saikawa, Takuya Kubo, Hitoshi Yoshiji
BACKGROUND: Inadequate response to ursodeoxycholic acid (UDCA) is associated with unfavorable outcomes in patients with primary biliary cholangitis (PBC). We aimed to identify surrogate markers for predicting long-term prognosis and biochemical response to UDCA in patients with PBC. PATIENTS AND METHODS: In this single-center, retrospective study, 99 patients with PBC were classified into responders (n=53) and nonresponders (n=46) based on reductions in the γ-glutamyl transpeptidase levels at 1 year after initiating UDCA therapy (Nara criteria)...
October 17, 2016: European Journal of Gastroenterology & Hepatology
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
Boyang Zheng, Catherine Vincent, Marvin J Fritzler, Jean-Luc Senécal, Martial Koenig, France Joyal
OBJECTIVE: Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria...
October 15, 2016: Journal of Rheumatology
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
Bi-Jhen Syu, Chia-En Loh, Yu-Hsin Hsueh, M Eric Gershwin, Ya-Hui Chuang
Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease with a long natural history. The pathogenesis of PBC is thought to be orchestrated by Th1 and/or Th17. In this study, we investigated the role of CD4(+) helper T subsets and their cytokines on PBC using our previous established murine model of 2-OA-OVA immunization. We prepared adeno-associated virus (AAV)-IFN-γ and AAV-IL-4 and studied their individual influences on the natural history of autoimmune cholangitis in this model. Administration of IFN-γ significantly promotes recruitment and lymphocyte activation in the earliest phases of autoimmune cholangitis but subsequently leads to downregulation of chronic inflammation through induction of the immunosuppressive molecule IL-30...
October 10, 2016: Scientific Reports
Nicholas L Webb, Ashish Jiwane, Chee Y Ooi, Scott Nightinghale, Susan E Adams, Usha Krishnan
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014...
October 7, 2016: Journal of Paediatrics and Child Health
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
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