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autoimmune cholangiopathy

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https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#1
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/29158418/a-novel-pkhd1-mutation-interacts-with-the-nonobese-1diabetic-genetic-background-to-cause-autoimmune-cholangitis
#2
Wenting Huang, Daniel B Rainbow, Yuehong Wu, David Adams, Pranavkumar Shivakumar, Leah Kottyan, Rebekah Karns, Bruce Aronow, Jorge Bezerra, M Eric Gershwin, Laurence B Peterson, Linda S Wicker, William M Ridgway
We previously reported that NOD.c3c4 mice develop spontaneous autoimmune biliary disease (ABD) with anti-mitochondrial Abs, histopathological lesions, and autoimmune T lymphocytes similar to human primary biliary cholangitis. In this article, we demonstrate that ABD in NOD.c3c4 and related NOD ABD strains is caused by a chromosome 1 region that includes a novel mutation in polycystic kidney and hepatic disease 1 (Pkhd1). We show that a long terminal repeat element inserted into intron 35 exposes an alternative polyadenylation site, resulting in a truncated Pkhd1 transcript...
November 20, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
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
#3
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/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#4
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end-points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29026064/the-effect-of-recipient-body-mass-index-and-its-extremes-on-survival-and-graft-vascular-and-biliary-complications-after-liver-transplantation-a-single-center-retrospective-study
#5
Emmanouil Giorgakis, Michele Tedeschi, Eliano Bonaccorsi-Riani, Shirin Elizabeth Khorsandi, Krishna Menon, Hector Vilca-Melendez, Wayel Jassem, Parthi Srinivasan, Andreas Prachalias, Nigel Heaton
BACKGROUND This is the largest UK-based study on the effect of recipient body mass index (BMI) and its extremes (BMI <18.5 and BMI ≥35 kg/m²) on liver transplant (LT) outcomes. Its purpose was to analyze the BMI effect on post-LT mortality, graft loss, primary non-function (PNF), and graft vascular and biliary complications. MATERIAL AND METHODS Data were retrieved from a single-center LT database of 2,115 consecutive patients receiving first LT during period February 2004 to September 2015. Survivals were compared across the BMI groups; the effects of recipient BMI on survival, PNF, and graft vascular and biliary complications were analyzed via regression...
October 13, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/29022092/unique-manifestations-of-biliary-atresia-provide-new-immunological-insight-into-its-etiopathogenesis
#6
REVIEW
Toshihiro Muraji, Haruo Ohtani, Satoshi Ieiri
Biliary atresia (BA) is a unique cholestatic disease of newborns with a background of exaggerated immune response in the liver of unknown mechanism. Three hypotheses have been proposed; autoimmune type of cholangiopathy triggered by virus infection, graft-versus-host disease type of immune-mediated disease associated with maternal microchimerism and ductal plate malformation theory. Researchers on virus infection theory have experimentally explored immune process causing cholangiopathy on murine models of this disease, while in maternal microchimerism hypothesis were detected maternal cells in the BA patients' liver, of which roles are yet to be determined...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28962898/role-of-the-bicarbonate-responsive-soluble-adenylyl-cyclase-in-cholangiocyte-apoptosis-in-primary-biliary-cholangitis-a-new-hypothesis
#7
REVIEW
Jung-Chin Chang, Simei Go, Arthur J Verhoeven, Ulrich Beuers, Ronald P J Oude Elferink
Primary biliary cholangitis (PBC) is a chronic fibrosing cholangiopathy characterized by an autoimmune stereotype and defective biliary bicarbonate secretion due to down-regulation of anion exchanger 2 (AE2). Despite the autoimmune features, immunosuppressants are ineffective while two bile acid-based therapies (ursodeoxycholic acid and obeticholic acid) have been shown to improve biochemical and histological features of cholestasis and long-term prognosis. However, the etiology and pathogenesis of PBC is largely unknown...
September 26, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#8
Kyle Geary, Cemal Yazici, Anita Seibold, Grace Guzman
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy...
September 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28844953/how-the-biliary-tree-maintains-immune-tolerance
#9
REVIEW
Haiyan Zhang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
The liver is a vital organ with distinctive anatomy, histology and heterogeneous cell populations. These characteristics are of particular importance in maintaining immune homeostasis within the liver microenvironments, notably the biliary tree. Cholangiocytes are the first line of defense of the biliary tree against foreign substances, and are equipped to participate through various immunological pathways. Indeed, cholangiocytes protect against pathogens by TLRs-related signaling; maintain tolerance by expression of IRAK-M and PPARγ; limit immune response by inducing apoptosis of leukocytes; present antigen by expressing human leukocyte antigen molecules and costimulatory molecules; recruit leukocytes to the target site by expressing cytokines and chemokines...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28735981/autoimmune-liver-disease-in-children-with-sickle-cell-disease
#10
Suttiruk Jitraruch, Emer Fitzpatrick, Maesha Deheragoda, Annamaria Deganello, Giorgina Mieli-Vergani, Susan Height, David Rees, Nedim Hadzic, Marianne Samyn
OBJECTIVE: To assess the incidence, clinical features, and outcome of autoimmune liver disease (AILD) in patients with sickle cell disease (SCD). STUDY DESIGN: Single center retrospective review of patients with SCD with AILD referred between 1999 and 2015. RESULTS: Thirteen of 77 (17%) patients with SCD with hepatic dysfunction were diagnosed with AILD (median age 11, range, 3.4-16 years) with a female preponderance (77%). Acute hepatitis and insidious onset were the commonest presentations...
October 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28249274/the-emerging-role-of-soluble-adenylyl-cyclase-in-primary-biliary-cholangitis
#11
REVIEW
Jung-Chin Chang, Ulrich Beuers, Ronald P J Oude Elferink
BACKGROUND: Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic acid and reduced expression of anion exchanger 2 (AE2) on canalicular membranes and small bile ducts underline the importance of bicarbonate transportation in its disease mechanism...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/27876037/the-efficacy-of-adalimumab-in-psoriatic-arthritis-concomitant-to-overlapping-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis-a-case-report
#12
Teresa Del Ross, Amelia Ruffatti, Annarosa Floreani, Ariela Hoxha, Leonardo Punzi
BACKGROUND: The overlap syndrome of primary biliary cholangitis (formerly called primary biliary cirrhosis) and primary sclerosing cholangitis is an extremely rare condition that has never been described in association with other immune-mediated diseases, including psoriatic arthritis. While treatment with anti-Tumour Necrosis Factor-alpha (TNF-α) agents has proved to be effective in inflammatory arthropathies such as psoriatic arthritis, they have been employed in only a limited number of patients with autoimmune hepatitis, and their effectiveness is unclear...
November 22, 2016: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/27852547/prevalence-of-igg-4-associated-cholangiopathy-based-on-serum-igg-4-levels-in-patients-with-primary-sclerosing-cholangitis-and-its-relationship-with-inflammatory-bowel-disease
#13
Seyed Alireza Taghavi, Shaghayegh Kaedi Majd, Mahnoosh Sianati, Masood Sepehrimanesh
BACKGROUND/AIMS: Autoimmune cholangiopathy is part of a fibro-inflammatory immunoglobulin G-4 (IgG-4)-related systemic disease that causes biliary tract strictures. Its clinical presentation is quite similar to that of more common diseases such as primary sclerosing cholangitis (PSC) and pancreatobilliary malignancies. The aims of the present study were to evaluate the prevalence of IgG-4-associated cholangiopathy (IAC) in patients diagnosed with PSC and its relationship with inflammatory bowel disease (IBD)...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27712010/hepatic-manifestations-of-inflammatory-bowel-diseases
#14
REVIEW
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases are associated with various hepatobiliary disorders, reported both in Crohn's disease and ulcerative colitis. 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 used in different studies. Fatty liver is considered as the most common hepatobiliary complication in inflammatory bowel diseases while primary sclerosing cholangitis is the most specific one. Less frequently, inflammatory bowel diseases-associated hepatobiliary disorders include: autoimmune hepatitis/ primary sclerosing cholangitis overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis, hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis and liver abscess...
April 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27618480/stress-induced-cellular-responses-and-cell-death-mechanisms-during-inflammatory-cholangiopathies
#15
REVIEW
Motoko Sasaki, Yasuni Nakanuma
Various cellular responses including apoptosis, necrosis, autophagy and cellular senescence are involved in the pathogenesis of inflammatory cholangiopathies, such as primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and biliary atresia (BA). For example, dysregulated autophagy may play a role in abnormal expression of mitochondrial antigens and following autoimmune pathogenesis in bile duct lesions in PBC. Recently, new types of regulated cell death including necroptosis, parthanatos, pyroptosis, immunogenic cell death are the subject of numerous reports and they may play roles in pathogenesis of liver diseases, such as nonalcoholic steatohepatitis...
September 8, 2016: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/27606144/sequential-evaluation-of-pancreato-biliary-findings-in-a-case-with-igg4-associated-cholangiopathy-and-autoimmune-pancreatitis-during-corticosteroid-treatment
#16
İsmail Şerifoğlu, İbrahim İlker Öz, Yücel Üstündağ, Sevil Uygun İlikhan, Özlem Tokgöz
BACKGROUND: Immunoglobulin G4 (IgG4)-associated diseases mostly involve the pancreaticobiliary tree and pancreatic parenchyma. This disease complex is characterized by marked response to corticosteroid therapy and response to steroids is incorporated in the diagnostic algorithm of IgG4 associated diseases. However, there is much unknown about the sequences and duration of healing during the corticosteroid therapy in the literature. CASE REPORT: In this case report, we report a young male patient with IgG4 associated extrahepatic biliary stricture and autoimmune pancreatitis successfully treated with corticosteroids...
July 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27306577/the-utility-of-repeat-liver-biopsy-in-autoimmune-hepatitis-a%C3%A2-series-of-20-consecutive-cases
#17
Juan Putra, Arifa Toor, Arief A Suriawinata
Liver biopsy is recommended to establish the diagnosis and to assess remission in autoimmune hepatitis (AIH) patients. The aim of our study was to assess the utility of repeat biopsy in AIH. Forty liver biopsies from 20 consecutive AIH patients who underwent repeat biopsy were evaluated. We assessed the biopsies for histological findings other than AIH and how often the repeat biopsy led to a change in clinical management. Furthermore, we correlated the changes in the laboratory findings with the histological features...
August 2016: Pathology
https://www.readbyqxmd.com/read/27257963/recurrence-of-autoimmune-liver-disease-and-inflammatory-bowel-disease-after-pediatric-liver-transplantation
#18
REVIEW
Rodrigo Liberal, Diego Vergani, Giorgina Mieli-Vergani
Approximately 10% of children with autoimmune hepatitis (AIH) and 30% of those with sclerosing cholangitis (SC) require liver transplantation (LT). LT is indicated in patients who present with fulminant hepatic failure (ie, with encephalopathy) and in those who develop end-stage liver disease despite treatment. After LT, recurrent AIH is reported in approximately 30% of patients and recurrent SC in up to 50%. Diagnosis of recurrence is based on biochemical abnormalities, seropositivity for autoantibodies, interface hepatitis on histology, steroid dependence, and, for SC, presence of cholangiopathy...
September 2016: Liver Transplantation
https://www.readbyqxmd.com/read/26558153/autoimmune-pancreatitis-and-cholangitis
#19
REVIEW
Niraj Jani, James Buxbaum
Autoimmune pancreatitis (AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4 (IgG4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreatic manifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature...
November 6, 2015: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/26350286/diagnostic-performance-of-imaging-criteria-for-distinguishing-autoimmune-cholangiopathy-from-primary-sclerosing-cholangitis-and-bile-duct-malignancy
#20
Carly S Gardner, Mustafa R Bashir, Daniele Marin, Rendon C Nelson, Kingshuk Roy Choudhury, Lisa M Ho
OBJECTIVE: To determine the diagnostic performance of imaging criteria for distinguishing Ig-G4-associated autoimmune cholangiopathy (IAC) from primary sclerosing cholangitis (PSC) and bile duct malignancy. METHODS: A medical records search between January 2008 and October 2013 identified 10 patients (8 M, 2 F, mean age 61 years, range 34-82) with a clinical diagnosis of IAC. Fifteen cases of PSC (6 M, 9 F, mean age 50, range 22-65) and 15 cases of biliary malignancy (7 M, 8 F, mean age 65, range 48-84) were randomly selected for comparative analysis...
October 2015: Abdominal Imaging
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