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Primary biliary cirrhosis in adults

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References

  • Addison T, Guys W. On a certain affection of the skin-vitiligoidea-a-plana f-tuberosa. Guys Hosp Rept 1851;7:265
  • Ahrens EH Jr, Kunkel H. The relationship between serum lipids and skin xanthomata in 18 patients with primary biliary cirrhosis. J Clin Invest 1949;28(6 Pt 2):1565-74
  • Myszor M, James OF. The epidemiology of primary biliary cirrhosis in north-east England: an increasingly common disease? Q J Med 1990;75(276):377-85
  • Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology 2000;119(6):1631-6
  • Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol 2012;56(5):1181-8
  • Selmi C, Mayo MJ, Bach N, et al. Primary biliary cirrhosis in monozygotic and dizygotic twins: genetics, epigenetics, and environment. Gastroenterology 2004;127(2):485-92
  • Zhang L, Weetman AP, Bassendine M, Oliveira DB. Major histocompatibility complex class-II alleles in primary biliary cirrhosis. Scand J Immunol 1994;39(1):104-6
  • Gershwin ME, Selmi C, Worman HJ, et al. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology 2005;42(5):1194-202
  • Ala A, Stanca CM, Bu-Ghanim M, et al. Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites. Hepatology 2006;43(3):525-31
  • Triger DR. Primary biliary cirrhosis: an epidemiological study. Br Med J 1980;281(6243):772-5
  • Prince MI, Chetwynd A, Diggle P, et al. The geographical distribution of primary biliary cirrhosis in a well-defined cohort. Hepatology 2001;34(6):1083-8
  • Leung PS, Park O, Tsuneyama K, et al. Induction of primary biliary cirrhosis in guinea pigs following chemical xenobiotic immunization. J Immunol 2007;179(4):2651-7
  • Lee J, Belanger A, Doucette JT, et al. Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007;5(11):1313-15
  • Poupon R, Poupon RE, Calmus Y, et al. Is ursodeoxycholic acid an effective treatment for primary biliary cirrhosis? Lancet 1987;1(8537):834-6
  • Corpechot C, Carrat F, Bahr A, et al. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005;128(2):297-303
  • Heathcote EJ, Cauch-Dudek K, Walker V, et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 1994;19(5):1149-56
  • Kuiper EM, Hansen BE, de Vries RA, et al. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009;136(4):1281-7
  • Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006;130(3):715-20
  • Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997;113(3):884-90
  • Poupon RE, Bonnand AM, Chrétien Y, Poupon R. Ten-year survival in ursodeoxycholic acid-treated patients with primary biliary cirrhosis. The UDCA-PBC Study Group. Hepatology 1999;29(6):1668-71
  • Gershwin ME, Mackay IR, Sturgess A, Coppel RL. Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis. J Immunol 1987;138(10):3525-31
  • Lacerda MA, Shi XH, Zhang FC, et al. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol 1995;90(2):247-9
  • Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology 2009;50(1):291-308
  • Kitami N, Ishii H, Shimizu H, et al. Immunoreactivity to M2 proteins in antimitochondrial antibody-negative patients with primary biliary cirrhosis. J Gastroenterol Hepatol 1994;9(1):7-12
  • Tsuneyama K, Van De Water J, Van Thiel D, et al. Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibody-negative primary biliary cirrhosis. Hepatology 1995;22(5):1440-6
  • Longo M, Crosignani A, Podda M. Hyperlipidemia in chronic cholestatic liver disease. Curr Treat Options Gastroenterol 2001;4(2):111-14
  • Zein CO, Angulo P, Lindor KD. When is liver biopsy needed in the diagnosis of primary biliary cirrhosis? Clin Gastroenterol Hepatol 2003;1(2):89-95
  • Prince M, Chetwynd A, Newman W, et al. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology 2002;123(4):1044-51
  • Levy C, Zein CO, Gomez J, et al. Prevalence and predictors of esophageal varices in patients with primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007;5(7):803-8
  • Patanwala I, McMeekin P, Walters R, et al. A validated clinical tool for the prediction of varices in PBC: the Newcastle Varices in PBC Score. J Hepatol 2013;59(2):327-35
  • Dickson ER, Grambsch PM, Fleming TR, et al. Prognosis in primary biliary cirrhosis: model for decision making. Hepatology 1989;10(1):1-7
  • Kim WR, Wiesner RH, Poterucha JJ, et al. Adaptation of the Mayo primary biliary cirrhosis natural history model for application in liver transplant candidates. Liver Transpl 2000;6(4):489-94
  • Nakamura M, Shimizu-Yoshida Y, Takii Y, et al. Antibody titer to gp210-C terminal peptide as a clinical parameter for monitoring primary biliary cirrhosis. J Hepatol 2005;42(3):386-92
  • Rigopoulou EI, Dalekos GN. Anti-sp100 antibodies in primary biliary cirrhosis. Scand J Gastroenterol 2004;39(4):406-7. author reply 407
  • Yang WH, Yu JH, Nakajima A, et al. Do antinuclear antibodies in primary biliary cirrhosis patients identify increased risk for liver failure? Clin Gastroenterol Hepatol 2004;2(12):1116-22
  • Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008;48(3):871-7
  • Corpechot C, Chazouilleres O, Poupon R. Early primary biliary cirrhosis: biochemical response to treatment and prediction of long-term outcome. J Hepatol 2011;55(6):1361-7
  • Momah N, Silveira MG, Jorgensen R, et al. Optimizing biochemical markers as endpoints for clinical trials in primary biliary cirrhosis. Liver Int 2012;32(5):790-5
  • Silveira MG, Brunt EM, Heathcote J, et al. American Association for the Study of Liver Diseases endpoints conference: design and endpoints for clinical trials in primary biliary cirrhosis. Hepatology 2010;52(1):349-59
  • National Environmental Policy Act of 1969, in U.S.C. 1994. p. 102-105
  • Jones DE, Bhala N, Burt J, et al. Four year follow up of fatigue in a geographically defined primary biliary cirrhosis patient cohort. Gut 2006;55(4):536-41
  • Swain MG. Fatigue in chronic disease. Clin Sci (Lond) 2000;99(1):1-8
  • Theal JJ, Toosi MN, Girlan L, et al. A randomized, controlled crossover trial of ondansetron in patients with primary biliary cirrhosis and fatigue. Hepatology 2005;41(6):1305-12
  • Talwalkar JA, Donlinger JJ, Gossard AA, et al. Fluoxetine for the treatment of fatigue in primary biliary cirrhosis: a randomized, double-blind controlled trial. Dig Dis Sci 2006;51(11):1985-91
  • Newton JL, Gibson GJ, Tomlinson M, et al. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology 2006;44(1):91-8
  • Ian Gan S, de Jongh M, Kaplan MM. Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience. Dig Dis Sci 2009;54(10):2242-6
  • Silveira M, Gossard AA, DeCook A, et al. Modafinil in the treatment of fatigue in patients with primary biliary cirrhosis Hepatology. 2011;54(Suppl 4):1211A-2A
  • Kremer AE, van Dijk R, Leckie P, et al. Serum autotaxin is increased in pruritus of cholestasis, but not of other origin, and responds to therapeutic interventions. Hepatology 2012;56(4):1391-400
  • Shoda M. Uber die ursodesoxycholsaure aus barengallen und ihre physiologische wirkung. J Biochem 1927;7:505-17
  • Iwasaki T. Uber die konstitution der urso-desoxycholsaure. Z Physiol Chem 1936;244:181-93
  • Cohen H, Alferiev IS, Mönkkönen J, et al. Synthesis and preclinical pharmacology of 2-(2-aminopyrimidinio) ethylidene-1,1-bisphosphonic acid betaine (ISA-13-1)-a novel bisphosphonate. Pharm Res 1999;16(9):1399-406
  • Hofmann AF. Pharmacology of ursodeoxycholic acid, an enterohepatic drug. Scand J Gastroenterol Suppl 1994;204:1-15
  • European Association for the Study of the Liver. EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51(2):237-67
  • Papastergiou V, Tsochatzis EA, Rodriquez-Peralvarez M, et al. Biochemical criteria at 1 year are not robust indicators of response to ursodeoxycholic acid in early primary biliary cirrhosis: results from a 29-year cohort study. Aliment Pharmacol Ther 2013;38(11-12):1354-64
  • Carbone M, Mells GF, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 2013;144(3):560-9. e7; quiz e13-4
  • Angulo P, Dickson ER, Therneau TM, et al. Comparison of three doses of ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a randomized trial. J Hepatol 1999;30(5):830-5
  • Gong Y, Gluud C. Colchicine for primary biliary cirrhosis. Cochrane Database Syst Rev 2004(2):CD004481
  • Kaplan MM, Cheng S, Price LL, Bonis PA. A randomized controlled trial of colchicine plus ursodiol versus methotrexate plus ursodiol in primary biliary cirrhosis: ten-year results. Hepatology 2004;39(4):915-23
  • Angulo P, Patel T, Jorgensen RA, et al. Silymarin in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology 2000;32(5):897-900
  • Charatcharoenwitthaya P, Talwalkar JA, Angulo P, et al. Moexipril for treatment of primary biliary cirrhosis in patients with an incomplete response to ursodeoxycholic acid. Dig Dis Sci 2010;55(2):476-83
  • Silveira MG, Lindor KD. Is there a role for tetrathiomolybdate in the treatment of primary biliary cirrhosis? Transl Res 2010;155(3):120-2
  • Gong Y, Christensen E, Gluud C. Azathioprine for primary biliary cirrhosis. Cochrane Database Syst Rev 2007(3):CD006000
  • Li WX, Yan X, Shi CR, Zhang AP. Chlorambucil for patients with primary biliary cirrhosis. Cochrane Database Syst Rev 2012;9:CD008714
  • Tsuda M, Moritoki Y, Lian ZX, et al. Biochemical and immunologic effects of rituximab in patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid. Hepatology 2012;55(2):512-21
  • Gong Y, Christensen E, Gluud C. Cyclosporin A for primary biliary cirrhosis. Cochrane Database Syst Rev 2007(3):CD005526
  • Leuschner M, Güldütuna S, You T, et al. Ursodeoxycholic acid and prednisolone versus ursodeoxycholic acid and placebo in the treatment of early stages of primary biliary cirrhosis. J Hepatol 1996;25(1):49-57
  • ClinicalTrials.gov identifier: NCT01829698. Available from: http://clinicaltrials.gov/ct2/show/NCT01829698
  • ClinicalTrials.gov identifier: NCT01904058. Available from: http://clinicaltrials.gov/ct2/show/NCT01904058
  • Devchand PR, Keller H, Peters JM, et al. The PPARalpha-leukotriene B4 pathway to inflammation control. Nature 1996;384(6604):39-43
  • Chianale J, Vollrath V, Wielandt AM, et al. Fibrates induce mdr2 gene expression and biliary phospholipid secretion in the mouse. Biochem J 1996;314(Pt 3):781-6
  • Levy C, Peter JA, Nelson DR, et al. Pilot study: fenofibrate for patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid. Aliment Pharmacol Ther 2011;33(2):235-42
  • Walker LJ, Newton J, Jones DE, Bassendine MF. Comment on biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2009;49(1):337-8. author reply 338
  • Han XF, Wang QX, Liu Y, et al. Efficacy of fenofibrate in Chinese patients with primary biliary cirrhosis partially responding to ursodeoxycholic acid therapy. J Dig Dis 2012;13(4):219-24
  • Rautiainen H, Kärkkäinen P, Karvonen AL, et al. Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial. Hepatology 2005;41(4):747-52
  • Angulo P, Jorgensen RA, Keach JC, et al. Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology 2000;31(2):318-23
  • Forman BM, Goode E, Chen J, et al. Identification of a nuclear receptor that is activated by farnesol metabolites. Cell 1995;81(5):687-93
  • Ananthanarayanan M, Balasubramanian N, Makishima M, et al. Human bile salt export pump promoter is transactivated by the farnesoid X receptor/bile acid receptor. J Biol Chem 2001;276(31):28857-65
  • Elfaki DA, Bjornsson E, Lindor KD. Review article: nuclear receptors and liver disease – current understanding and new therapeutic implications. Aliment Pharmacol Ther 2009;30(8):816-25
  • Fiorucci S, Clerici C, Antonelli E, et al. Protective effects of 6-ethyl chenodeoxycholic acid, a farnesoid X receptor ligand, in estrogen-induced cholestasis. J Pharmacol Exp Ther 2005;313(2):604-12
  • Liu Y, Binz J, Numerick MJ, et al. Hepatoprotection by the farnesoid X receptor agonist GW4064 in rat models of intra- and extrahepatic cholestasis. J Clin Invest 2003:112(11):1678-87
  • Pellicciari R, Fiorucci S, Camaioni E, et al. 6alpha-ethyl-chenodeoxycholic acid (6-ECDCA), a potent and selective FXR agonist endowed with anticholestatic activity. J Med Chem 2002;45(17):3569-72
  • Fiorucci S, Antonelli E, Rizzo G, et al. The nuclear receptor SHP mediates inhibition of hepatic stellate cells by FXR and protects against liver fibrosis. Gastroenterology 2004;127(5):1497-512
  • Mason A, Luketic V, Lindor K, et al. Farnesoid-x receptor agonists: a new class of drugs for the treatment of PBC? An International study evaluating the addition of INT-747 to ursodeoxycholic acid. J Hepatol 2010;52(Suppl 1):S1-2
  • Hirschfield G, Kowdley K, Mason A, et al. Long-term (LT) therapy of a farnesoid X receptor (FXR) agonist obeticholic acid (OCA) maintains biochemical response in primary biliray cirrhosis (PBC). J Hepatol 2012;56(Suppl 2):S372

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