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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
Ashwin Rammohan, Sripriya Srinivas, Yahya Al Azri, Mettu Srinivas Reddy, Mohamed Rela
Roux-en-Y (RY) hepaticojejunostomy (HJ) is a critical component of complex hepatobiliary operations, and it is the treatment of choice for post-cholecystectomy biliary strictures (PCBS). Complications related to the Roux limb, particularly those leading to cholangitis, can severely compromise outcomes. We present a case of a 54-year-old lady who had previously undergone two HJ procedures for recurrent PCBS. The same Roux limb had been used in each of the operations. Short Roux/Roux reflux syndrome led to recurrent cholangitis in this patient, as proven by the reflux of oral contrast into the biliary tree on imaging...
February 2018: Annals of Hepato-Biliary-Pancreatic Surgery
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
Akihisa Kato, Itaru Naitoh, Katsuyuki Miyabe, Kazuki Hayashi, Hiromu Kondo, Michihiro Yoshida, Hiroyuki Kato, Toshiya Kuno, Satoru Takahashi, Takashi Joh
BACKGROUND: Fluorescence in situ hybridization (FISH) of cytology specimens has been used to diagnose biliary strictures. However, the usefulness of FISH for differentiating between cholangiocarcinoma (CCA) and IgG4-related sclerosing cholangitis (IgG4-SC) has not been evaluated in forceps biopsy specimens. METHODS: We retrospectively reviewed 74 specimens obtained by transpapillary forceps biopsy between 2008 and 2015 from 49 consecutive patients with CCA and 25 with IgG4-SC...
March 2018: Journal of Hepato-biliary-pancreatic Sciences
Lucy Meunier, Jose Ursic-Bedoya, Georges-Philippe Pageaux, Dominique Larrey
BACKGROUND: Although very rare, pantoprazole can result in acute hepatitis. It has yet to be reported, however, that it can also cause chronic autoimmune hepatitis. AIM, METHOD, AND RESULTS: We report the case of a patient in whom pantoprazole administration for two months was followed by acute liver injury with severe jaundice and features of autoimmunity. A liver biopsy revealed acute hepatocellular lesions associated with cholestasis, acute cholangitis, and polymorphous inflammatory infiltration suggestive of drug-induced liver injury...
March 12, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Felicia Turowski, Ulrich Hügle, Arno Dormann, Matthias Bechtler, Ralf Jakobs, Uwe Gottschalk, Ellen Nötzel, Dirk Hartmann, Albrecht Lorenz, Frank Kolligs, Wilfried Veltzke-Schlieker, Andreas Adler, Olaf Becker, Bertram Wiedenmann, Nataly Bürgel, Hanno Tröger, Michael Schumann, Severin Daum, Britta Siegmund, Christian Bojarski
BACKGROUND AND AIMS: The aim of the study was to evaluate the usefulness and diagnostic and therapeutic outcome of the single-operator cholangiopancreatoscopy (SOC) with SpyGlassDS™. METHODS: In a retrospective multicenter study between November 2015 and January 2017, SpyGlassDS™ procedures were analyzed in participating centers. Indications, accuracy of SOC-guided biopsies, management of large bile duct stones, and complications were analyzed. Follow-up was 4 months...
March 12, 2018: Surgical Endoscopy
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
Bibigul Ilyassova, Yevgeny Yenin, Bolatbek Baymakhanov, Maksat Doskhanov
OBJECTIVES: At the National Scientific Center of Surgery named A. Syzganova, more than 100 liver transplants have been conducted from 2012 to November 2017. Survival and quality of life of patients in the posttransplant period largely depend on the compliance of patients, monitoring of complications, cause of liver disease, the severity of the condition of patients in the pretransplant period, and the technical features of the operation. The aims of the study were to analyze the results of liver biopsy in patients after liver transplant and to evaluate the value of morphologic studies of the liver for the diagnosis of complications in the posttransplant period...
March 2018: Experimental and Clinical Transplantation
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
Hajime Okada, Minami Yamada, Kenji Kamimoto, Cindy Yuet-Yin Kok, Kota Kaneko, Masatsugu Ema, Atsushi Miyajima, Tohru Itoh
Under various conditions of liver injury, the intrahepatic biliary epithelium undergoes dynamic tissue expansion and remodeling, a process known as ductular reaction. Mouse models defective in inducing such a tissue-remodeling process are more susceptible to liver injury, suggesting a crucial role of this process in liver regeneration. However, the molecular mechanisms regulating the biliary epithelial cell (BEC) dynamics in the ductular reaction remain largely unclear. Here, we demonstrate that the transcription factor Krüppel-like factor 5 (Klf5) is highly enriched in mouse liver BECs and plays a key role in regulating the ductular reaction, specifically under cholestatic injury conditions...
March 9, 2018: Journal of Biological Chemistry
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
Tao Wang, Hao Zou, Yun-Xia Liu, Xiao-Wen Zhang
Background: Paclitaxel (PTX) could inhibit the growth of fibroblasts, which occurs in proliferative cholangitis and leads to biliary stricture. However, its use has been limited due to poor bioavailability and local administration for short time. This study designed and synthesized a new PTX-conjugated chitosan film (N-succinyl-hydroxyethyl chitosan containing PTX [PTX-SHEC]) and evaluated its safety and efficiency using in vivo and in vitro experiments. Methods: The SHEC conjugated with PTX was confirmed by nuclear magnetic resonance (NMR) and Fourier-transform infrared spectroscopy (FT-IR) measurements...
March 20, 2018: Chinese Medical Journal
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