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Primary sclerosing cholangitis

Lars Bossen, Alessio Gerussi, Vasiliki Lygoura, George F Mells, Marco Carbone, Pietro Invernizzi
Autoimmune liver diseases (AILDs) are complex conditions, which arise from the interaction between a genetic susceptibility and unknown environmental triggers. They represent a relevant cause of liver failure and liver transplantation worldwide. As a testimony of our progress in understanding the biology of AILDs and the disease progression is the overall median survival which has increased over the last decade. However, there are still major challenges such as the lack of therapies and surveillance strategies in primary sclerosing cholangitis (PSC), the management and treatment of non-responders to first-line therapies in primary biliary cholangitis (PBC) and the need for tailoring immunosuppressive drugs in autoimmune hepatitis (AIH)...
July 10, 2018: Autoimmunity Reviews
Carlie S Sigel, Esther Drill, Yi Zhou, Olca Basturk, Gokce Askan, Linda M Pak, Efsevia Vakiani, Tao Wang, Thomas Boerner, Richard K G Do, Amber L Simpson, William Jarnagin, David S Klimstra
Intrahepatic cholangiocarcinomas are histologically heterogenous. Using a cohort of 184 clinically defined, resected intrahepatic cholangiocarcinomas, we retrospectively classified the histology into 4 subtypes: large duct (LD), small duct (SD) (predominantly tubular [SD1] or predominantly anastomosing/cholangiolar, [SD2]), or indeterminate. Then, we tested the 4 subtypes for associations with risk factors, patient outcomes, histology, and immunophenotypic characteristics. SD was the most common (84%; 24% SD1 and 60% SD2) with lower proportions of LD (8%), and indeterminate (8%)...
July 11, 2018: American Journal of Surgical Pathology
James H Tabibian, Ahmad H Ali, Keith D Lindor
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholangiopathy that can progress to cirrhosis, end-stage liver disease, hepatobiliary cancer, and/or colorectal cancer. The course of PSC is often complicated by portal hypertension, symptoms of cholestasis, and recurrent bacterial cholangitis, among other conditions, with a consequent decrease in survival (median, approximately 20 years) and quality of life. The etiopathogenesis of PSC remains poorly understood, and, as such, pharmacotherapy has yet to be definitively established...
May 2018: Gastroenterology & Hepatology
Yong Eun Park, Jae Hee Cheon, Jae Jun Park, Yoon Jae Kim, Chang Hwan Choi, Yehyun Park, Soo Jung Park, Tae Il Kim, Won Ho Kim
BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare progressive cholestatic liver disease of unknown causes, but is strongly associated with inflammatory bowel diseases (IBDs), particularly ulcerative colitis (UC). However, studies comparing risk factors and clinical courses of patients with concomitant UC and PSC with those of patients with PSC alone are lacking. METHODS: We retrospectively reviewed patients with PSC diagnosed between 2005 and 2017 in four tertiary hospitals in Korea...
July 9, 2018: International Journal of Colorectal Disease
M E Joosse, M A Aardoom, P Kemos, D Turner, D C Wilson, S Koletzko, J Martin-de-Carpi, U L Fagerberg, C Spray, C Tzivinikos, M Sladek, R Shaoul, E Roma-Giannikou, J Bronsky, D E Serban, F M Ruemmele, H Garnier-Lengline, G Veres, I Hojsak, K L Kolho, I H Davies, M Aloi, P Lionetti, S Hussey, G Veereman, C P Braegger, E Trindade, A V Wewer, A C Hauer, A C H de Vries, R Sigall Boneh, C Sarbagili Shabat, A Levine, L de Ridder
BACKGROUND: Risk benefit strategies in managing inflammatory bowel diseases (IBD) are dependent upon understanding the risks of uncontrolled inflammation vs those of treatments. Malignancy and mortality in IBD have been associated with disease-related inflammation and immune suppression, but data are limited due to their rare occurrence. AIM: To identify and describe the most common causes of mortality, types of cancer and previous or current therapy among children and young adults with paediatric-onset IBD...
July 8, 2018: Alimentary Pharmacology & Therapeutics
(no author information available yet)
No abstract text is available yet for this article.
July 5, 2018: Inflammatory Bowel Diseases
James H Tabibian, Christopher L Bowlus
Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibro-obliterative inflammation of the bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and/or hepatobiliary cancer, though the disease course can be highly variable. Despite clinical trials of numerous pharmacotherapies over several decades, safe and effective medical therapy remains to be established...
December 2017: Liver Research
Fatima Isa, Grace M Turner, Geetinder Kaur, Derek Kyte, Anita Slade, Tanya Pankhurst, Larissa Kerecuk, Thomas Keeley, James Ferguson, Melanie Calvert
BACKGROUND: Primary Sclerosing Cholangitis (PSC) is a rare chronic, cholestatic liver condition in which patients can experience a range of debilitating symptoms. Patient reported outcome measures (PROMs) could provide a valuable insight into the impact of PSC on patient quality of life and symptoms. A previous review has been conducted on the quality of life instruments used in liver transplant recipients. However, there has been no comprehensive review evaluating PROM use or measurement properties in PSC patients' to-date...
July 5, 2018: Health and Quality of Life Outcomes
Marinos Pericleous, Claire Kelly, Aftab Ala, Simon De Lusignan
INTRODUCTION: The chronic care model (CCM) provides a holistic approach for managing chronic illnesses. Patients with rare liver diseases (RLD) have complex needs, impaired quality of life and often life-threatening complications. Most RLD meet the criteria for a long-term chronic condition and should be viewed through the prism of CCM. We aimed to ascertain whether the CCM has been considered for the frequently-encountered RLD. METHODS: MEDLINE®/PubMed®/Cochrane/EMBASE were searched to identify publications relating to the use of the CCM for the management of six RLD...
July 13, 2018: Expert Review of Gastroenterology & Hepatology
E Veitsman, E Pras, O Pappo, A Arish, R Eshkenazi, C Feray, J Calderaro, D Azoulay, Z Ben Ari
Hereditary hemochromatosis (HH) is a genetic disease associated with progressive iron overload, eventually leading in some cases to damage of parenchymal organs, such as the liver, pancreas, and heart. Although the gene had been identified (HFE), HH pathogenesis remains to be fully elucidated. We report here, for the first time, a case of inadvertent transplantation of a liver from a donor with C282Y/H63D compound heterozygosity into a nonhemochromatotic 19-year-old Caucasian male recipient with primary sclerosing cholangitis...
2018: Case Reports in Hepatology
Hiroyuki Isayama, Susumu Tazuma, Norihiro Kokudo, Atsushi Tanaka, Toshio Tsuyuguchi, Takahiro Nakazawa, Kenji Notohara, Suguru Mizuno, Nobuhisa Akamatsu, Masahiro Serikawa, Itaru Naitoh, Yoshiki Hirooka, Toshifumi Wakai, Takao Itoi, Tomoki Ebata, Shinji Okaniwa, Terumi Kamisawa, Hiroki Kawashima, Atsushi Kanno, Keiichi Kubota, Masami Tabata, Michiaki Unno, Hajime Takikawa
BACKGROUND: Primary sclerosing cholangitis (PSC) is relatively rare disease and pathogenesis and methods of treatments were still not established. Then, we had conducted the making clinical guidelines to manage patients with PSC based on the literature review and expert opinions. These clinical guidelines were made for the medical doctors on the management of PSC, except child case of PSC. METHODS: We had employed modified Delphi method. The production committee decided guidelines, strength of recommendations and evidence level after reviewed literatures systematically, and The Expert panel evaluated those...
June 27, 2018: Journal of Gastroenterology
Ashish Joshi, Sushil Falodia, Naveen Kumar, Pawan Gupta, P C Khatri
BACKGROUND: Liver involvement in celiac disease (CD) is classified into autoimmune and cryptogenic. The association between CD and autoimmune liver diseases like autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis is well-established; however, the data on patients with cryptogenic cirrhosis, particularly from India, are scanty. So we did this study to find the prevalence of CD in patients with cryptogenic cirrhosis. METHODS: This was a prospective observational study, involving children of less than 18 years old attending Pediatric and Gastroenterology clinic with a diagnosis of cryptogenic cirrhosis...
June 13, 2018: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Katharina Staufer, Danijel Kivaranovic, Susanne Rasoul-Rockenschaub, Thomas Soliman, Michael Trauner, Gabriela Berlakovich
BACKGROUND: This study investigated the impact of Model of end-stage liver disease (MELD)-score introduction (MELDi) on waitlist mortality and post-liver transplant (LT) survival in primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). METHODS: LT candidates with PSC or PBC listed between January 1983 and March 2016 were included and followed until December 2016. After MELDi in 2004, PBC patients were listed according to labMELD, PSC patients according to the highest MELD during active cholangitis (chMELD)...
June 21, 2018: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Amy Tyberg, Ming-Ming Xu, Monica Gaidhane, Michel Kahaleh
INTRODUCTION: Evaluation of indeterminate biliary strictures remains a diagnostic challenge. Optical coherence tomography (OCT) provides in-vivo, wide-field, cross-sectional imaging at the microstructure level. We present the first preliminary data using a second-generation OCT system using volumetric laser endomicroscopy (VLE) in biliary and pancreatic duct strictures. METHODS: 10 consecutive patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) and OCT for indeterminate biliary or pancreatic stricture evaluation were captured in a registry...
June 8, 2018: Digestive and Liver Disease
Allyson Wyatt, Richard Kellermayer
No abstract text is available yet for this article.
June 20, 2018: Journal of Crohn's & Colitis
Kilian Friedrich, Carina Baumann, Andreas Wannhoff, Christian Rupp, Arianeb Mehrabi, Karl Heinz Weiss, Daniel N Gotthardt
BACKGROUND AND AIMS: The disease course of primary sclerosing cholangitis (PSC) is variable and difficult to predict. MicroRNA-122 (miR-122) is associated with various liver diseases. We investigated the value of miR-122 as a biomarker for the disease course of PSC. METHODS: We determined serum miR-122 levels in a long-term, prospective cohort of 114 PSC patients and a second validation cohort. RESULTS: Based on miR-122 levels, PSC patients were assigned to low or high level miR-122 groups...
June 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
Juan E Corral, Omar Y Mousa, Murli Krishna, Iris J M Levink, Khela R Pursell, Mohammad Afsh, Paul T Kröner, Denise M Harnois, Herbert C Wolfsen, Michael B Wallace, Frank J Lukens
BACKGROUND:  Volumetric laser endomicroscopy (VLE) provides circumferential images 3 mm into the biliary and pancreatic ducts. We aimed to correlate VLE images with the normal and abnormal microstructure of these ducts. METHODS: Samples from patients undergoing hepatic or pancreatic resection were evaluated. VLE images were collected using a low-profile VLE catheter inserted manually into the biliary and pancreatic ducts ex vivo. Histological correlation was assessed by two unblinded investigators...
June 18, 2018: Endoscopy
Daisuke Imai, Tomoharu Yoshizumi, Kazuhito Sakata, Toru Ikegami, Shinji Itoh, Noboru Harada, Takashi Motomura, Takeo Toshima, Yohei Mano, Yuji Soejima, Yoshihiko Maehara
BACKGROUND: Although risk factors for the long-term mortality of liver transplantation are well described, there is a lack of detailed study regarding these factors for adult living donor liver transplantation (LDLT). METHODS: We retrospectively analyzed 528 adult LDLT recipients in our hospital. The risk factors were analyzed for overall deaths >5 years post-LDLT. RESULTS: Over the 20-year follow-up, 137 patients died. Patient survival at 1, 3, 5, and 10 years post-LDLT was 87...
June 13, 2018: Transplantation
Andrea Tenca, Harri Mustonen, Kati Lind, Eila Lantto, Kaija-Leena Kolho, Sonja Boyd, Johanna Arola, Kalle Jokelainen, Martti Färkkilä
BACKGROUND AND AIMS: Endoscopic retrograde cholangiography (ERCP) has been considered the gold standard for the diagnosis and follow-up of primary sclerosing cholangitis, but it has been replaced by less invasive magnetic resonance imaging and cholangio-pancreatography (MRI-MRCP). However, the role of these two techniques in the evaluation of disease activity and severity needs to be elucidated. METHODS: Patients with primary sclerosing cholangitis (n: 48, male 31, median age: 35...
June 14, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Takashi Hashimoto, Jordan D Rosen, Kristen M Sanders, Gil Yosipovitch
Basophils are blood granulocytes and normally constitute less than 1% of blood peripheral leukocytes. Basophils share some morphological and functional similarities with mast cells, and basophils were once regarded as redundant and negligible circulating mast cells. However, recent studies reveal the indispensable roles of basophils in various diseases, including allergic and pruritic diseases. Basophils may be involved in itch through the mediation of a Th2 immune response, interaction with other cells in the skin, and secretion of a wide variety of itch-related mediators, e...
June 12, 2018: Experimental Dermatology
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