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https://www.readbyqxmd.com/read/29785006/the-era-of-immunogenomics-immunopharmacogenomics
#1
REVIEW
Makda Zewde, Kazuma Kiyotani, Jae-Hyun Park, Hua Fang, Kai Lee Yap, Poh Yin Yew, Houda Alachkar, Taigo Kato, Tu H Mai, Yuji Ikeda, Tatsuo Matsuda, Xiao Liu, Lili Ren, Boya Deng, Makiko Harada, Yusuke Nakamura
Although germline alterations and somatic mutations in disease cells have been extensively analyzed, molecular changes in immune cells associated with disease conditions have not been characterized in depth. It is clear that our immune system has a critical role in various biological and pathological conditions, such as infectious diseases, autoimmune diseases, drug-induced skin and liver toxicity, food allergy, and rejection of transplanted organs. The recent development of cancer immunotherapies, particularly drugs modulating the immune checkpoint molecules, has clearly demonstrated the importance of host immune cells in cancer treatments...
May 21, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29783682/heat-shock-gene-inactivation-and-protein-aggregation-with-links-to-chronic-diseases
#2
Ian James Martins
The heat shock response involved in protein misfolding is linked to the formation of toxic immunogenic proteins with heat shock proteins (HSP) as regulators of amyloid beta aggregation. The defective amyloid beta trafficking between different intracellular compartments is now relevant to HSPs and autoimmunity. Overnutrition, temperature dysregulation, and stress repress the heat shock gene Sirtuin 1 with the induction of HSP regulated amyloid beta aggregation involved in the autoimmune response. Defective circadian rhythm alterations are connected to inactivation of the peripheral sink amyloid beta clearance pathway and related to insulin resistance, protein aggregation, and autoimmune disease in non-alcoholic fatty liver disease (NAFLD) and various neurodegenerative diseases such as Alzheimer's disease...
May 18, 2018: Diseases (Basel)
https://www.readbyqxmd.com/read/29780579/managing-portal-hypertension-in-patients-with-liver-cirrhosis
#3
REVIEW
Tilman Sauerbruch, Robert Schierwagen, Jonel Trebicka
Portal hypertension is one cause and a part of a dynamic process triggered by chronic liver disease, mostly induced by alcohol or incorrect nutrition and less often by viral infections and autoimmune or genetic disease. Adequate staging - continuously modified by current knowledge - should guide the prevention and treatment of portal hypertension with defined endpoints. The main goals are interruption of etiology and prevention of complications followed, if necessary, by treatment of these. For the past few decades, shunts, mostly as intrahepatic stent bypass between portal and hepatic vein branches, have played an important role in the prevention of recurrent bleeding and ascites formation, although their impact on survival remains ambiguous...
2018: F1000Research
https://www.readbyqxmd.com/read/29779314/-impact-of-psoas-muscle-index-on-early-postoperative-mortality-and-complications-after-liver-transplantation
#4
J C Hou, H Zheng, Z Qiang, Y M Zhang, W T Jiang, W Gao, J Z Cai, J J Zhang, Z Y Shen
Objective: To investigate the relationship between psoas muscle index (PMI) and early postoperative survival rate and the incidence of complications after liver transplantation in adults. Methods: The clinical data of 225 patients (male, n =184; female, n =41) underwent liver transplantation at the Organ Transplantation Department of First Central Clinic Institute of Tianjin Medical University from January 2014 to December 2016 were analyzed, retrospectively.Original disease: hepatitis B liver cirrhosis(44 cases), hepatitis C cirrhosis(10 cases), autoimmune liver cirrhosis(29 cases), other benign liver diseases(24 cases), liver cirrhosis with liver cancer(116 cases), hilar cholangiocarcinoma(1 case) and hepatic vascular sarcoma(1 case)...
May 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29777721/pentraxins-crp-i-and-crp-ii-are-post-translationally-deiminated-and-differ-in-tissue-specificity-in-cod-gadus-morhua-l-ontogeny
#5
Bergljót Magnadóttir, Polly Hayes, Berglind Gísladóttir, Birkir Þór Bragason, Mariya Hristova, Anthony P Nicholas, Sigríður Guðmundsdóttir, Sigrun Lange
Pentraxins are fluid phase pattern recognition molecules that form an important part of the innate immune defence and are conserved between fish and human. In Atlantic cod (Gadus morhua L.), two pentraxin-like proteins have been described, CRP-I and CRP-II. Here we show for the first time that these two CRP forms are post-translationally deiminated (an irreversible conversion of arginine to citrulline) and differ with respect to tissue specific localisation in cod ontogeny from 3 to 84 days post hatching. While both forms are expressed in liver, albeit at temporally differing levels, CRP-I shows a strong association with nervous tissue while CRP-II is strongly associated to mucosal tissues of gut and skin...
May 16, 2018: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29775966/an-enigmatic-case-presentation-of-budd-chiari-syndrome-with-pulmonary-embolism-an-unusual-syndrome-with-an-uncommon-complication
#6
A M Al-Sharydah, A H Al-Abdulwahhab, H A Abu AlOla
INTRODUCTION: In patients with a hypercoagulable state, such as patients with pulmonary embolism and/or Budd-Chiari syndrome, the complications from multiple gene mutations are more numerous than those from a single mutation. The authors present a woman with no major prior medical history who presented with pulmonary embolism and Budd-Chiari syndrome; this enigmatic co-occurrence has never been solely reported without underlying aetiology in a patient without prior medical conditions...
May 3, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29774578/the-immunobiology-of-rankl-and-myeloid-derived-suppressor-cell-activation-in-igg4-related-sclerosing-cholangitis
#7
Min Lian, Qixia Wang, Xiang Jiang, Jun Zhang, Yiran Wei, Yanmei Li, Bo Li, Weihua Chen, Haiyan Zhang, Qi Miao, Yanshen Peng, Xiao Xiao, Li Sheng, Weici Zhang, Jingyuan Fang, Ruqi Tang, M Eric Gershwin, Xiong Ma
The primary function of myeloid derived suppressor cells (MDSCs) is reflected in their immune modulatory role in several immune-mediated diseases. In IgG4-related disease (IgG4-RD), it has been hypothesized that there are selective regulatory defects that lead to a Th2 bias immune response. Herein we have taken advantage of a large cohort of patients with IgG4-related sclerosing cholangitis (IgG4-SC), the most common extra-pancreatic involvement of IgG4-RD, as well as controls consisting of primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and healthy volunteers, to study MDSC...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29766815/old-and-new-treatments-for-pediatric-autoimmune-hepatitis
#8
Silvia Nastasio, Giuseppe Maggiore, Marco Sciveres, Lorenza Matarazzo
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29766398/hepatosplenic-t-cell-lymphoma-a-unifying-entity-in-a-patient-with-hemolytic-anemia-massive-splenomegaly-and-liver-dysfunction
#9
Marianna Mavilia, Agnes McAuliffe, Safina Hafeez, Haleh Vaziri
Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL...
May 15, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29764441/case-report-identification-of-an-hnf1b-p-arg527gln-mutation-in-a-maltese-patient-with-atypical-early-onset-diabetes-and-diabetic-nephropathy
#10
Nikolai Paul Pace, Johann Craus, Alex Felice, Josanne Vassallo
BACKGROUND: The diagnosis of atypical non-autoimmune forms of diabetes mellitus, such as maturity onset diabetes of the young (MODY) presents several challenges, in view of the extensive clinical and genetic heterogeneity of the disease. In this report we describe a case of atypical non autoimmune diabetes associated with a damaging HNF1β mutation. This is distinguished by a number of uncharacteristic clinical features, including early-onset obesity, the absence of renal cysts and diabetic nephropathy...
May 15, 2018: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/29761535/diagnostic-performance-of-shear-wave-elastography-in-patients-with-autoimmune-liver-disease
#11
Changtian Li, Manish Dhyani, Atul K Bhan, Joseph R Grajo, Daniel S Pratt, Michael S Gee, Anthony E Samir
OBJECTIVES: To assess performance of shear wave elastography for evaluation of fibrosis and the histologic stage in patients with autoimmune liver disease (ALD) and to validate previously established advanced fibrosis cutoff values in this cohort. METHODS: Shear wave elastography was performed on patients with ALD with an Aixplorer ultrasound system (SuperSonic Imagine, Aix-en-Provence, France) using an SC6-1 transducer. The median estimated tissue Young modulus was calculated from sets of 8 to 10 elastograms...
May 15, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29756667/soluble-fgl2-restricts-autoimmune-hepatitis-progression-via-suppressing-tc17-and-conventional-cd8-t-cells-function
#12
Guo Ai, Weiming Yan, Haijing Yu, Fang Xiao, Dong Xi, Ke Ma, Jiaquan Huang, Xiaoping Luo, Xiaoyang Wan, Qin Ning
OBJECTIVE: Autoimmune hepatitis (AIH) is an inflammatory disease caused by aberrant immune response to hepatic self-antigens in which regulatory T cells (Tregs) are critical to maintain immunosupression. Soluble form of Fibrinogen-like protein 2 (sFGL2), a novel effector molecule of Treg is rarely investigated in AIH. Here, we dissected the role of sFGL2 in the autoimmune hepatitis and its potential mechanism underlying AIH progression. METHODS: Plasma and intrahepatic sFGL2 levels, as well as Treg cells were measured in both AIH patients and experimental autoimmune hepatitis (EAH) mice...
May 14, 2018: Journal of Gene Medicine
https://www.readbyqxmd.com/read/29755125/clinical-narrative-autoimmune-hepatitis
#13
REVIEW
Paul A Schmeltzer, Mark W Russo
Autoimmune hepatitis (AIH) is an inflammatory liver disease that is characterized by circulating autoantibodies, hypergammaglobulinemia, and a lymphoplasmocytic infiltration with interface hepatitis on liver biopsy. Treatment with corticosteroids and other immunosuppressive agents is effective and early diagnosis can result in near-normal life expectancy. Untreated patients, however, can progress to cirrhosis and liver failure. The clinical presentation is heterogeneous and may pose diagnostic and therapeutic dilemmas...
May 14, 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29753567/junctional-adhesion-molecules-jam-b-and-jam-c-promote-autoimmune-mediated-liver-fibrosis-in-mice
#14
Edith Hintermann, Monika Bayer, Clara Benedetta Conti, Sina Fuchs, Michel Fausther, Patrick S Leung, Michel Aurrand-Lions, Richard Taubert, Josef M Pfeilschifter, Mireen Friedrich-Rust, Detlef Schuppan, Jonathan A Dranoff, M Eric Gershwin, Michael P Manns, Beat A Imhof, Urs Christen
Fibrosis remains a serious health concern in patients with chronic liver disease. We recently reported that chemically induced chronic murine liver injury triggers increased expression of junctional adhesion molecules (JAMs) JAM-B and JAM-C by endothelial cells and de novo synthesis of JAM-C by hepatic stellate cells (HSCs). Here, we demonstrate that biopsies of patients suffering from primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) display elevated levels of JAM-C on portal fibroblasts (PFs), HSCs, endothelial cells and cholangiocytes, whereas smooth muscle cells expressed JAM-C constitutively...
May 9, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29751878/primary-biliary-cholangitis-and-autoimmune-hepatitis
#15
REVIEW
Raul S Gonzalez, Kay Washington
Primary biliary cholangitis and autoimmune hepatitis are common autoimmune diseases of the liver. Both have typical clinical presentations, including certain autoantibodies on serologic testing. Histologic features are also often typical: primary biliary cholangitis shows bile duct destruction (sometimes with granulomas), and autoimmune hepatitis shows prominent portal and lobular lymphoplasmacytic inflammation. Both have a wide differential diagnosis, including one another; they may also simultaneously occur within the same patient...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29751873/acute-viral-hepatitis-beyond-a-b-and-c
#16
REVIEW
Venancio Avancini Ferreira Alves
From the standpoint of the surgical pathologist "hepatitis" is defined as the set of histologic patterns of lesions found in livers infected by hepatotropic viruses, by non-hepatotrophic viruses leading to liver inflammation in the context of systemic infection, or due to an autoimmune disease, drug, or toxin involving the liver. This article is centered on the histologic patterns of injury in acute viral hepatitis, encompassing the hepatotropic viruses A, B, C, D, and E and the "icteric hemorrhagic fevers" (dengue, hantavirus, yellow fever)...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29748743/erythropoietin-and-nrf2-key-factors-in-the-neuroprotection-provided-by-apo-lactoferrin
#17
E T Zakharova, A V Sokolov, N N Pavlichenko, V A Kostevich, I N Abdurasulova, A V Chechushkov, I V Voynova, A Yu Elizarova, N N Kolmakov, M G Bass, I V Semak, A I Budevich, P M Kozhin, N K Zenkov, V M Klimenko, O V Kirik, D E Korzhevskii, E B Menshchikova, V B Vasilyev
Among the properties of lactoferrin (LF) are bactericidal, antianemic, immunomodulatory, antitumour, antiphlogistic effects. Previously we demonstrated its capacity to stabilize in vivo HIF-1-alpha and HIF-2-alpha, which are redox-sensitive multiaimed transcription factors. Various tissues of animals receiving recombinant human LF (rhLF) responded by expressing the HIF-1-alpha target genes, hence such proteins as erythropoietin (EPO), ceruloplasmin, etc. were synthesized in noticeable amounts. Among organs in which EPO synthesis occurred were brain, heart, spleen, liver, kidneys and lungs...
May 10, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/29746674/considerations-in-the-diagnosis-of-chronic-granulomatous-disease
#18
Joyce E Yu, Antoine E Azar, Hey J Chong, Artemio M Jongco, Benjamin T Prince
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency that is caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. The disease presents in most patients initially with infection, especially of the lymph nodes, lung, liver, bone, and skin. Patients with CGD are susceptible to a narrow spectrum of pathogens, and Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, Nocardia species, and Aspergillus species are the most common organisms implicated in North America...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29743796/increased-incidence-of-post-transplant-lymphoproliferative-disorder-in-autoimmune-liver-disease-an-irish-national-experience
#19
Ahmed Abu-Shanab, Yasser Ged, Naeem Ullah, Diarmaid Houlihan, Aiden McCormick
Background: Post-Transplant Lymphoproliferative Disorder (PTLD) is a well-recognized complication post solid organs transplant. PTLD represents a broad spectrum of abnormalities ranging from an infectious mononucleosis like illness to malignant lymphoma. Methods: A retrospective study was performed by collecting data of orthotopic liver transplant (OLT) patients in the National Liver Unit in Ireland from December 1993 to December 2014. Data was analyzed to identify PTLD patients and determine their demographic details, the indication for liver transplant, presenting symptoms, immunosuppression regimens, Epstein"Barr virus (EBV) status and PTLD outcome...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29742944/subcentimetric-incidental-intrahepatic-cholangiocarcinoma-in-an-explant-liver-diagnostic-difficulty-of-a-rare-entity
#20
Suvradeep Mitra, Gude Geethanjali, Sahaj Rathi, Arunanshu Behera, Ashim Das
Incidental intrahepatic cholangiocarcinoma (iCCA) is a rare neoplastic lesion in the explant liver specimens with an approximate incidence of 0.7%. The detection of iCCA is associated with poor prognosis in the posttransplant setting. The occurrence of a subcentimetric iCCA is very rare and poses a major diagnostic challenge to the pathologist. This article presents a rare case of subcentimetric iCCA in a young male in the background of advanced stage chronic liver disease resulting from autoimmune hepatitis possibly with chronic cholangiopathy along with the histomorphological differentials...
May 1, 2018: International Journal of Surgical Pathology
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