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autoimmune cholangiopathy

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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
#1
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
#2
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...
October 11, 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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
https://www.readbyqxmd.com/read/26090510/what-causes-biliary-atresia-unique-aspects-of-the-neonatal-immune-system-provide-clues-to-disease-pathogenesis
#17
Cara L Mack
Biliary atresia (BA) is the most frequent identifiable cause of neonatal cholestasis and the majority of patients will need liver transplantation for survival. Despite surgical intervention with the Kasai portoenterostomy, significant fibrosis and cirrhosis develops early in life. An increased understanding of what causes this inflammatory fibrosing cholangiopathy will lead to therapies aimed at protecting the intrahepatic biliary system from immune-mediated damage. This review focuses on studies pertaining to the role of the adaptive immune response in bile duct injury in BA, including cellular and humoral immunity...
May 1, 2015: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/25730176/immunoglobulin-g4-related-cholangiopathy-clinical-and-experimental-insights
#18
REVIEW
Lucas J Maillette de Buy Wenniger, Ulrich Beuers
PURPOSE OF REVIEW: The clinical spectrum of immunoglobulin G4-related disease (IgG4-RD) is diverse and the disease may affect multiple organ systems. The pancreatobiliary tract probably is the clinically most important localization of the disease but diagnosing IgG4-related cholangiopathy (synonym: IgG4-associated cholangitis) or autoimmune pancreatitis is often challenging. This review summarizes the current best practice in diagnosing and treating IgG4-related cholangiopathy and recent advances in our understanding of its cause...
May 2015: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/25435435/autophagy-and-senescence-in-fibrosing-cholangiopathies
#19
REVIEW
Yasuni Nakanuma, Motoko Sasaki, Kenichi Harada
Fibrosing cholangiopathy such as primary sclerosing cholangitis (PSC) and biliary atresia (BA) is characterized by biliary epithelial injuries and concentric fibrous obliteration of the biliary tree together with inflammatory cell infiltration. In these diseases, inappropriate innate immunity is reported to contribute more to bile duct pathology as compared with various aspects of "classical" autoimmune diseases. Primary biliary cirrhosis (PBC) is characterized by chronic cholangitis with bile duct loss and classical autoimmune features...
April 2015: Journal of Hepatology
https://www.readbyqxmd.com/read/25417613/hepatic-granulomas-in-turkey-a-6-year-clinicopathological-study-of-35-cases
#20
Memduh Sahin, Güldal Yılmaz, Mehmet Arhan, Ilker Sen
BACKGROUND/AIMS: Granulomas are focal aggregates of modified macrophages that are surrounded by a rim of lymphocytes and fibroblasts. The present study aimed to evaluate the prevalence and etiology of hepatic granulomas (HGs) in the Department of Gastroenterology with a wider population. MATERIALS AND METHODS: We performed a retrospective study on 2662 liver biopsy specimens analyzed between 2005 and 2011 at Gazi University Department of Gastroenterology to determine the presence of HGs...
October 2014: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
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