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Autoimmune liver disease

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https://www.readbyqxmd.com/read/29230534/the-burden-of-comorbidity-and-complexity-in-sarcoidosis-impact-of-associated-chronic-diseases
#1
Pilar Brito-Zerón, Nihan Acar-Denizli, Antoni Sisó-Almirall, Xavier Bosch, Fernanda Hernández, Sergi Vilanova, Mireia Villalta, Belchin Kostov, Marina Paradela, Marcelo Sanchez, José Ramírez, Africa Muxí, Antonio Berruezo, Celeste Galceran-Chaves, Antoni Xaubet, Carles Agustí, Jacobo Sellarés, Manuel Ramos-Casals
PURPOSE: To evaluate comorbidity, complexity and poor outcomes in patients with sarcoidosis and to compare those scores with a control group. METHODS: 218 consecutive patients were diagnosed with sarcoidosis according to the ATS/ERS/WASOG criteria; extrathoracic involvement was evaluated using the 2014 WASOG organ assessment instrument. Sarcoidosis patients were compared with an age- and gender-matched control group of primary care outpatients without sarcoidosis...
December 11, 2017: Lung
https://www.readbyqxmd.com/read/29230304/coil-embolization-for-intrahepatic-haemorrhage-following-liver-biopsy-in-a-patient-with-hepatitis-c-virus-infection-and-hepatic-microaneurysms
#2
Apostolos Koffas, John Karani, Roger Williams
Intrahepatic bleeding secondary to rupture of hepatic microaneurysms is an uncommon clinical entity more frequently associated with polyarteritis nodosa (PAN) or rarely with other vasculitis or autoimmune disease. Hepatic vasculitis is reported in chronic hepatitis C virus (HCV) infection and an association between hepatitis C and PAN is described. The current report presents the case of a middle-aged female patient with a medical history remarkable for HCV infection who underwent a percutaneous liver biopsy, which was complicated by severe intrahepatic and perihepatic haemorrhage...
December 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29226354/liquid-chromatographic-methods-for-the-therapeutic-drug-monitoring-of-methotrexate-as-clinical-decision-support-for-personalized-medicine-a-brief-review
#3
REVIEW
Mónica Francisco Silva, Cláudia Ribeiro, Virgínia M F Gonçalves, Maria Elizabeth Tiritan, Áurea Lima
Methotrexate (MTX) is an antifolate drug used for several diseases. Depending on the disease, MTX can be administered at low-dose (LDMTX) in some autoimmune diseases, like rheumatoid arthritis, or at high-dose (HDMTX) in some cancers, such as acute lymphoblastic leukaemia. After absorption, MTX is metabolized in the liver to 7-hydroxymethotrexate (7-OH-MTX) and in the intestine to 2,4-diamino-N10-methylpteroic acid (DAMPA). Moreover, inside red blood cells (RBCs), MTX is converted to active metabolites, MTX polyglutamates (MTXPGs), contributing to its pharmacodynamics...
December 11, 2017: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/29225604/transcriptional-regulation-of-t-cell-lipid-metabolism-implications-for-plasma-membrane-lipid-rafts-and-t-cell-function
#4
REVIEW
George A Robinson, Kirsty E Waddington, Ines Pineda-Torra, Elizabeth C Jury
It is well established that cholesterol and glycosphingolipids are enriched in the plasma membrane (PM) and form signaling platforms called lipid rafts, essential for T-cell activation and function. Moreover, changes in PM lipid composition affect the biophysical properties of lipid rafts and have a role in defining functional T-cell phenotypes. Here, we review the role of transcriptional regulators of lipid metabolism including liver X receptors α/β, peroxisome proliferator-activated receptor γ, estrogen receptors α/β (ERα/β), and sterol regulatory element-binding proteins in T-cells...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29224419/expanding-the-phenotype-of-alk-positive-histiocytosis-a-report-of-2-cases
#5
Huiya Huang, Gabriela Gheorghe, Paula E North, Mariko Suchi
ALK-positive histiocytosis is a recently described rare histiocytic proliferative disorder of early infancy. When infants present with anemia, thrombocytopenia, and hepatosplenomegaly, this entity should be included in the differential diagnosis along with hemophagocytic lymphohistiocytosis, metabolic/storage diseases, hematopoietic malignancies, and autoimmune thrombocytopenia. We report 2 new cases of ALK-positive histiocytosis, one with kidney involvement and the other with extensive bone marrow involvement in addition to an overt liver disease...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29217467/why-should-we-measure-free-25-oh-vitamin-d
#6
REVIEW
Oleg Tsuprykov, Xin Chen, Carl-Friedrich Hocher, LianghongYin, Berthold Hocher
Vitamin D, either in its D2 or D3 form, is essential for normal human development during intrauterine life, kidney function and bone health. Vitamin D deficiency has also been linked to cancer development and some autoimmune diseases. Given this huge impact of vitamin D on human health, it is important for daily clinical practice and clinical research to have reliable tools to judge on the vitamin D status. The major circulating form of vitamin D3 is 25-hydroxyvitamin D3 (25(OH)D), although is not the most active metabolite, the concentrations of total 25-hydroxy vitamin D3 in the serum are currently routinely used in clinical practice to assess vitamin D status...
December 4, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/29215462/liver-transplantation-in-cryptogenic-cirrhosis-outcome-comparisons-between-nash-alcoholic-and-aih-cirrhosis
#7
Paul Joseph Thuluvath, Steven Hanish, Yulia Savva
BACKGROUND: The outcomes of liver transplantation (LT) in patients with cryptogenic cirrhosis (CC) have not been adequately examined except for small case series. We believe that patients currently listed as CC have truly cryptogenic liver disease and may have different post-LT outcomes compared to nonalcoholic steatohepatitis (NASH). METHODS: We compared the post-LT outcomes of adults with CC (n=3241) and compared them with cirrhosis from NASH (n=4089), alcohol (AC, n=7837) and autoimmune hepatitis (AIH, n=1435) using the UNOS database from 2002-16...
December 5, 2017: Transplantation
https://www.readbyqxmd.com/read/29212909/pd-l1-prevents-the-development-of-autoimmune-heart-disease-in-graft-versus-host-disease
#8
Kathryn W Juchem, Faruk Sacirbegovic, Cuiling Zhang, Arlene H Sharpe, Kerry Russell, Jennifer M McNiff, Anthony J Demetris, Mark J Shlomchik, Warren D Shlomchik
Effector memory T cells (TEM) are less capable of inducing graft-versus-host disease (GVHD) compared with naive T cells (TN). Previously, in the TS1 TCR transgenic model of GVHD, wherein TS1 CD4 cells specific for a model minor histocompatibility Ag (miHA) induce GVHD in miHA-positive recipients, we found that cell-intrinsic properties of TS1 TEM reduced their GVHD potency relative to TS1 TN Posttransplant, TS1 TEM progeny expressed higher levels of PD-1 than did TS1 TN progeny, leading us to test the hypothesis that TEM induce less GVHD because of increased sensitivity to PD-ligands...
December 6, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29207801/autoimmune-haemolytic-anaemia-a-spectrum-of-presentation-in-children
#9
Amitabh Singh, Anirban Mandal, Ankita Patel, Sataroopa Mishra
Autoimmune Haemolytic Anaemias (AIHAs) are rare in children. They can be either a primary disease or secondary to/triggered by a host of other clinical conditions. We present five interesting cases of paediatric AIHA associated with infections (viral, bacterial and atypical) and autoimmune diseases {Systemic Lupus Erythematosus (SLE) and Autoimmune Hepatitis (AIH)}. The H1N1 influenza associated AIHA responded to oseltamivir and Intravenous Immunoglobulin (IvIg) while the cases secondary to Mycoplasma pneumoniae and pneumococcal bacteraemia required only treatment of the primary infection...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29207430/-novel-developments-in-autoimmune-liver-diseases
#10
Christoph Schramm, Christian P Strassburg
No abstract text is available yet for this article.
December 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29201778/liver-disease-in-sri-lanka
#11
REVIEW
Hasitha S Wijewantha
Liver disease in Sri Lanka is mainly due to alcoholic liver disease and nonalcoholic fatty liver disease. In contrast to other South Asian countries, the prevalence of hepatitis B and C is low in Sri Lanka and prevalence of hepatitis A is intermediate. The few reported cases of hepatitis E in Sri Lanka are mainly in people who have traveled to neighboring South Asian countries. Wilson's disease, autoimmune hepatitis, hemochromatosis, drug-induced liver disease, and primary biliary cirrhosis are recognized causes of liver disease in Sri Lanka...
January 2017: Euroasian Journal of Hepato-Gastroenterology
https://www.readbyqxmd.com/read/29180000/usefulness-of-biochemical-remission-and-transient-elastography-in-monitoring-disease-course-in-autoimmune-hepatitis
#12
Johannes Hartl, Hanno Ehlken, Marcial Sebode, Moritz Peiseler, Till Krechl, Roman Zenouzi, Johann von Felden, Christina Weiler-Normann, Christoph Schramm, Ansgar W Lohse
BACKGROUND: Liver fibrosis regression but also progression may occur in patients with autoimmune hepatitis (AIH) under treatment. There is a need for non-invasive surrogate markers for fibrosis development in AIH to better guide immunosuppressive treatment. AIMS: The aims of the study were to assess the impact of complete biochemical remission defined as normalisation of transaminases and IgG on histological activity and fibrosis development, and the value of repeat transient elastography (TE) measurement for monitoring disease progression in AIH...
November 24, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29174094/missense-mutation-of-ttc7a-mimicking-tricho-hepato-enteric-sd-the-syndrome-in-a-patient-with-very-early-onset-inflammatory-bowel-disease
#13
João Farela Neves, Isabel Afonso, Luis Borrego, Catarina Martins, Ana Isabel Cordeiro, Conceição Neves, Caroline Lacoste, Catherine Badens, Alexandre Fabre
Tricho-hepato-enteric syndrome (SD/THE) and Multiple intestinal atresia with combined immune deficiency (MIA-CID) are autosomal recessive disorders that present immunological and gastrointestinal features. There are two different phenotypes of patients with TTC7A mutations: the severe form, caused by null mutations and leading to the classical MIA-CID; and the mild form, caused by missense mutations and leading to predominant features of VEO-IBD, less severe immunological involvement and hair abnormalities...
November 22, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29167408/conjugated-linoleic-acid-prevents-age-dependent-neurodegeneration-in-a-mouse-model-of-neuropsychiatric-lupus-via-the-activation-of-an-adaptive-response
#14
Antonio Monaco, Ida Ferrandino, Floriana Boscaino, Ennio Cocca, Luisa Cigliano, Francesco Maurano, Diomira Luongo, Maria Stefania Spagnuolo, Mauro Rossi, Paolo Bergamo
Oxidative stress is a key mediator of autoimmune/neurodegenerative disorders. The antioxidant/anti-inflammatory effect of a synthetic conjugated linoleic acid (CLA) mixture in MRL/MpJ-Faslpr mice (MRL/lpr), an animal model of neuropsychiatric lupus, was previously associated with the improvement of Nuclear factor-E2-related factor 2 (Nrf2) defences in the spleen and liver. However, little is known about the neuroprotective ability of CLA mixture. This study investigates the age-dependent progression of oxidative stress and the hyperactivation of redox-sensitive compensatory pathways (macroautophagy, Nrf2) in old/diseased MRL/lpr mice brains, and examines the effect produced by dietary CLA supplementation...
November 22, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/29164395/primary-biliary-cholangitis-a-comprehensive-overview
#15
REVIEW
Ana Lleo, Simona Marzorati, Juan-Manuel Anaya, M Eric Gershwin
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear...
November 21, 2017: Hepatology International
https://www.readbyqxmd.com/read/29162389/is-cryptogenic-cirrhosis-different-from-nash-cirrhosis
#16
Paul J Thuluvath, Sergey Kantsevoy, Avesh J Thuluvath, Yulia Savva
BACKGROUND & AIMS: We hypothesized that patients currently diagnosed as CC have truly 'cryptogenic' liver disease, and unlikely to have evolved from NASH. METHODS: To investigate this, we compared the clinical characteristics of adults with CC (n=7,999) with NASH (n=11,302), alcohol (AC, n=21,714) and autoimmune hepatitis (AIH, n=3,447) using the UNOS database from 2002-16. We performed an age, gender and year of listing matched comparison of CC and NASH (n= 7,201 in each group), and also stratified by the presence of obesity or diabetes mellitus (DM)...
November 18, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29159718/genetics-and-epigenetics-in-the-pathogenesis-of-primary-biliary-cholangitis
#17
REVIEW
Satoru Joshita, Takeji Umemura, Eiji Tanaka, Masao Ota
Primary biliary cholangitis (PBC) is a chronic, slowly progressive cholestatic autoimmune liver disease predominantly afflicting women. PBC is characterized by the presence of disease-specific antimitochondrial antibodies and the histological destruction of intrahepatic bile ducts, which eventually lead to cirrhosis and hepatic failure. Fortunately, ursodeoxycholic acid therapy has improved the outcome of the vast majority of PBC cases. Although the etiology of PBC has not yet been elucidated, human leukocyte antigen (HLA) class II alleles have been consistently associated with disease onset for decades...
November 20, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#18
Marlen Ivón Castellanos Fernández, Deyanira la Rosa Hernández, Diego Enrique Cabrera Eugenio, Wilson Palanca, Zaily Dorta Guridi, Licet González Fabián
Background: Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean countries. Objectives: The aim of our study was to investigate the clinical patterns, laboratory findings, and immunologic features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. Methods: A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba, from May 2012 to April 2016...
2017: Current Therapeutic Research, Clinical and Experimental
https://www.readbyqxmd.com/read/29156507/liver-transplantation-in-adults-with-liver-disease-due-to-common-variable-immunodeficiency-leads-to-early-recurrent-disease-and-poor-outcome
#19
Vian Azzu, Joshua E Elias, Adam Duckworth, Susan Davies, Rebecca Brais, Dinakantha S Kumararatne, Alexander E S Gimson, William J H Griffiths
Common variable immunodeficiency (CVID) is the commonest from of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features such as nodular regenerative hyperplasia, inflammation, fibrosis and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence...
November 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29154757/macrophage-polarization-and-meta-inflammation
#20
REVIEW
Chuan Li, Maria M Xu, Kepeng Wang, Adam J Adler, Anthony T Vella, Beiyan Zhou
Chronic overnutrition and obesity induces low-grade inflammation throughout the body. Termed "meta-inflammation," this chronic state of inflammation is mediated by macrophages located within the colon, liver, muscle, and adipose tissue. A sentinel orchestrator of immune activity and homeostasis, macrophages adopt variable states of activation as a function of time and environmental cues. Meta-inflammation phenotypically skews these polarization states and has been linked to numerous metabolic disorders. The past decade has revealed several key regulators of macrophage polarization, including the signal transducer and activator of transcription family, the peroxisome proliferator-activated receptor gamma, the CCAAT-enhancer-binding proteins (C/EBP) family, and the interferon regulatory factors...
November 3, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
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