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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
Deniz Koksal, Aydin Seref Koksal, Ahmet Gurakar
Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC...
September 28, 2016: Journal of Clinical and Translational Hepatology
Lian Gu, Jinying Zhou, Jinjing Tan, Junwei Yang, Tingting Shen, Haiyun Jiang, Qianli Tang
Inflammatory response and lipid metabolism influence the development of ischemic stroke (IS). TLRs have an important function in inflammation and lipid metabolism. To investigate association of TLR8 rs3764880 polymorphism with susceptibility of IS and the relationship between rs3764880 with inflammation response- and lipid metabolism-related quantitative traits, we conducted a case-control study in a Han Chinese population comprising 816 cases and 816 controls being matched for age and gender. The distribution of allele of TLR8 rs3764880 had statistical significance in male group (P=0...
November 15, 2016: Journal of the Neurological Sciences
Ching-Fang Lee, I-Chyun Chiang, Fang-Ming Hwang, Li-Kang Chi, Hsien-Ming Lin
OBJECTIVE: the objectives of this study were to use the Theory of Planned Behavior (TPB) in order to determine to what degree pregnant women intended to exercise regularly during pregnancy, and then to evaluate the TPB model fit. DESIGN: this study was based on a cross-sectional survey with a tool which was developed specifically for this study. SETTING: we identified and invited pregnant women from two medical center hospitals and one certified prenatal center in northern Taiwan to participate...
October 5, 2016: Midwifery
Andres M Alvarez-Pinzon, Aizik L Wolf, Heather N Swedberg, Kory A Barkley, Juan Cucalon, Luciana Curia, Jose E Valerio
OBJECTIVE: The purpose of this retrospective study was to compare and evaluate Percutaneous Retrogasserian Balloon Compression (PBC) and Gamma Knife Radiosurgery (GKRS) for the treatment of trigeminal neuralgia (TN) in patients with multiple sclerosis (MS). METHODS: In this single-center, retrospective comparative study, 202 patients with MS and concomitant TN were evaluated. A minimum follow-up of 24 months was required. Patients with a history of microvascular decompression or previous intervention were excluded...
October 15, 2016: World Neurosurgery
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
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
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
Antonio B Porcaro, Nicolò de Luyk, Paolo Corsi, Marco Sebben, Alessandro Tafuri, Irene Tamanini, Tania Processali, Maria A Cerruto, Filippo Migliorini, Matteo Brunelli, Salvatore Siracusano, Walter Artibani
AIM: To determine clinical factors associated with multiple bilateral lymph node micrometastases and seminal vesicle invasion (pT3b) in organ-confined prostate cancer (PCa). METHODS: The study excluded patients under androgen deprivation, with lymph node involvement (cN1 status), and having undergone unilateral pelvic lymph node dissection (PLND) during radical prostatectomy (RP). Lymph node micrometastases were classified as unilateral (pN1m) and bilateral (pN1b)...
September 27, 2016: Tumori
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
Yuan He, Fan Yang, Dongqin Mu, Yuan Xing, Xin Li
OBJECTIVES: Main study aim was as follows: (1) to explore the usefulness of the theory of planned behaviour (TPB) model in predicting Chinese hospital pharmacists' intention to provide clinical pharmacy services (CPSs), including auxiliary CPSs and core CPSs; (2) to identify the main factors affecting the Chinese hospital pharmacists' intention to provide core CPSs based on TPB quantitatively. DESIGN: Cross-sectional questionnaire study. SETTING: The study was conducted in 22 general hospitals in seven cities located in the eastern and western part of China...
October 5, 2016: BMJ Open
Bruno Baudin, Arnaud Bruneel, Raoul Poupon, Michel Vaubourdolle
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease considered as an autoimmune disease. To identify new biomarkers of PBC, serum profiling analysis using Surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF-MS) was employed. Twelve patients with either asymptomatic PBC (group 1, n=6) or PBC with a poor response to UDCA (group 2, n=6), were compared to healthy controls (group 3, n=6). Analysing the 18 sera by using four SELDI-TOF arrays under various conditions, we found four biomarkers of PBC at 5...
October 1, 2016: Annales de Biologie Clinique
Christopher L Bowlus, James T Kenney, Gary Rice, Robert Navarro
BACKGROUND: Chronic liver disease and cirrhosis are a leading cause of morbidity and mortality in the United States. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis and which has been designated an orphan condition, is a chronic autoimmune disease resulting in the destruction of the small bile ducts in the liver. Without effective treatment, disease progression frequently leads to liver failure and death. Until May 2016, the only FDA-approved treatment for PBC was ursodiol (UDCA), an oral hydrophilic bile acid, which can slow progression of liver damage due to PBC...
October 2016: Journal of Managed Care & Specialty Pharmacy
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
Géraldine Dahlqvist, Farid Gaouar, Fabrice Carrat, Sofia Meurisse, Olivier Chazouillères, Raoul Poupon, Catherine Johanet, Christophe Corpechot
: The prevalence, clinical characteristics and outcomes of patients with antimitochondrial antibodies (AMA) but no clinical evidence of primary biliary cholangitis (PBC) are largely unknown. A prospective study of AMA incidence was conducted through a nationwide network of 63 French immunology laboratories. Clinical data from 720 out of 1318 AMA-positive patients identified in one year were collected. The patients were categorized as either newly diagnosed with PBC (n=275), previously diagnosed with PBC (n=216), or with non-established diagnosis of PBC (n=229)...
September 30, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Ying Wang, Xiyu Ke, Zhi Xiang Voo, Serene Si Ling Yap, Chuan Yang, Shujun Gao, Shaoqiong Liu, Shrinivas Venkataraman, Sybil Akua Okyerewa Obuobi, Jasmeet Singh Khara, Yi Yan Yang, Pui Lai Rachel Ee
: Amphotericin B (AmB), a poorly soluble and toxic antifungal drug, was encapsulated into polymeric micelles self-assembled from phenylboronic acid-functionalized polycarbonate/PEG (PEG-PBC) and urea-functionalized polycarbonate/PEG (PEG-PUC) diblock copolymers via hydrogen-bonding, boronate ester bond, and/or ionic interactions between the boronic acid group in the micellar core and amine group in AmB. Three micellar formulations were prepared: AmB/B micelles using PEG-PBC, AmB/U micelles using PEG-PUC and AmB/B+U mixed micelles using 1:1molar ratio of PEG-PBC and PEG-PUC...
September 26, 2016: Acta Biomaterialia
Marta B Afonso, Pedro M Rodrigues, André L Simão, Dimitry Ofengeim, Tânia Carvalho, Joana D Amaral, Maria M Gaspar, Helena Cortez-Pinto, Rui E Castro, Junying Yuan, Cecília M P Rodrigues
Cholestasis encompasses liver injury and inflammation. Necroptosis, a necrotic cell death pathway regulated by receptor-interacting protein (RIP) 3, may mediate cell death and inflammation in the liver. We aimed to investigate the role of necroptosis in mediating deleterious processes associated with cholestatic liver disease. Hallmarks of necroptosis were evaluated in liver biopsies of primary biliary cholangitis (PBC) patients and in wild-type and RIP3-deficient (RIP3(-/-)) mice subjected to common bile duct ligation (BDL)...
2016: Cell Death & Disease
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