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primary cholangitis

Tatsuo Kanda, Shin Yasui, Masato Nakamura, Shingo Nakamoto, Koji Takahashi, Shuang Wu, Reina Sasaki, Yuki Haga, Sadahisa Ogasawara, Tomoko Saito, Kazufumi Kobayashi, Soichiro Kiyono, Yoshihiko Ooka, Eiichiro Suzuki, Tetsuhiro Chiba, Hitoshi Maruyama, Fumio Imazeki, Mitsuhiko Moriyama, Naoya Kato
Background: Interferon-free treatment can achieve higher sustained virological response (SVR) rates, even in patients in whom hepatitis C virus (HCV) could not be eradicated in the interferon treatment era. Immune restoration in the liver is occasionally associated with HCV infection. We examined the safety and effects of interferon-free regimens on HCV patients with autoimmune liver diseases. Results: All 7 HCV patients with autoimmune hepatitis (AIH) completed treatment and achieved SVR...
February 20, 2018: Oncotarget
Tatenda C Nzenza, Yahya Al-Habbal, Glen R Guerra, S Manolas, Tuck Yong, Trevor McQuillan
BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy (ES) has become well established as a modality for the management of common bile duct stones (CBDS), especially in the setting of associated cholangitis. Our study aims to determine the rate of long term morbidity of recurrent CBDS post ES. METHODS: A retrospective analysis of patients who underwent ERCP and ES (ERCP+ES) was undertaken on a prospectively maintained database from 1998 to 2012 at the Northern Hospital, Melbourne...
March 15, 2018: BMC Gastroenterology
Axel Andres, Aldo Montano-Loza, Russell Greiner, Max Uhlich, Ping Jin, Bret Hoehn, David Bigam, James Andrew Mark Shapiro, Norman Mark Kneteman
Deciding who should receive a liver transplant (LT) depends on both urgency and utility. Most survival scores are validated through discriminative tests, which compare predicted outcomes between patients. Assessing post-transplant survival utility is not discriminate, but should be "calibrated" to be effective. There are currently no such calibrated models. We developed and validated a novel calibrated model to predict individual survival after LT for Primary Sclerosing Cholangitis (PSC). We applied a software tool, PSSP, to adult patients in the Scientific Registry of Transplant Recipients (n = 2769) who received a LT for PSC between 2002 and 2013; this produced a model for predicting individual survival distributions for novel patients...
2018: PloS One
Sainan Bian, Hua Chen, Li Wang, Yunyun Fei, Yunjiao Yang, Linyi Peng, Yongzhe Li, Fengchun Zhang
Patients with primary biliary cholangitis (PBC) can have extrahepatic manifestations. However, data about cardiac involvement of PBC is limited. We aimed in this study to analyze the clinical characteristics in patients with PBC complicated with and without cardiac involvement, and the risk factors of cardiac involvement in PBC. PBC patients admitted to Peking Union Medical College Hospital between January 2002 and February 2016 were consecutively enrolled. Structured interview, systemic rheumatologic examination, and laboratory tests were conducted for each patient, and risk factors of cardiac involvement were analyzed by comparing patients with and without cardiac involvement...
2018: PloS One
Motoko Sasaki, Yasunori Sato, Yasuni Nakanuma
Dysregulated autophagy may be a central player in trehe pathogenesis of primary biliary cholangitis (PBC) by inducing autoimmune processes via abnormal expression of mitochondrial antigens such as pyruvate dehydrogenase complex, E2 component (PDC-E2) and also by inducing cellular senescence in biliary epithelial cells (BECs) in bile duct lesions in PBC. We examined the association of an impaired "biliary bicarbonate umbrella" due to dysfunction of anion exchanger 2 (AE2) with dysregulated autophagy and cellular senescence in PBC...
March 14, 2018: Laboratory Investigation; a Journal of Technical Methods and Pathology
Jing Li, Xiaoli Fan, Qin Wang
RATIONALE: Glycyrrhizin is the main active component of licorice. Licorice and glycyrrhizin induced hypertension has been widely reported, yet licorice and glycyrrhizin induced hypertensive crisis has been rarely known. PATIENT CONCERNS: The case of this report was a 47-year-old woman, who took 225 mg of glycyrrhizin daily for 3 years due to primary biliary cholangitis. She was found to have a dramatically elevated blood pressure of about 230/110 mmHg without a history of hypertension and was referred to the emergency department...
March 2018: Medicine (Baltimore)
Lars Bossen, Henriette Ytting, Peter Jepsen, Ole Hamberg, Peter Ott, Henning Grønbæk
The name of chronic liver disease: primary biliary cirrhosis, has been changed to: primary biliary cholangitis, primarily because of the stigma associated with the word "cirrhosis", as only a minority of the patients develop cirrhosis. In this review we present data on epidemiology and discuss the current treatments with focus on ursodeoxycholic acid and the newly described effects of the farnesoid receptor agonist obeticholic acid.
March 5, 2018: Ugeskrift for Laeger
Yoshihide Ueda, Toshimi Kaido, Hideaki Okajima, Koichiro Hata, Takayuki Anazawa, Atsushi Yoshizawa, Shintaro Yagi, Kojiro Taura, Toshihiko Masui, Noriyo Yamashiki, Hironori Haga, Miki Nagao, Hiroyuki Marusawa, Hiroshi Seno, Shinji Uemoto
Background: Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease, with liver transplantation being the sole life-saving treatment for end-stage PSC-related liver disease. However, recurrence of PSC after liver transplantation is a common complication, with the risk factors for recurrence being controversial. Methods: We conducted a retrospective chart review of 45 patients who had undergone liver transplantation for PSC at our institute...
December 2017: Transplantation Direct
Xihui Jiang, Ya Wang, Zhengyan Su, Fang Yang, Houning Lv, Lin Lin, Chao Sun
We aimed to investigate the relationship between the histologic severity and red blood cell distribution width to platelet ratio (RPR) in patients with primary biliary cholangitis (PBC). One hundred and seven consecutive patients with liver biopsy-proven and as yet treatment-naïve PBC were enrolled as the primary and validation cohort. The histologic stages were divided into early stage (Scheuer's stage 1 & 2) and late stage (Scheuer's stage 3 & 4). The overall patient demographics, clinical manifestations, hematological tests and biochemical profile were retrospectively collected from our database...
March 13, 2018: Scandinavian Journal of Clinical and Laboratory Investigation
Eleni Theocharidou, Michael A Heneghan
Autoimmune hepatitis occurs in genetically susceptible individuals as a result of loss of immunological tolerance to hepatic autoantigens that can be precipitated by environmental triggers. The clinical manifestation is usually insidious but can be also acute with liver failure. The diagnosis is made on the basis of antibody positivity, elevated immunoglobulin G levels and interface hepatitis on liver histology. Induction of remission is achieved with high-dose steroids in the majority of cases, and maintenance of remission with azathioprine...
March 2, 2018: British Journal of Hospital Medicine
Albert Parés
Primary cholangitis (cirrhosis) is a chronic cholestatic disease with an unquestionable female predominance. It is characterised by inflammation of the small and medium size bile ducts, and can eventually progress to cirrhosis. Most patients remain asymptomatic and are diagnosed by the casual finding of an anicteric biochemical cholestasis with increased alkaline phosphatase. The pathogenesis is unknown and of presumed autoimmune origin in genetic susceptible subjects. M2-type antimitochondrial antibodies, and specific antinuclear antibodies (gp210 and Sp100) are typical and specific of the disease...
March 8, 2018: Medicina Clínica
Mohamad A Mouchli, Siddharth Singh, Lisa Boardman, David H Bruining, Amy L Lightner, Charles B Rosen, Julie K Heimbach, Bashar Hasan, John J Poterucha, Kymberly D Watt, Sunanda V Kane, Laura E Raffals, Edward V Loftus
Background: The course of inflammatory bowel disease (IBD) after liver transplantation (LT) for primary sclerosing cholangitis (PSC) is poorly understood. We describe the natural history of established IBD after LT (including risk of disease progression, colectomy, and neoplasia) and de novo IBD. Methods: In a retrospective cohort, we identified all patients with PSC who underwent LT for advanced PSC at Mayo Clinic, Rochester, Minnesota. Risk factors were identified using multivariate Cox proportional hazard analysis...
March 7, 2018: Inflammatory Bowel Diseases
Martine A Aardoom, Maria E Linda Joosse, Andrica C H de Vries, Arie Levine, Lissy de Ridder
Background: Cancer and death are the most severe outcomes that affect patients with inflammatory bowel disease (IBD). These outcomes are even more severe if they occur at a young age but are rare, even in the general population. We conducted a systematic review to provide an overview of all reported pediatric (PIBD) patients with severe outcome. Methods: A literature search identified publications that reported development of cancer or fatal outcome in PIBD patients...
March 7, 2018: Inflammatory Bowel Diseases
M Tieleman, A P van den Berg, B van Hoek, W G Polak, J Dubbeld, R J Porte, C Konijn, R A de Man, B E Hanssen, H J Metselaar
OBJECTIVE: To calculate the chance of receiving a liver transplant for patients on the liver transplant waiting list in the Netherlands. DESIGN: Retrospective cohort research. METHOD: Data of all patients in the Netherlands on the waiting list for liver transplantation, from the introduction of the model of end-stage liver disease score on 16th December 2006 through to 31st December 2013 were collected. Survival analysis was computed with competing risk analyses...
2018: Nederlands Tijdschrift Voor Geneeskunde
Ifeyinwa E Obiorah, Alicia Henao Velasquez, Bhaskar Kallakury, Metin Özdemirli
BACKGROUND: Langerhans cell histiocytosis is characterized by abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population and presentation in adults remain a rare event. The presentation of langerhans cell histiocytosis is highly variable but skin, bone and lung involvement are very common. Langerhans cell histiocytosis presenting as a bile duct mass is rare and usually occurs as part of a multi-organ system disease. CASE REPORT: We present a case of langerhans cell histiocytosis confined to the extrahepatic bile duct in a 62-year-old female with sclerosing cholangitis...
March 8, 2018: Balkan Medical Journal
Sarah Keller, Annette Aigner, Roman Zenouzi, Anne C Kim, Arnoud Meijer, Sören A Weidemann, Till Krech, Ansgar W Lohse, Gerhard Adam, Christoph Schramm, Jin Yamamura
OBJECTIVE: To evaluate magnetic resonance imaging (MRI) parameters T2 signal, contrast enhancement (CE), and relative liver enhancement (RLE) of extracellular gadolinium-based contrast agent (GBCA)-enhanced MRI as a marker for hepatic fibrosis and inflammation in patients with primary sclerosing cholangitis (PSC). METHODS: 3.0-Tesla MRI scans and liver biopsies of 40 patients (41.2 ± 17.1 years) were retrospectively reviewed. Biopsies were obtained within a mean time of 54 ± 55 days to MRI scans and specimens were categorized according to Ishak modified hepatic activity index (mHAI) and Scheuer staging of fibrosis...
2018: PloS One
Christian Rupp, Alexander Rössler, Taotao Zhou, Conrad Rauber, Kilian Friedrich, Andreas Wannhoff, Karl-Heinz Weiss, Peter Sauer, Peter Schirmacher, Caner Süsal, Wolfgang Stremmel, Daniel N Gotthardt
Background: The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30-40 years. Objective: We aimed to analyse disease progression and liver-dependent survival in patients diagnosed with PSC after 50 years of age. Methods: Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late-onset group. Results: A total of 32/215 (14...
March 2018: United European Gastroenterology Journal
Wei Zhang, Debapriya De, Kahee A Mohammed, Satish Munigala, Guilan Chen, Jin-Ping Lai, Bruce R Bacon
Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune-mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histologic features of AIH and PBC along with modifications of biochemical and immunologic characteristics...
March 2018: Hepatology communications
Urs Christen, Edith Hintermann
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are serious autoimmune liver diseases that are characterized by a progressive destruction of the liver parenchyma and/or the hepatic bile ducts and the development of chronic fibrosis. Left untreated autoimmune liver diseases are often life-threatening, and patients require a liver transplantation to survive. Thus, an early and reliable diagnosis is paramount for the initiation of a proper therapy with immunosuppressive and/or anticholelithic drugs...
2018: Frontiers in Immunology
Melahat Kul, Diğdem Kuru Öz, Ayşe Erden
No abstract text is available yet for this article.
March 3, 2018: Abdominal Radiology
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