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Tatsuo Kanda, Shin Yasui, Masato Nakamura, Shingo Nakamoto, Koji Takahashi, Shuang Wu, Reina Sasaki, Yuki Haga, Sadahisa Ogasawara, Tomoko Saito, Kazufumi Kobayashi, Soichiro Kiyono, Yoshihiko Ooka, Eiichiro Suzuki, Tetsuhiro Chiba, Hitoshi Maruyama, Fumio Imazeki, Mitsuhiko Moriyama, Naoya Kato
Background: Interferon-free treatment can achieve higher sustained virological response (SVR) rates, even in patients in whom hepatitis C virus (HCV) could not be eradicated in the interferon treatment era. Immune restoration in the liver is occasionally associated with HCV infection. We examined the safety and effects of interferon-free regimens on HCV patients with autoimmune liver diseases. Results: All 7 HCV patients with autoimmune hepatitis (AIH) completed treatment and achieved SVR...
February 20, 2018: Oncotarget
Sareh Shahmohammadi, Mohammad Ali Sahraian, Abootorab Shahmohammadi, Rozita Doosti, Ali Zare-Mirzaie, Abdorreza Naser Moghadasi
Multiple sclerosis (MS) is one of the most important demyelinating diseases that affects the central nervous system. Its treatment often involves a long-term disease modifying therapy. According to some studies, the prevalence of autoimmune disorders, such as autoimmune hepatitis (AIH) and ulcerative colitis (UC) is higher in MS patients than in the normal population. There are also few studies that have reported the onset of UC after rituximab therapy. The present study presents a report of a 31-years old female patient suffering from aggressive multiple sclerosis, which developed into autoimmune hepatitis during the MS therapy...
March 1, 2018: Multiple Sclerosis and related Disorders
Ibis M Agosto, Nicole L Nichols, Gordon S Mitchell
Although systemic inflammation induced by even a low dose of lipopolysaccharide (LPS, 100uL/kg) impairs respiratory motor plasticity, little is known concerning cellular mechanisms giving rise to this inhibition. Phrenic motor facilitation (pMF) is a form of respiratory motor plasticity elicited by pharmacological agents applied to the cervical spinal cord, or by acute intermittent hypoxia (AIH; 3, 5 min hypoxic episodes); when elicited by AIH, pMF is known as phrenic long-term facilitation (pLTF). AIH consisting of moderate hypoxic episodes (mAIH, PaO2 = 35-55 mmHg) elicits pLTF via the Q- pathway to pMF, a mechanism that requires spinal serotonin (5HT2) receptor activation and new BDNF protein synthesis...
March 7, 2018: Journal of Neurophysiology
Hella Wobser, Tanja Paur, Elisabeth Schnoy, Janine Hartl, Gabriele I Kirchner
Background: Several years ago the International Autoimmune Hepatitis Group simplified the previous revised original scoring system for diagnosis of autoimmune hepatitis (AIH) into a scoring system based on only four instead of 13 parameters. Objective: We aimed to evaluate the suitability of the simplified AIH score for diagnosis of AIH in a German cohort with chronic liver diseases. Methods: In this retrospective single-center study, we compared the accuracy of both AIH scores in 70 patients with AIH and 211 patients with chronic liver diseases (PBC ( n  = 52), PSC ( n  = 27), NASH ( n  = 67), DILI ( n  = 15), CHB/C ( n  = 50))...
March 2018: United European Gastroenterology Journal
Wei Zhang, Debapriya De, Kahee A Mohammed, Satish Munigala, Guilan Chen, Jin-Ping Lai, Bruce R Bacon
Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune-mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histologic features of AIH and PBC along with modifications of biochemical and immunologic characteristics...
March 2018: Hepatology communications
Urs Christen, Edith Hintermann
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are serious autoimmune liver diseases that are characterized by a progressive destruction of the liver parenchyma and/or the hepatic bile ducts and the development of chronic fibrosis. Left untreated autoimmune liver diseases are often life-threatening, and patients require a liver transplantation to survive. Thus, an early and reliable diagnosis is paramount for the initiation of a proper therapy with immunosuppressive and/or anticholelithic drugs...
2018: Frontiers in Immunology
Melisa Dirchwolf, Sebastián Marciano, Diego H Giunta, Maria L Posadas-Martínez, Scott W Biggins, Andrés E Ruf
BACKGROUND: Hepatitis C virus (HCV) related cirrhosis is a leading indication for liver transplantation (LT) worldwide. Access to effective HCV treatment is inequitable globally. We aimed to analyze whether the introduction of effective HCV treatment caused an impact in LT trends in a middle-income country. METHODS: Cross sectional analysis of all adult patients who were listed/received a LT in Argentina for HCV, alcohol-related liver disease (ALD) or autoimmune hepatitis/primary biliary cirrhosis (AIH/PBC) from 2007 to 2017...
February 27, 2018: Clinical Transplantation
Satoru Joshita, Kaname Yoshizawa, Takeji Umemura, Hiromasa Ohira, Atsushi Takahashi, Kenichi Harada, Nguyen Canh Hiep, Koichi Tsuneyama, Masayoshi Kage, Masayuki Nakano, Jong-Hon Kang, Kazuhiko Koike, Mikio Zeniya, Tetsuya Yasunaka, Akinobu Takaki, Takuji Torimura, Masanori Abe, Osamu Yokosuka, Atsushi Tanaka, Hajime Takikawa
BACKGROUND: Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease...
February 23, 2018: Journal of Gastroenterology
Tobias Schmidt, Constantin Schmidt, Felix N Schmidt, Sebastian Butscheidt, Haider Mussawy, Jan Hubert, Thelonius Hawellek, Nicola Oehler, Florian Barvencik, Ansgar W Lohse, Thorsten Schinke, Christoph Schramm, Michael Amling, Tim Rolvien
Primary biliary cholangitis (PBC) is known to be a major risk factor for osteoporosis reflected by a reduction of bone mineral density (BMD). However, both the extent of the macro- and microstructural alterations of bone as well as the causative factors are unknown. We have retrospectively analyzed a total of 96 patients with PBC and 53 healthy controls matched for age, sex and body mass index. In addition to dual-energy X-ray absorptiometry (DXA) measurements at the lumbar spine and hip, high-resolution peripheral quantitative computed tomography (HR-pQCT) was used to assess the geometric, volumetric and microstructural changes of bone at the distal radius and tibia...
February 22, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
Avesh J Thuluvath, Po-Hung Chen, Paul J Thuluvath, Sergey Kantsevoy, Yulia Savva
BACKGROUND: Nonalcoholic steatohepatitis (NASH) cirrhosis is a common indication for liver transplantation (LT) in the U.S. There is a paucity of data on retransplantation (re-LT) in those who were initially transplanted for NASH. METHODS: We queried the UNOS data sets from 2002-2016 to analyze the outcomes of adults with NASH (n=128) and compared them to groups that received re-LT for cryptogenic cirrhosis (CC, n=189), alcoholic cirrhosis (AC, n=300) or autoimmune hepatitis cirrhosis (AIH-C, n=118) after excluding multiple-organ re-LT and individuals with HCC...
February 20, 2018: Transplantation
Isadora de Carvalho Trevizoli, Carolina Silva Pinedo, Vanessa Oliveira Teles, Renata Belém Pessoa de Melo Seixas, Elisa de Carvalho
OBJECTIVES: The aim of the to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health-related quality of life (HRQoL). METHODS: The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In addition, a questionnaire was applied which included signs, symptoms, and use of medications. RESULTS: A total of 43 patients (mean age, 15...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
Harveen Singh, Fariha Balouch, Charlton Noble, Peter Lewindon
OBJECTIVES: Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods. METHODS: Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2. RESULTS: Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2)...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
Xin Yan, Jinglan Jin
RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. PATIENT CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department...
February 2018: Medicine (Baltimore)
Hui-Min Wu, Li Sheng, Qi Wang, Han Bao, Qi Miao, Xiao Xiao, Can-Jie Guo, Hai Li, Xiong Ma, De-Kai Qiu, Jing Hua
AIM: To investigate the performance of transient elastography (TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome. METHODS: A total of 70 patients with biopsy-proven AIH-PBC overlap syndrome were included. Spearman correlation test was used to analyze the correlation of liver stiffness measurement (LSM) and fibrosis stage. Independent samples Student's t -test or one-way analysis of variance was used to compare quantitative variables...
February 14, 2018: World Journal of Gastroenterology: WJG
Victoria Gordon, Ratul Adhikary, Victoria Appleby, Debasis Das, James Day, Toby Delahooke, Selena Dixon, David Elphick, Claire Hardie, Barbara Hoeroldt, Patricia Hooper, John Hutchinson, Rebecca Jones, Faisal Khan, Guruprasad P Aithal, John McGonigle, Andrew Nelson, Alick Nkhoma, Stavroula Pelitari, Martin Prince, Annell Prosser, Vinay Sathanarayana, Sophia Savva, Naina Shah, Sushma Saksena, Sreedhari Thayalasekaran, Deven Vani, Andrew Yeoman, Dermot Gleeson
BACKGROUND AND AIMS: There is limited information regarding patients with AIH outside relatively few large centres. We describe here the presenting features of patients with AIH, collected as part of an audit involving 28 UK hospitals. METHODS: Patients (incident since 1/1/2007 or prevalent since 1/1/2000) were >18 years and either met 1999 International AIH Group (IAIHG) diagnostic criteria (n=1164), or received immunosuppressive therapy for clinically diagnosed AIH (n=103)...
February 17, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Keiichi Fujiwara, Yoshihiro Fukuda, Katsushi Seza, Masaya Saito, Shin Yasui, Masayuki Nakano, Osamu Yokosuka, Naoya Kato
BACKGROUND: There is yet no gold standard for the diagnosis of acute-onset autoimmune hepatitis (A-AIH), especially histologically acute AIH. As a result, long-term observation of A-AIH has been difficult and the nature is not well known. We retrospectively analyzed the clinicopathological features of A-AIH over a long prospective follow-up period. METHODS: Clinical, biochemical, immunological and pathological features of 30 patients (21 female, mean age 55.1 ± 13...
February 15, 2018: Hepatology International
Saumya Pathak, Deepak Kamat
Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease...
February 1, 2018: Pediatric Annals
Tingting Wang, Ruoting Men, Mingxing Hu, Xiaoli Fan, Xiaoxue Yang, Xiaojun Huang, Tinghong Ye, Li Yang
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease of an unknown etiology, glucocorticoid therapy is currently recognized as an effective treatment for AIH, but conventional application and patient compliance are both hindered by its side effects. The exploration of the AIH pathogenesis and the searching for the new candidate drugs that exert potential activity and low toxicity are urgently needed. Pomegranate peel extract (PoPx) is a natural extract of Punica granatum and has been reported to have anti-inflammatory and antioxidative properties...
February 8, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Cheng-Peng Zhong, Zhi-Feng Xi, Qiang Xia
BACKGROUND: Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation. METHODS: The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively...
February 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Amira Ibrahim Mansour, Ola Galal Behairy, Eman Rateb Abd Almonaem, Rasha Mahmoud Abd-Rabuh, Inas Abd Elmonem Ahmed
OBJECTIVE: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease mediated by an autoimmune reaction to hepatocytes, the present study aimed to assess the possible associations between interleukin-4 (IL-4) variable number of tandem repeats (VNTRs) and IL-4-590 promoter polymorphisms and susceptibility to autoimmune hepatitis type 1 in children. SUBJECTS AND METHODS: The study was performed on 101 children diagnosed with AIH and 104 apparently healthy, age and sex-matched control children, diagnosis of AIH was based on the simplified score for the diagnosis of AIH...
January 26, 2018: Cytokine
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