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1)Nakamura M, Nishida N, Kawashima M, et al. Genome-wide association study identifies TNFSF15 and POU2AF1 as susceptibility loci for primary biliary cirrhosis in the Japanese population. Am J Hum Genet. 2012; 91: 721-8
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2)Aiba Y, Harada K, Komori A, et al. Systemic and local expression levels of TNF-like ligand 1A and its decoy receptor 3 are increased in primary biliary cirrhosis. Liver Int. 2014; 34: 679-88
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3)Hitomi Y, Kawashima M, Aiba Y, et al. Human primary biliary cirrhosis-susceptible allele of rs4979462 enhances TNFSF15 expression by binding NF-1. Hum Genet. 2015; 134: 737-47
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4)Padgett KA, Lan RY, Leung PC, et al. Primary biliary cirrhosis is associated with altered hepatic microRNA expression. J Autoimmun. 2009; 32: 246-53
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5)Tan Y, Pan T, Ye Y, et al. Serum microRNAs as potential biomarkers of primary biliary cirrhosis. PLoS One. 2014; 9: e111424
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6)Ninomiya M, Kondo Y, Funayama R, et al. Distinct microRNAs expression profile in primary biliary cirrhosis and evaluation of miR 505-3p and miR197-3p as novel biomarkers. PLoS One. 2013; 8: e66086
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7)勝美智大,二宮匡史,上野義之.血清内micro RNAプロファイリングを用いた原発性胆汁性肝硬変の病型分類応用と病態に関与するメカニズム.肝臓.2014; 55 suppl 1: A91
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8)Katsushima F, Takahashi A, Sakamoto N, et al. Expression of micro-RNAs in peripheral blood mononuclear cells from primary biliary cirrhosis patients. Hepatol Res. 2014; 44: E189-97
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9)Shimoda S. The Onset Mechanism of Primary Biliary Cirrhosis. In: Ohira H, editors. Autoimmune Liver Diseases. Tokyo: Springer; 2014. p.147-56
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10)Shimoda S, Harada K, Niiro H, et al. CX3CL1 (fractalkine): a signpost for biliary inflammation in primary biliary cirrhosis. Hepatology. 2010; 51: 567-75
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11)Shimoda S, Harada K, Niiro H, et al. Interaction between Toll-like receptors and natural killer cells in the destruction of bile ducts in primary biliary cirrhosis. Hepatology. 2011; 53: 1270-81
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12)Lan RY, Cheng C, Lian ZX, et al. Liver-targeted and peripheral blood alterations of regulatory T cells in primary biliary cirrhosis. Hepatology. 2006; 43: 729-37
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13)Hirschfield GM, Heathcote EJ, Gershwin ME. Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology. 2010; 139: 1481-96
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14)Concepcion AR, Lopez M, Ardura-Fabregat A, et al. Role of AE2 for pHi regulation in biliary epithelial cells. Front Physiol. 2014; 4: 413
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15)Sasaki M, Miyakoshi M, Sato Y, et al. Increased expression of mitochondrial proteins associated with autophagy in biliary epithelial lesions in primary biliary cirrhosis. Liver Int. 2013; 33: 312-20
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16)Sasaki M, Yoshimura-Miyakoshi M, Sato Y, et al. A possible involvement of endoplasmic reticulum stress in biliary epithelial autophagy and senescence in primary biliary cirrhosis. J Gastroenterol. 2015 Jan 1. [Epub ahead of print]
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17)Khan FM, Komarla AR, Mendoza PG, et al. Keratin 19 demonstration of canal of Hering loss in primary biliary cirrhosis: “minimal change PBC”? Hepatology. 2013; 57: 700-7
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18)Kakuda Y, Harada K, Nakanuma Y. Canals of Hering loss relates to the progression of the histological stages of primary biliary cirrhosis. J Clin Pathol. 2015; 68: 141-7
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19)Kuno A, Ikehara Y, Tanaka Y, et al. A serum ‘sweet-doughnut’ protein facilitates fibrosis evaluation and therapy assessment in patients with viral hepatitis. Sci Rep. 2013; 3: 1065
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20)Toshima T, Shirabe K, Ikegami T, et al. A novel serum marker, glycosylated Wisteria floribunda agglutinin-positive Mac-2 binding protein (WFA(+)-M2BP), for assessing liver fibrosis. J Gastroenterol. 2015; 50: 76-84
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21)Yamasaki K, Tateyama M, Abiru S, et al. Elevated serum levels of Wisteria floribunda agglutinin-positive human Mac-2 binding protein predict the development of hepatocellular carcinoma in hepatitis C patients. Hepatology. 2014; 60: 1563-70
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22)Toyoda H, Kumada T, Tada T, et al. Serum WFA+ -M2BP levels as a prognostic factor in patients with early hepatocellular carcinoma undergoing curative resection. Liver Int. 2015 Jul 2. [Epub ahead of print]
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23)Abe M, Miyake T, Kuno A, et al. Association between Wisteria floribunda agglutinin-positive Mac-2 binding protein and the fibrosis stage of non-alcoholic fatty liver disease. J Gastroenterol. 2015; 50: 776-84
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24)Umemura T, Joshita S, Sekiguchi T, et al. Serum Wisteria floribunda Agglutinin-Positive Mac-2-Binding Protein Level Predicts Liver Fibrosis and Prognosis in Primary Biliary Cirrhosis. Am J Gastroenterol. 2015; 110: 857-64
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25)Iwasaki S, Tsuda K, Ueta H, et al. Bezafibrate may have a beneficial effect in pre-cirrhotic primary biliary cirrhosis. Hepatol Res. 1999; 16: 12-8
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26)Kurihara T, Niimi A, Maeda A, et al. Bezafibrate in the treatment of primary biliary cirrhosis: comparison with ursodeoxycholic acid. Am J Gastroenterol. 2000; 95: 2990-2
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27)Itakura J, Izumi N, Nishimura Y, et al. Prospective randomized crossover trial of combination therapy with bezafibrate and UDCA for primary biliary cirrhosis. Hepatol Res. 2004; 29: 216-22
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28)Ohmoto K, Yoshioka N, Yamamoto S. Long-term effect of bezafibrate on parameters of hepatic fibrosis in primary biliary cirrhosis. J Gastroenterol. 2006; 41: 502-3
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29)Iwasaki S, Ohira H, Nishiguchi S, et al. The efficacy of ursodeoxycholic acid and bezafibrate combination therapy for primary biliary cirrhosis: A prospective, multicenter study. Hepatol Res. 2008; 38: 557-64
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30)Lens S, Leoz M, Nazal L, et al. Bezafibrate normalizes alkaline phosphatase in primary biliary cirrhosis patients with incomplete response to ursodeoxycholic acid. Liver Int. 2014; 34: 197-203
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31)Working Subgroup (English version) for Clinical Practice Guidelines for Primary Biliary Cirrhosis. Guidelines for the management of primary biliary cirrhosis: The Intractable Hepatobiliary Disease Study Group supported by the Ministry of Health, Labour and Welfare of Japan. Hepatol Res. 2014; 44 Suppl S1: 71-90
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32)Tanaka A, Hirohara J, Nakanuma Y, et al. Biochemical responses to bezafibrate improve long-term outcome in asymptomatic patients with primary biliary cirrhosis refractory to UDCA. J Gastroenterol. 2015; 50: 675-82
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33)Hosonuma K, Sato K, Yamazaki Y, et al. A prospective randomized controlled study of long-term combination therapy using ursodeoxycholic acid and bezafibrate in patients with primary biliary cirrhosis and dyslipidemia. Am J Gastroenterol. 2015; 110: 423-31
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34)Forman BM, Goode E, Chen J, et al. Identification of a nuclear receptor that is activated by farnesol metabolites. Cell. 1995; 81: 687-93
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35)Calkin AC, Tontonoz P. Transcriptional integration of metabolism by the nuclear sterol-activated receptors LXR and FXR. Nat Rev Mol Cell Biol. 2012; 13: 213-24
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36)Pellicciari R, Costantino G, Camaioni E, et al. Bile acid derivatives as ligands of the farnesoid X receptor. Synthesis, evaluation, and structure-activity relationship of a series of body and side chain modified analogues of chenodeoxycholic acid. J Med Chem. 2004; 47: 4559-69
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37)Ali AH, Carey EJ, Lindor KD. Recent advances in the development of farnesoid X receptor agonists. Ann Transl Med. 2015; 3: 5
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38)de Boer YS, van Gerven NM, Zwiers A, et al. Genome-wide association study identifies variants associated with autoimmune hepatitis type 1. Gastroenterology. 2014; 147: 443-52
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39)van Gerven NM, de Boer YS, Zwiers A, et al. HLA-DRB1*03:01 and HLA-DRB1*04:01 modify the presentation and outcome in autoimmune hepatitis type-1. Genes Immun. 2015; 16: 247-52
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40)Kirstein MM, Metzler F, Geiger E, et al. Prediction of short- and long-term outcome in patients with autoimmune hepatitis. Hepatology. 2015; 62: 1524-35
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41)Yuksel M, Wang Y, Tai N, et al. A novel “humanized mouse” model for autoimmune hepatitis and the association of gut microbiota with liver inflammation. Hepatology. 2015; 62: 1536-50
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42)Lin R, Zhou L, Zhang J, et al. Abnormal intestinal permeability and microbiota in patients with autoimmune hepatitis. Int J Clin Exp Pathol. 2015; 8: 5153-60
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43)Onji M, Zeniya M, Yamamoto K, et al. Autoimm-une hepatitis: Diagnosis and treatment guide in Japan, 2013. Hepatol Res. 2014; 44: 368-70
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44)Matsumoto K, Miyake Y, Matsushita H, et al. Anti-programmed cell death-1 antibody as a new serological marker for type 1 autoimmune hepatitis. J Gastroenterol Hepatol. 2014; 29: 110-5
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45)Kanno Y, Watanabe H, Takahashi A, et al. Anti-phosphoenolpyruvate carboxykinase 2 antibody in patients with autoimmune hepatitis. Hepatol Res. 2014; 44: 1019-25
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46)Villalta D, Mytilinaiou MG, Elsner M, et al. Autoantibodies to asialoglycoprotein receptor (ASGPR) in patients with autoimmune liver diseases. Clin Chim Acta. 2015; 450: 1-5
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47)Beyazit Y, Efe C, Tanoglu A, et al. Nitric oxide is a potential mediator of hepatic inflammation and fibrogenesis in autoimmune hepatitis. Scand J Gastroenterol. 2015; 50: 204-10
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48)Efe C, Kav T, Aydin C, et al. Low serum vitamin D levels are associated with severe histological features and poor response to therapy in patients with autoimmune hepatitis. Dig Dis Sci. 2014; 59: 3035-42
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49)持田 智.我が国における急性肝不全および遅発性肝不全(LOHF)の実態(2013年)―平成26年度全国調査― 厚生労働科学研究費補助金 難治性疾患等政策研究事業(難治性疾患政策研究事業)難治性の肝・胆道疾患に関する調査研究 平成26年度 総括・分担研究報告書.2015. p.92-109
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50)Mendizabal M, Marciano S, Videla MG, et al. Fulminant presentation of autoimmune hepatitis: clinical features and early predictors of corticosteroid treatment failure. Eur J Gastroenterol Hepatol. 2015; 27: 644-8
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51)Tanaka T, Sugawara Y, Kokudo N. Liver transplantation and autoimmune hepatitis. Intractable Rare Dis Res. 2015; 4: 33-8
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52)Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis--Update 2015. J Hepatol. 2015; 62(1 Suppl): S100-11
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53)Béland K, Marceau G, Labardy A, et al. Depletion of B cells induces remission of autoimmune hepatitis in mice through reduced antigen presentation and help to T cells. Hepatology. 2015; 62: 1511-23
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54)Takahashi A, Ohira H, Abe K, et al. Rapid corticosteroid tapering: Important risk factor for type 1 autoimmune hepatitis relapse in Japan. Hepatol Res. 2015; 45: 638-44
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55)Dhaliwal HK, Hoeroldt BS, Dube AK, et al. Long-Term Prognostic Significance of Persisting Histological Activity Despite Biochemical Remission in Autoimmune Hepatitis. Am J Gastroenterol. 2015; 110: 993-9
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