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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/28935574/taurocholate-induces-biliary-differentiation-of-liver-progenitor-cells-causing-hepatic-stellate-cell-chemotaxis-in-the-ductular-reaction-role-in-pediatric-cystic-fibrosis-liver-disease
#1
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia Sm Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina Ee Tirnitz-Parker, John K Olynyk, Ross W Shepherd, Peter J Lewindon, Grant A Ramm
Cystic fibrosis liver disease (CFLD) in children causes progressive fibrosis leading to biliary cirrhosis, however its cause(s) and early pathogenesis are unclear. We hypothesised that a bile acid-induced Ductular Reaction (DR) drives fibrogenesis. We evaluated the DR by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD and demonstrated that the DR was significantly correlated with hepatic fibrosis stage and biliary taurocholate levels. To examine the mechanisms involved in DR induction liver progenitor cells (LPCs) were treated with taurocholate and key events in DR evolution were assessed: LPC proliferation, LPC biliary differentiation and hepatic stellate cell (HSC) chemotaxis...
September 18, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28933417/serum-wisteria-floribunda-agglutinin-positive-mac-2-binding-protein-could-not-always-predict-early-cirrhosis-in-non-viral-liver-diseases
#2
Yuki Haga, Tatsuo Kanda, Reina Sasaki, Masato Nakamura, Koji Takahashi, Shuang Wu, Shin Yasui, Makoto Arai, Shingo Nakamoto, Osamu Yokosuka
BACKGROUND: Wisteria floribunda agglutinin-positive human Mac-2-binding protein (WFA(+)-M2BP) is a novel non-invasive marker of liver fibrosis. The goal of the study was to investigate whether the novel serum biomarker WFA(+)-M2BP or other non-invasive markers are useful for the prediction of liver fibrosis in patients with nonalcoholic steatohepatitis (NASH), autoimmune hepatitis (AIH), and primary biliary cholangitis (PBC). METHODS: We examined a significant correlation between serum WFA(+)-M2BP levels and histological staging of fibrosis in several chronic liver diseases, such as NASH, AIH, and PBC...
December 14, 2016: Diseases (Basel)
https://www.readbyqxmd.com/read/28922436/genetic-risk-factors-for-autoimmune-thyroid-disease-might-affect-the-susceptibility-to-and-modulate-the-progression-of-primary-biliary-cholangitis
#3
Aleksander Kuś, Magdalena Arłukowicz-Grabowska, Konrad Szymański, Ewa Wunsch, Małgorzata Milkiewicz, Rafał Płoski, Zakera Shums, Gary L Norman, Piotr Milkiewicz, Tomasz Bednarczuk, Marcin Krawczyk
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD). METHODS: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#4
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#5
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913620/a-brief-review-on-prognostic-models-of-primary-biliary-cholangitis
#6
REVIEW
Sha Chen, Weijia Duan, Hong You, Jidong Jia
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016...
September 14, 2017: Hepatology International
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#7
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28892963/autoimmune-hepatitis-primary-biliary-cirrhosis-overlap-syndrome
#8
Indira Bairy, Anupam Berwal, Shubha Seshadri
Autoimmune Hepatitis (AIH) and Primary Biliary Cirrhosis (PBC) are important immune mediated liver diseases. They are usually differentiated based on clinical, biochemical, serological and histological parameters. The presence of autoantibodies, clinical and serological findings can sometimes occur in different combinations leading to overlap syndromes, which is rare. Early recognition of such overlap syndromes is clinically significant from treatment point of view. Here, we report a case of AIH-PBC overlap syndrome with a brief review of literature on overlap syndromes...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28882625/drug-induced-bile-duct-injury
#9
REVIEW
Michele Visentin, Daniela Lenggenhager, Zhibo Gai, Gerd A Kullak-Ublick
Drug-induced liver injury includes a spectrum of pathologies, some related to the mode of injury, some to the cell type primarily damaged. Among these, drug-induced bile duct injury is characterized by the destruction of the biliary epithelium following exposure to a drug. Most of the drugs associated with bile duct injury cause immune-mediated lesions to the epithelium of interlobular ducts. These share common histopathological features with primary biliary cholangitis, such as inflammation and necrosis at the expense of cholangiocytes and, if the insult persists, bile duct loss and biliary cirrhosis...
September 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28860789/new-developments-in-the-treatment-of-primary-biliary-cholangitis-role-of-obeticholic-acid
#10
REVIEW
Manan A Jhaveri, Kris V Kowdley
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease that predominantly affects women in early to middle age. It is typically associated with autoantibodies to mitochondrial antigens and results in immune-mediated destruction of small and medium-sized intrahepatic bile ducts leading to cholestasis, hepatic fibrosis and may progress to cirrhosis or hepatic failure and, in some cases, hepatocellular carcinoma. The clinical presentation and the natural history of PBC have improved over the years due to recognition of earlier widespread use of ursodeoxycholic acid (UDCA); about one-third of patients show suboptimal biochemical response to UDCA with poor prognosis...
2017: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/28858291/epidemiology-and-natural-history-of-primary-biliary-cholangitis-in-the-chinese-a-territory-based-study-in-hong-kong-between-2000-and-2015
#11
Ka-Shing Cheung, Wai-Kay Seto, James Fung, Ching-Lung Lai, Man-Fung Yuen
OBJECTIVES: Studies on the epidemiology of primary biliary cholangitis (PBC) in the Chinese population are lacking. We aimed to determine the epidemiology of PBC in Hong Kong (HK) with a population of 7.3 million. METHODS: We retrieved data from the electronic database of the HK Hospital Authority, the only public healthcare provider in Hong Kong. PBC cases between 2000 and 2015 were identified by International Classification of Diseases (ICD)-9 code. We estimated the age-/sex-adjusted incidence rate and prevalence of PBC, and analyzed the adverse outcomes (hepatocellular carcinoma (HCC), liver transplantation, and death)...
August 31, 2017: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/28858131/recurrent-bacteremia-and-liver-abscess-caused-by-clostridium-difficile-a-case-report
#12
Hiroshi Morioka, Mitsutaka Iguchi, Teiji Kuzuya, Hiroshige Mikamo, Tetsuya Yagi
RATIONALE: Clostridium difficile bacteremia (CDB) and liver abscess is a quite rare presentation of C. difficile infection. PATIENTS CONCERNS: A 74-year-old male with primary biliary cirrhosis and hepatocellular carcinoma who underwent transarterial chemoembolization (TACE) developed a high fever on post-TACE day 14. Intravenous ceftriaxone and following meropenem were administered, however, his clinical response was poor. On post-TACE day 24, 2 sets of blood culture were taken due to elevation of C-reactive protein levels...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28843649/change-in-nomenclature-for-the-immunologic-synapse-from-troxis-necrosis-to-trogocytosis
#13
Samuel W French
The immunologic synapse mechanism of liver necrosis was termed Troxis Necrosis meaning "nibbling". (Wang MX et al. and French SW. Exp Mol Pathol 2001, 71: 137-146). This mechanism of liver injury was first named "Piecemeal Necrosis" by Hans Popper. It is involved in autoimmune hepatitis, HCV, HBV, primary biliary cirrhosis and steatohepatitis. This process involves the T cell receptor (TCR) which binds to the hepatocyte antigen presenting major histocompatibility complex (MHC) on the hepatocytic plasma membrane which quickly leads to the removal of the complex from the liver and uptake by the CD4 lymphocyte...
August 24, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/28836457/investigational-drugs-in-phase-ii-clinical-trials-for-primary-biliary-cholangitis
#14
REVIEW
Marina G Silveira, Keith D Lindor
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease that may lead to biliary fibrosis, and eventually cirrhosis. The primary treatment for PBC is ursodeoxycholic acid (UDCA), which has favorably altered its natural history. However, up to 40% of patients have an inadequate response to UDCA, and are therefore at high risk of liver-related complications. Obeticholic acid has recently been approved for use in patients with PBC with inadequate response or who are intolerant to UDCA, but improvement in long-term outcomes has not yet been demonstrated...
October 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28835055/-patients-of-escherichia-coli-bloodstream-infection-analysis-of-antibiotic-resistance-and-predictors-of-mortality
#15
S J Zhao, Y Q Fu, M X Zhu, H Zhou, M Xu, R L Yan, Y X Shui, J Y Zhou
Objective: To analyze the drug resistance of Escherichia coli (E.coli) from bloodstream infection (BSI) and the predictors of mortality in E. coli bloodstream infection (BSI). Methods: The clinical data of 139 E. coli BSI cases diagnosed from January 2012 to December 2015 in The Second Affiliated Hospital of Zhejiang University School of Medicine, Lanxi Branch (Lanxi People's Hospital), Zhejiang Province, were retrospectively analyzed. The antimicrobial susceptibility testing was performed using Vitek 2 system...
August 22, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28833412/a-comparison-of-group-prediction-approaches-in-longitudinal-discriminant-analysis
#16
David M Hughes, Riham El Saeiti, Marta García-Fiñana
Longitudinal discriminant analysis (LoDA) can be used to classify patients into prognostic groups based on their clinical history, which often involves longitudinal measurements of various clinically relevant markers. Patients' longitudinal data is first modelled using multivariate generalised linear mixed models, allowing markers of different types (e.g. continuous, binary, counts) to be modelled simultaneously. We describe three approaches to calculating a patient's posterior group membership probabilities which have been outlined in previous studies, based on the marginal distribution of the longitudinal markers, conditional distribution and distribution of the random effects...
August 21, 2017: Biometrical Journal. Biometrische Zeitschrift
https://www.readbyqxmd.com/read/28826527/chromosome-17q21-genes-ormdl3-and-gsdmb-in-asthma-and-immune-diseases
#17
Sudipta Das, Marina Miller, David H Broide
Chromosome 17q21 contains a cluster of genes including ORMDL3 and GSDMB, which have been highly linked to asthma in genome-wide association studies. ORMDL3 is localized to the endoplasmic reticulum and regulates downstream pathways including sphingolipids, metalloproteases, remodeling genes, and chemokines. ORMDL3 inhibits serine palmitoyl-CoA transferase, the rate-limiting enzyme for sphingolipid biosynthesis. In addition, ORMDL3 activates the ATF6α branch of the unfolded protein response which regulates SERCA2b and IL-6, pathways of potential importance to asthma...
2017: Advances in Immunology
https://www.readbyqxmd.com/read/28792089/plasma-exchange-for-the-management-of-refractory-pruritus-of-cholestasis-a-report-of-three-cases-and-review-of-literature
#18
Rati Chkheidze, Ranjit Joseph, James Burner, Karen Matevosyan
BACKGROUND: Intractable pruritus of cholestasis leads to significant morbidity. Therapeutic plasma exchange (TPE) has been shown to be an effective alternative in the setting of refractory pruritus associated with cholestatic liver disease based on several individual reports. Due to rarity of this approach to intractable pruritus, the literature is sparse and therefore TPE, as a treatment for refractory pruritus is currently not in the apheresis guidelines. We present three additional patients with severe intractable pruritus of cholestasis successfully treated with plasma exchange to add to the mounting literature showing this as an effective and safe adjunctive therapy...
August 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28770708/the-interaction-between-anti-ro-ssa-and-anti-la-ssb-autoantibodies-and-anti-infectious-antibodies-in-a-wide-spectrum-of-auto-immune-diseases-another-angle-of-the-autoimmune-mosaic
#19
Nancy Agmon-Levin, Amir Dagan, Yogev Peri, Juan-Manuel Anaya, Carlo Selmi, Angela Tincani, Nicola Bizzaro, Ljudmila Stojanovich, Jan Damoiseaux, Jan Willem Cohen Tervaert, Marta Mosca, Ricard Cervera, Yehuda Shoenfeld
OBJECTIVES: The presence of anti-Ro/SSA and anti-La/SSB antibodies has been linked with autoimmunity in general and with several autoimmune diseases (AID) in particular. In the current study we evaluated these antibodies in a wide spectrum of AID as well as the links between them and anti-infectious antibodies. METHODS: We examined 2082 sera from patients with 16 different AID compared to 524 sera from geographically-matched healthy controls, for the presence and titres of anti-Ro/SSA and anti-La/SSB...
July 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28768257/autoimmunity-in-the-elderly-insights-from-basic-science-and-clinics-a-mini-review
#20
Abdulla Watad, Nicola Luigi Bragazzi, Mohammad Adawi, Howard Amital, Elias Toubi, Bat-Sheva Porat, Yehuda Shoenfeld
Advancements in the field of biomedicine, including the control of infectious diseases through antibiotics and vaccination practices and the prevention of chronic disorders, have led to reduced mortality, increased life expectancy and, as such, growth of the older population. Ageing is accompanied by profound morphological and physiological alterations. In particular, the immune system undergoes a complex series of remodeling/restructuring events, involving almost all compartments - both the innate and the adaptive system...
July 29, 2017: Gerontology
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