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"Autoimmune hepatitis"

Andrea D Praticò, Stefania Salafia, Elena R Praticò, Chiara Franzonello, Patrizia Barone, Salvatore Leonardi
No abstract text is available yet for this article.
December 2016: Minerva Pediatrica
Lily Dara, Zhang-Xu Liu, Neil Kaplowitz
Hepatocyte death, which can be apoptosis or necrosis depending on the context, is a prominent feature of liver disease. The lectin concanavalin A (ConA) activates immune cells, resulting in inflammatory liver injury and hepatocyte necrosis. In this issue of the JCI, Günther et al. demonstrate that the pseudokinase mixed lineage kinase domain-like protein (MLKL) participates in hepatocyte death in ConA injury and that MLKL-mediated death is independent of the receptor-interacting protein kinase RIPK3. RIPK3 was absent in hepatocytes, and MLKL-deficient mice, but not RIPK3-deficient mice, were protected from ConA-induced liver injury...
October 17, 2016: Journal of Clinical Investigation
E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leukocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged 3-15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011 and followed up for 4...
October 19, 2016: Acta Paediatrica
Claudia Günther, Gui-Wei He, Andreas E Kremer, James M Murphy, Emma J Petrie, Kerstin Amann, Peter Vandenabeele, Andreas Linkermann, Christopher Poremba, Ulrike Schleicher, Christin Dewitz, Stefan Krautwald, Markus F Neurath, Christoph Becker, Stefan Wirtz
Although necrosis and necroinflammation are central features of many liver diseases, the role of programmed necrosis in the context of inflammation-dependent hepatocellular death remains to be fully determined. Here, we have demonstrated that the pseudokinase mixed lineage kinase domain-like protein (MLKL), which plays a key role in the execution of receptor-interacting protein (RIP) kinase-dependent necroptosis, is upregulated and activated in human autoimmune hepatitis and in a murine model of inflammation-dependent hepatitis...
October 18, 2016: Journal of Clinical Investigation
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
Yan-Ni Li, Huan Ma, Lu Zhou, Jie Zhang, Li-Ping Guo, Shu-Qian Li, Yi-Qi Qian, Bang-Mao Wang
BACKGROUND: The long-term outcomes of patients with autoimmune hepatitis (AIH) given the immunosuppressive treatment are considered to be preferable. However, little is known about the response of AIH patients with cirrhosis to immunosuppressive treatment. We assessed the effects of immunosuppressive therapy in Chinese AIH patients with cirrhosis from a tertiary hospital. METHODS: Patients with a clinical diagnosis of AIH January 2000 and December 2015 were retrospectively reviewed...
2016: Chinese Medical Journal
Shiv Kumar Sarin, Ashok Choudhury
Acute-on-chronic liver failure (ACLF) is a distinct entity that differs from acute liver failure and decompensated cirrhosis in timing, presence of treatable acute precipitant, and course of disease, with a potential for self-recovery. The core concept is acute deterioration of existing liver function in a patient of chronic liver disease with or without cirrhosis in response to an acute insult. The insult should be a hepatic one and presentation in the form of liver failure (jaundice, encephalopathy, coagulopathy, ascites) with or without extrahepatic organ failure in a defined time frame...
December 2016: Current Gastroenterology Reports
Sern Wei Yeoh
Direct acting antiviral (DAA) regimens containing ritonavir have been developed to treat hepatitis C, with fewer side effects than that by interferon-based regimens. However prescribers must be aware of drug-drug interactions. There are multiple reports of iatrogenic Cushing syndrome (CS) caused by ritonavir, when used to treat human immunodeficiency virus, increasing the bioavailability of exogenous steroids by inhibiting cytochrome p450 enzymes in the liver and gut wall and thus reducing steroid metabolism...
September 2016: Journal of Clinical and Experimental Hepatology
Anil C Anand, Radha K Dhiman
Acute on chronic liver failure (ACLF) is a recently recognized syndrome and its definition has been evolving over last two decades. Currently, there is no universal consensus about the definition as kind of cases being seen in the Western world appear to be somewhat different from those that are seen in Asia Pacific region. But every one agrees that definition of ACLF should include following components. (a) The status of pre-existing liver disease, (b) defining the acute insult that leads to rapid deterioration of liver status, (c) time frame during which the acute insult can lead to rapid deterioration, (d) the quantification and definition of liver failure status after deterioration, which will determine the severity of ACLF, and (e) prediction of prognosis after analyzing first four components in the short and long terms...
September 2016: Journal of Clinical and Experimental Hepatology
Zeinab A Elkabbany, Nancy S Elbarbary, Eman A Ismail, Nesrine A Mohamed, Dina Ragab, Shereen Abdel Alem, Yasmine M Ezzat, Sarah S Maurice, Noha U Hashem
AIM: To identify the prevalence and effect of hepatopathies of different etiologies among pediatric patients with type 1 diabetes mellitus (T1DM) using transient elastography (TE) and its relation to glycemic control. METHODS: One hundred T1DM patients were studied focusing on liver functions, fasting lipid profile, hemoglobin A1c (HbA1c), hepatitis C virus (HCV), serum immunoglobulins, autoimmune antibodies; anti-nuclear antibody (ANA), anti-smooth muscle antibody (ASMA), and anti-liver kidney microsomal antibody (anti-LKM)...
September 26, 2016: Journal of Diabetes and its Complications
S Pereira, C M Cruz, M Soares, J Gandara, S Ferreira, V Lopes, R Vizcaíno, J Daniel, H P Miranda
INTRODUCTION: In liver transplantation, late graft dysfunction can have several causes, particularly rejection, infection, vascular, biliary complications, and others, usually suspected by abnormal liver tests. However, normal liver tests do not confirm a normal graft and liver biopsy could identify unexpected features with repercussions in immunosuppressive therapy. The aim of this study was to determinate the histological abnormalities in patients 10 years after liver allograft transplantation with sustainably normal liver tests and evaluate the changes in immunosuppressive therapy triggered by histological data...
September 2016: Transplantation Proceedings
Rodrigo Liberal, Charlotte R Grant
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues...
October 8, 2016: World Journal of Hepatology
Vaibhavi Velangi, A Bellad
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Petr Husa
Extrahepatic manifestations of hepatitis C virus infection (HCV) are very common. The most common of these is mixed cryoglobulinaemia. Anti-HCV antibodies and viral ribonucleic acid, HCV RNA, can be found in the cryoprecipitates, together with the rheumatoid factor. Cryoglobulins consist of a complex of immunoglobulins that in vitro precipitate upon the cooling bellow the human body temperature. Vasculitis is caused by the deposition of such immune complexes in the small blood vessels. A link with the HCV infection is considered to be established with membranoproliferative glomerulonephritis, leukocytoclastic vasculitis, lymphoproliferative disorders (in particular B cell lymphoma), Sjögren and sicca syndrome, lichen planus, porfyria cutanea tarda and diabetes mellitus...
2016: Vnitr̆ní Lékar̆ství
Atsushi Takahashi, Teruko Arinaga-Hino, Hiromasa Ohira, Takuji Torimura, Mikio Zeniya, Masanori Abe, Kaname Yoshizawa, Akinobu Takaki, Yoshiyuki Suzuki, Jong-Hon Kang, Nobuhiro Nakamoto, Tomoo Fujisawa, Koji Yonemoto, Atsushi Tanaka, Hajime Takikawa
BACKGROUND: A nationwide survey of autoimmune hepatitis (AIH) patients was performed in Japan in 2015. The aims of this study were to elucidate the trends and characteristics of AIH in Japan, in addition to identifying differences in AIH between acute hepatitis and chronic hepatitis. METHODS: Questionnaires about patients with AIH diagnosed from 2009 to 2013 were sent to 437 hospitals or clinics with hepatology specialists. RESULTS: A total of 1682 patients were enrolled...
October 8, 2016: Journal of Gastroenterology
Luis Ibáñez-Samaniego, Magdalena Salcedo, Javier Vaquero, Rafael Bañares
De novo autoimmune hepatitis (DAIH) is a rare clinical condition with features that resemble those of autoimmune hepatitis in patients undergoing liver transplantation (LT) for non-autoimmune liver disease. The diagnosis of this entity has been based on the presence of biochemical and histological patterns similar to those observed in the primary autoimmune hepatitis, although several considerations must be taken into account. The impact of DAIH on graft survival is relevant, and early diagnosis and treatment is associated with a good long-term outcome...
October 6, 2016: Liver Transplantation
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). 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. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
David N Assis, Hiroki Takahashi, Lin Leng, Mikio Zeniya, James L Boyer, Richard Bucala
BACKGROUND: The pathogenesis of autoimmune hepatitis (AIH) is incompletely understood. Macrophage migration inhibitory factor (MIF) is an inflammatory cytokine implicated in the pathophysiology of multiple autoimmune diseases. We recently reported that MIF expression was increased in a US AIH cohort. MIF expression in non-Western AIH patients is unknown. A MIF-173 GC single nucleotide polymorphism in the MIF promoter (rs755622) is clinically associated with steroid resistance in several inflammatory disorders but has not been evaluated in AIH...
October 1, 2016: Digestive Diseases and Sciences
Gamal Hasan, Asaad Assiri, Naglaa Marzuuk, Enas Daef, Sayed Abdelwahab, Ahmed Ahmed, Ismail Mohamad, Ayman Al-Eyadhy, Ali Alhaboob, Mohamad-Hani Temsah
OBJECTIVE: To describe the characteristics of hepatitis E virus (HEV) infection in a cohort of children from Upper Egypt using data from a large multicentre prospective study of acute viral hepatitis (AVH). METHODS: Data from subjects aged 2-18 years with AVH or close contacts of those with AVH found to have asymptomatic AVH were included in the analysis. Information concerning medical history, clinical examination, liver function tests and screening for hepatotropic viruses was recorded and analysed...
September 29, 2016: Journal of International Medical Research
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