EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

Journal of Hepatology

Volumn 51, Issue 2, 2009, Pages 237-267

  European Association for the Study of the Liver, ^a  

^a NONE

Author keywords

Cholestatic liver diseases in pregnancy; Drug induced cholestatic liver disease; Fatigue; Genetic cholestatic liver disease; Immunoglobulin G4 associated cholangitis; Intrahepatic cholestasis of pregnancy; Overlap syndrome; Primary biliary cirrhosis; Primary sclerosing cholangitis; Pruritus

Indexed keywords

ALLOPURINOL; ATORVASTATIN; AZATHIOPRINE; BARBITURIC ACID DERIVATIVE; BEZAFIBRATE; BUDESONIDE; CAPTOPRIL; CARBAMAZEPINE; CHLORAMBUCIL; CHLORPROPAMIDE; COLCHICINE; CORTICOSTEROID; CYCLOSPORIN A; ETANERCEPT; LAMIVUDINE PLUS ZIDOVUDINE; MALOTILATE; METHOTREXATE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PENICILLAMINE; PENTOXIFYLLINE; PIRFENIDONE; PREDNISOLONE; RIFAMPICIN; SILYMARIN; SULINDAC; TACROLIMUS; TAMOXIFEN; THALIDOMIDE; UNINDEXED DRUG; URSODEOXYCHOLIC ACID;

ALAGILLE SYNDROME; ARTICLE; AUTOIMMUNE HEPATITIS; BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS; CHOLANGITIS; CHOLESTASIS; CHOLESTATIC HEPATITIS; CLINICAL DECISION MAKING; CLINICAL FEATURE; CLINICAL PRACTICE; CLINICAL TRIAL; COLON CANCER; DIAGNOSTIC APPROACH ROUTE; DIFFERENTIAL DIAGNOSIS; DRUG DOSE INCREASE; DRUG MEGADOSE; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; ENTERITIS; FATIGUE; HUMAN; IMMUNOGLOBULIN G4 ASSOCIATED CHOLANGITIS; INTRAHEPATIC CHOLESTASIS; LABORATORY TEST; LIVER BIOPSY; LIVER DISEASE; LIVER FIBROSIS; LIVER INJURY; LIVER TRANSPLANTATION; LOW DRUG DOSE; MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY; OSTEOPOROSIS; PRACTICE GUIDELINE; PREGNANCY COMPLICATION; PRIMARY BILIARY CIRRHOSIS; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS; PRURITUS; SCLEROSING CHOLANGITIS;

ADULT; CHILD; CHOLANGITIS; CHOLANGITIS, SCLEROSING; CHOLESTASIS, INTRAHEPATIC; CYSTIC FIBROSIS; FEMALE; HEPATITIS, AUTOIMMUNE; HUMANS; IMMUNOGLOBULIN G; INFANT; LIVER CIRRHOSIS, BILIARY; MALE; OSTEOPOROSIS; PREGNANCY; PREGNANCY COMPLICATIONS; SYNDROME;

EID: 67649205149     PISSN: 01688278     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jhep.2009.04.009     Document Type: Article

References (247)

1. Guyatt G.H., Oxman A.D., Vist G.E., Kunz R., Falck-Ytter Y., Alonso-Coello P., et al. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 336 (2008) 924-926
2. Chazouillères O., and Housset C. Intrahepatic cholestasis. In: Rodés J. (Ed). Textbook of hepatology: from basic science to clinical practice. 3rd ed. (2007), Blackwell, Oxford 1481-1500
3. Heathcote E.J. Diagnosis and management of cholestatic liver disease. Clin Gastroenterol Hepatol 5 (2007) 776-782
4. Freeman M.L., Nelson D.B., Sherman S., Haber G.B., Herman M.E., Dorsher P.J., et al. Complications of endoscopic biliary sphincterotomy. N Engl J Med 335 (1996) 909-918
5. Ludwig J. Idiopathic adulthood ductopenia: an update. Mayo Clin Proc 73 (1998) 285-291
6. Heathcote E.J. Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines. Hepatology 31 (2000) 1005-1013
7. Metcalf J.V., Mitchison H.C., Palmer J.M., Jones D.E., Bassendine M.F., and James O.F. Natural history of early primary biliary cirrhosis. Lancet 348 (1996) 1399-1402
8. Invernizzi P., Lleo A., and Podda M. Interpreting serological tests in diagnosing autoimmune liver diseases. Semin Liver Dis 27 (2007) 161-172
9. Vergani D., Alvarez F., Bianchi F.B., Cancado E.L., Mackay I.R., Manns M.P., et al. Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group. J Hepatol 41 (2004) 677-683
10. Ludwig J., Dickson E.R., and McDonald G.S. Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis). Virchows Arch A Pathol Anat Histol 379 (1978) 103-112
11. Scheuer P.J. Primary biliary cirrhosis: diagnosis, pathology and pathogenesis. Postgrad Med J 59 (1983) 106-115
12. Beuers U. Drug insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis. Nat Clin Pract Gastroenterol Hepatol 3 (2006) 318-328
13. Poupon R.E., Balkau B., Eschwege E., and Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N Engl J Med 324 (1991) 1548-1554
14. Heathcote E.J., Cauch-Dudek K., Walker V., Bailey R.J., Blendis L.M., Ghent C.N., et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 19 (1994) 1149-1156
15. Lindor K.D., Dickson E.R., Baldus W.P., Jorgensen R.A., Ludwig J., Murtaugh P.A., et al. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. Gastroenterology 106 (1994) 1284-1290
16. Combes B., Carithers Jr. R.L., Maddrey W.C., Lin D., McDonald M.F., Wheeler D.E., et al. A randomized, double-blind, placebo-controlled trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 22 (1995) 759-766
17. Pares A., Caballeria L., Rodes J., Bruguera M., Rodrigo L., Garcia-Plaza A., et al. Long-term effects of ursodeoxycholic acid in primary biliary cirrhosis: results of a double-blind controlled multicentric trial. UDCA-Cooperative Group from the Spanish Association for the Study of the Liver. J Hepatol 32 (2000) 561-566
18. Corpechot C., Carrat F., Bonnand A.M., Poupon R.E., and Poupon R. The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatology 32 (2000) 1196-1199
19. Poupon R.E., Lindor K.D., Cauch-Dudek K., Dickson E.R., Poupon R., and Heathcote E.J. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 113 (1997) 884-890
20. Goulis J., Leandro G., and Burroughs A. Randomised controlled trials of ursodeoxycholic-acid therapy for primary biliary cirrhosis: a meta-analysis. Lancet 354 (1999) 1053-1060
21. Gong Y., Huang Z., Christensen E., and Gluud C. Ursodeoxycholic acid for patients with primary biliary cirrhosis: an updated systematic review and meta-analysis of randomized clinical trials using Bayesian approach as sensitivity analyses. Am J Gastroenterol 102 (2007) 1799-1807
22. Shi J., Wu C., Lin Y., Chen Y.X., Zhu L., and Xie W.F. Long-term effects of mid-dose ursodeoxycholic acid in primary biliary cirrhosis: a meta-analysis of randomized controlled trials. Am J Gastroenterol 101 (2006) 1529-1538
23. Lindor K.D., Poupon R., Heathcote E.J., and Therneau T. Ursodeoxycholic acid for primary biliary cirrhosis. Lancet 355 (2000) 657-658
24. Angulo P., Dickson E.R., Therneau T.M., Jorgensen R.A., Smith C., DeSotel C.K., et al. Comparison of three doses of ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a randomized trial. J Hepatol 30 (1999) 830-835
25. Corpechot C., Carrat F., Bahr A., Chretien Y., Poupon R.E., and Poupon R. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 128 (2005) 297-303
26. ter Borg P.C., Schalm S.W., Hansen B.E., and van Buuren H.R. Prognosis of ursodeoxycholic acid-treated patients with primary biliary cirrhosis. Results of a 10-yr cohort study involving 297 patients. Am J Gastroenterol 101 (2006) 2044-2050
27. Pares A., Caballeria L., and Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 130 (2006) 715-720
28. Corpechot C., Abenavoli L., Rabahi N., Chretien Y., Andreani T., Johanet C., et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 48 (2008) 871-877
29. Mitchison H.C., Bassendine M.F., Malcolm A.J., Watson A.J., Record C.O., and James O.F. A pilot, double-blind, controlled 1-year trial of prednisolone treatment in primary biliary cirrhosis: hepatic improvement but greater bone loss. Hepatology 10 (1989) 420-429
30. Leuschner M., Guldutuna S., You T., Hubner K., Bhatti S., and Leuschner U. Ursodeoxycholic acid and prednisolone versus ursodeoxycholic acid and placebo in the treatment of early stages of primary biliary cirrhosis. J Hepatol 25 (1996) 49-57
31. Leuschner M., Maier K.P., Schlichting J., Strahl S., Herrmann G., Dahm H.H., et al. Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial. Gastroenterology 117 (1999) 918-925
32. Rautiainen H., Karkkainen P., Karvonen A.L., Nurmi H., Pikkarainen P., Nuutinen H., et al. Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial. Hepatology 41 (2005) 747-752
33. Angulo P., Jorgensen R.A., Keach J.C., Dickson E.R., Smith C., and Lindor K.D. Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology 31 (2000) 318-323
34. Hempfling W., Grunhage F., Dilger K., Reichel C., Beuers U., and Sauerbruch T. Pharmacokinetics and pharmacodynamic action of budesonide in early- and late-stage primary biliary cirrhosis. Hepatology 38 (2003) 196-202
35. Christensen E., Neuberger J., Crowe J., Altman D.G., Popper H., Portmann B., et al. Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis. Final results of an international trial. Gastroenterology 89 (1985) 1084-1091
36. Lombard M., Portmann B., Neuberger J., Williams R., Tygstrup N., Ranek L., et al. Cyclosporin A treatment in primary biliary cirrhosis: results of a long-term placebo controlled trial. Gastroenterology 104 (1993) 519-526
37. Hendrickse M.T., Rigney E., Giaffer M.H., Soomro I., Triger D.R., Underwood J.C., et al. Low-dose methotrexate is ineffective in primary biliary cirrhosis: long-term results of a placebo-controlled trial. Gastroenterology 117 (1999) 400-407
38. Gonzalez-Koch A., Brahm J., Antezana C., Smok G., and Cumsille M.A. The combination of ursodeoxycholic acid and methotrexate for primary biliary cirrhosis is not better than ursodeoxycholic acid alone. J Hepatol 27 (1997) 143-149
39. Combes B., Emerson S.S., Flye N.L., Munoz S.J., Luketic V.A., Mayo M.J., et al. Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. Hepatology 42 (2005) 1184-1193
40. Hoofnagle J.H., Davis G.L., Schafer D.F., Peters M., Avigan M.I., Pappas S.C., et al. Randomized trial of chlorambucil for primary biliary cirrhosis. Gastroenterology 91 (1986) 1327-1334
41. Talwalkar J.A., Angulo P., Keach J.C., Petz J.L., Jorgensen R.A., and Lindor K.D. Mycophenolate mofetil for the treatment of primary biliary cirrhosis in patients with an incomplete response to ursodeoxycholic acid. J Clin Gastroenterol 39 (2005) 168-171
42. Vuoristo M., Farkkila M., Karvonen A.L., Leino R., Lehtola J., Makinen J., et al. A placebo-controlled trial of primary biliary cirrhosis treatment with colchicine and ursodeoxycholic acid. Gastroenterology 108 (1995) 1470-1478
43. Poupon R.E., Huet P.M., Poupon R., Bonnand A.M., Nhieu J.T., and Zafrani E.S. A randomized trial comparing colchicine and ursodeoxycholic acid combination to ursodeoxycholic acid in primary biliary cirrhosis. UDCA-PBC Study Group. Hepatology 24 (1996) 1098-1103
44. Neuberger J., Christensen E., Portmann B., Caballeria J., Rodes J., Ranek L., et al. Double blind controlled trial of d-penicillamine in patients with primary biliary cirrhosis. Gut 26 (1985) 114-119
45. Gong Y., Klingenberg S.L., and Gluud C. Systematic review and meta-analysis: d-penicillamine vs. placebo/no intervention in patients with primary biliary cirrhosis-Cochrane Hepato-Biliary Group. Aliment Pharmacol Ther 24 (2006) 1535-1544
46. The results of a randomized double blind controlled trial evaluating malotilate in primary biliary cirrhosis. A European multicentre study group. J Hepatol 1993;17:227-35.
47. McCormick P.A., Scott F., Epstein O., Burroughs A.K., Scheuer P.J., and McIntyre N. Thalidomide as therapy for primary biliary cirrhosis: a double-blind placebo controlled pilot study. J Hepatol 21 (1994) 496-499
48. Angulo P., Patel T., Jorgensen R.A., Therneau T.M., and Lindor K.D. Silymarin in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology 32 (2000) 897-900
49. Stojakovic T., Putz-Bankuti C., Fauler G., Scharnagl H., Wagner M., Stadlbauer V., et al. Atorvastatin in patients with primary biliary cirrhosis and incomplete biochemical response to ursodeoxycholic acid. Hepatology 46 (2007) 776-784
50. Leuschner M., Holtmeier J., Ackermann H., and Leuschner U. The influence of sulindac on patients with primary biliary cirrhosis that responds incompletely to ursodeoxycholic acid: a pilot study. Eur J Gastroenterol Hepatol 14 (2002) 1369-1376
51. Iwasaki S., Ohira H., Nishiguchi S., Zeniya M., Kaneko S., Onji M., et al. The efficacy of ursodeoxycholic acid and bezafibrate combination therapy for primary biliary cirrhosis: a prospective, multicenter study. Hepatol Res 38 (2008) 557-564
52. Invernizzi P., Alvaro D., Crosignani A., Gaudio E., and Podda M. Tamoxifen in treatment of primary biliary cirrhosis. Hepatology 39 (2004) 1175-1176
53. Mason A.L., Farr G.H., Xu L., Hubscher S.G., and Neuberger J.M. Pilot studies of single and combination antiretroviral therapy in patients with primary biliary cirrhosis. Am J Gastroenterol 99 (2004) 2348-2355
54. MacQuillan G.C., and Neuberger J. Liver transplantation for primary biliary cirrhosis. Clin Liver Dis 7 (2003) 941-956
55. Milkiewicz P. Liver transplantation in primary biliary cirrhosis. Clin Liver Dis 12 (2008) 461-472
56. Gautam M., Cheruvattath R., and Balan V. Recurrence of autoimmune liver disease after liver transplantation: a systematic review. Liver Transpl 12 (2006) 1813-1824
57. Chazouilleres O., Wendum D., Serfaty L., Montembault S., Rosmorduc O., and Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 28 (1998) 296-301
58. Lohse A.W., zum Buschenfelde K.H., Franz B., Kanzler S., Gerken G., and Dienes H.P. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology 29 (1999) 1078-1084
59. Woodward J., and Neuberger J. Autoimmune overlap syndromes. Hepatology 33 (2001) 994-1002
60. Beuers U., and Rust C. Overlap syndromes. Semin Liver Dis 25 (2005) 311-320
61. Heathcote E.J. Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system. Am J Gastroenterol 97 (2002) 1090-1092
62. Alvarez F., Berg P.A., Bianchi F.B., Bianchi L., Burroughs A.K., Cancado E.L., et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 31 (1999) 929-938
63. Talwalkar J.A., Keach J.C., Angulo P., and Lindor K.D. Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system. Am J Gastroenterol 97 (2002) 1191-1197
64. Chazouilleres O., Wendum D., Serfaty L., Rosmorduc O., and Poupon R. Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. J Hepatol 44 (2006) 400-406
65. Hennes E.M., Zeniya M., Czaja A.J., Pares A., Dalekos G.N., Krawitt E.L., et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 48 (2008) 169-176
66. Yamamoto K., Terada R., Okamoto R., Hiasa Y., Abe M., Onji M., et al. A scoring system for primary biliary cirrhosis and its application for variant forms of autoimmune liver disease. J Gastroenterol 38 (2003) 52-59
67. Poupon R., Chazouilleres O., Corpechot C., and Chretien Y. Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis. Hepatology 44 (2006) 85-90
68. Czaja A.J. The variant forms of autoimmune hepatitis. Ann Intern Med 125 (1996) 588-598
69. Rust C., and Beuers U. Overlap syndromes among autoimmune liver diseases. World J Gastroenterol 14 (2008) 3368-3373
70. Silveira M.G., Talwalkar J.A., Angulo P., and Lindor K.D. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol 102 (2007) 1244-1250
71. Joshi S., Cauch-Dudek K., Wanless I.R., Lindor K.D., Jorgensen R., Batts K., et al. Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. Hepatology 35 (2002) 409-413
72. Csepregi A., Rocken C., Treiber G., and Malfertheiner P. Budesonide induces complete remission in autoimmune hepatitis. World J Gastroenterol 12 (2006) 1362-1366
73. Duclos-Vallee J.C., Hadengue A., Ganne-Carrie N., Robin E., Degott C., and Erlinger S. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Corticoresistance and effective treatment by cyclosporine A. Dig Dis Sci 40 (1995) 1069-1073
74. Maggs J.R., and Chapman R.W. An update on primary sclerosing cholangitis. Curr Opin Gastroenterol 24 (2008) 377-383
75. Karlsen T.H., Schrumpf E., and Boberg K.M. Genetic epidemiology of primary sclerosing cholangitis. World J Gastroenterol 13 (2007) 5421-5431
76. Abdalian R., and Heathcote E.J. Sclerosing cholangitis: a focus on secondary causes. Hepatology 44 (2006) 1063-1074
77. Broome U., Olsson R., Loof L., Bodemar G., Hultcrantz R., Danielsson A., et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 38 (1996) 610-615
78. Chapman R.W., Arborgh B.A., Rhodes J.M., Summerfield J.A., Dick R., Scheuer P.J., et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 21 (1980) 870-877
79. Tischendorf J.J., Hecker H., Kruger M., Manns M.P., and Meier P.N. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: a single center study. Am J Gastroenterol 102 (2007) 107-114
80. Boberg K.M., Fausa O., Haaland T., Holter E., Mellbye O.J., Spurkland A., et al. Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis. Hepatology 23 (1996) 1369-1376
81. Mendes F.D., Jorgensen R., Keach J., Katzmann J.A., Smyrk T., Donlinger J., et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 101 (2006) 2070-2075
82. Hov J.R., Boberg K.M., and Karlsen T.H. Autoantibodies in primary sclerosing cholangitis. World J Gastroenterol 14 (2008) 3781-3791
83. Ludwig J. Surgical pathology of the syndrome of primary sclerosing cholangitis. Am J Surg Pathol 13 (1989) 43-49
84. van de Meeberg P.C., Portincasa P., Wolfhagen F.H., van Erpecum K.J., and VanBerge-Henegouwen G.P. Increased gall bladder volume in primary sclerosing cholangitis. Gut 39 (1996) 594-599
85. Said K., Glaumann H., and Bergquist A. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 48 (2008) 598-605
86. Lee Y.M., and Kaplan M.M. Primary sclerosing cholangitis. N Engl J Med 332 (1995) 924-933
87. MacCarty R.L., LaRusso N.F., Wiesner R.H., and Ludwig J. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology 149 (1983) 39-44
88. Bangarulingam S.Y., Gossard A.A., Petersen B.T., Ott B.J., and Lindor K.D. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 104 (2009) 855-860
89. Berstad A.E., Aabakken L., Smith H.J., Aasen S., Boberg K.M., and Schrumpf E. Diagnostic accuracy of magnetic resonance and endoscopic retrograde cholangiography in primary sclerosing cholangitis. Clin Gastroenterol Hepatol 4 (2006) 514-520
90. Angulo P., Pearce D.H., Johnson C.D., Henry J.J., LaRusso N.F., Petersen B.T., et al. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol 33 (2000) 520-527
91. Bjornsson E., Olsson R., Bergquist A., Lindgren S., Braden B., Chapman R.W., et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 134 (2008) 975-980
92. Broome U., Glaumann H., Lindstom E., Loof L., Almer S., Prytz H., et al. Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC). J Hepatol 36 (2002) 586-589
93. Angulo P., Maor-Kendler Y., and Lindor K.D. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Hepatology 35 (2002) 1494-1500
94. Bjornsson E., Boberg K.M., Cullen S., Fleming K., Clausen O.P., Fausa O., et al. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 51 (2002) 731-735
95. Gotthardt D., Runz H., Keitel V., Fischer C., Flechtenmacher C., Wirtenberger M., et al. A mutation in the canalicular phospholipid transporter gene, ABCB4, is associated with cholestasis, ductopenia, and cirrhosis in adults. Hepatology 48 (2008) 1157-1166
96. Wilschanski M., Chait P., Wade J.A., Davis L., Corey M., St. Louis P., et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology 22 (1995) 1415-1422
97. Feldstein A.E., Perrault J., El-Youssif M., Lindor K.D., Freese D.K., and Angulo P. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology 38 (2003) 210-217
98. Gregorio G.V., Portmann B., Karani J., Harrison P., Donaldson P.T., Vergani D., et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 33 (2001) 544-553
99. Tanaka A., Takamori Y., Toda G., Ohnishi S., and Takikawa H. Outcome and prognostic factors of 391 Japanese patients with primary sclerosing cholangitis. Liver Int 28 (2008) 983-989
100. Loftus Jr. E.V., Harewood G.C., Loftus C.G., Tremaine W.J., Harmsen W.S., Zinsmeister A.R., et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 54 (2005) 91-96
101. Soetikno R.M., Lin O.S., Heidenreich P.A., Young H.S., and Blackstone M.O. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis. Gastrointest Endosc 56 (2002) 48-54
102. Broome U., and Bergquist A. Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer. Semin Liver Dis 26 (2006) 31-41
103. Bergquist A., Ekbom A., Olsson R., Kornfeldt D., Loof L., Danielsson A., et al. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 36 (2002) 321-327
104. Lazaridis K.N., and Gores G.J. Primary sclerosing cholangitis and cholangiocarcinoma. Semin Liver Dis 26 (2006) 42-51
105. Harnois D.M., Gores G.J., Ludwig J., Steers J.L., LaRusso N.F., and Wiesner R.H. Are patients with cirrhotic stage primary sclerosing cholangitis at risk for the development of hepatocellular cancer?. J Hepatol 27 (1997) 512-516
106. Fevery J., Verslype C., Lai G., Aerts R., and Van Steenbergen W. Incidence, diagnosis, and therapy of cholangiocarcinoma in patients with primary sclerosing cholangitis. Dig Dis Sci 52 (2007) 3123-3135
107. Charatcharoenwitthaya P., Enders F.B., Halling K.C., and Lindor K.D. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. Hepatology 48 (2008) 1106-1117
108. Prytz H., Keiding S., Bjornsson E., Broome U., Almer S., Castedal M., et al. Dynamic FDG-PET is useful for detection of cholangiocarcinoma in patients with PSC listed for liver transplantation. Hepatology 44 (2006) 1572-1580
109. Boberg K.M., Jebsen P., Clausen O.P., Foss A., Aabakken L., and Schrumpf E. Diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 45 (2006) 568-574
110. Chazouilleres O., Poupon R., Capron J.P., Metman E.H., Dhumeaux D., Amouretti M., et al. Ursodeoxycholic acid for primary sclerosing cholangitis. J Hepatol 11 (1990) 120-123
111. O'Brien C.B., Senior J.R., Arora-Mirchandani R., Batta A.K., and Salen G. Ursodeoxycholic acid for the treatment of primary sclerosing cholangitis: a 30-month pilot study. Hepatology 14 (1991) 838-847
112. Beuers U., Spengler U., Kruis W., Aydemir U., Wiebecke B., Heldwein W., et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology 16 (1992) 707-714
113. Stiehl A. Ursodeoxycholic acid therapy in treatment of primary sclerosing cholangitis. Scand J Gastroenterol Suppl 204 (1994) 59-61
114. Lindor K.D. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 336 (1997) 691-695
115. Mitchell S.A., Bansi D.S., Hunt N., Von Bergmann K., Fleming K.A., and Chapman R.W. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 121 (2001) 900-907
116. Cullen S.N., Rust C., Fleming K., Edwards C., Beuers U., and Chapman R. High dose ursodeoxycholic acid for the treatment of primary sclerosig cholangitis is safe and effective. J Hepatol 48 (2008) 792-800
117. Olsson R., Boberg K.M., de Muckadell O.S., Lindgren S., Hultcrantz R., Folvik G., et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology 129 (2005) 1464-1472
118. Lindor K.D., Enders F.B., Schmoll J.A., Hoskin T.L., Jorgensen R.A., Petz J.L., et al. Randomized, double-blind controlled trial of high-dose ursodeoxycholic acid for primary sclerosing cholangitis. Hepatology 48 (2008) 378A
119. Tung B.Y., Emond M.J., Haggitt R.C., Bronner M.P., Kimmey M.B., Kowdley K.V., et al. Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Ann Intern Med 134 (2001) 89-95
120. Wolf J.M., Rybicki L.A., and Lashner B.A. The impact of ursodeoxycholic acid on cancer, dysplasia and mortality in ulcerative colitis patients with primary sclerosing cholangitis. Aliment Pharmacol Ther 22 (2005) 783-788
121. Pardi D.S., Loftus Jr. E.V., Kremers W.K., Keach J., and Lindor K.D. Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis. Gastroenterology 124 (2003) 889-893
122. Rudolph G., Kloeters-Plachky P., Rost D., and Stiehl A. The incidence of cholangiocarcinoma in primary sclerosing cholangitis after long-time treatment with ursodeoxycholic acid. Eur J Gastroenterol Hepatol 19 (2007) 487-491
123. Brandsaeter B., Isoniemi H., Broome U., Olausson M., Backman L., Hansen B., et al. Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 40 (2004) 815-822
124. Boberg K.M., Egeland T., and Schrumpf E. Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol 38 (2003) 991-995
125. Etzel J.P., Eng S.C., Ko C.W., Lee S.D., Saunders M.D., Tung B.Y., et al. Complications after ERCP in patients with primary sclerosing cholangitis. Gastrointest Endosc 67 (2008) 643-648
126. Andriulli A., Loperfido S., Napolitano G., Niro G., Valvano M.R., Spirito F., et al. Incidence rates of post-ERCP complications: a systematic survey of prospective studies. Am J Gastroenterol 102 (2007) 1781-1788
127. van den Hazel S.J., Wolfhagen E.H., van Buuren H.R., van de Meeberg P.C., and Van Leeuwen D.J. Prospective risk assessment of endoscopic retrograde cholangiography in patients with primary sclerosing cholangitis. Dutch PSC Study Group. Endoscopy 32 (2000) 779-782
128. Bjornsson E., Lindqvist-Ottosson J., Asztely M., and Olsson R. Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 99 (2004) 502-508
129. Hammel P., Couvelard A., O'Toole D., Ratouis A., Sauvanet A., Flejou J.F., et al. Regression of liver fibrosis after biliary drainage in patients with chronic pancreatitis and stenosis of the common bile duct. N Engl J Med 344 (2001) 418-423
130. Stiehl A., Rudolph G., Klo-ters-Plachky P., Sauer P., and Walker S. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol 36 (2002) 151-156
131. Baluyut A.R., Sherman S., Lehman G.A., Hoen H., and Chalasani N. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 53 (2001) 308-312
132. Johnson G.K., Geenen J.E., Venu R.P., Schmalz M.J., and Hogan W.J. Endoscopic treatment of biliary tract strictures in sclerosing cholangitis: a larger series and recommendations for treatment. Gastrointest Endosc 37 (1991) 38-43
133. Stiehl A., Rudolph G., Sauer P., Benz C., Stremmel W., Walker S., et al. Efficacy of ursodeoxycholic acid treatment and endoscopic dilation of major duct stenoses in primary sclerosing cholangitis. An 8-year prospective study. J Hepatol 26 (1997) 560-566
134. van Milligen de Wit A.W., Rauws E.A., van Bracht J., Mulder C.J., Jones E.A., Tytgat G.N., et al. Lack of complications following short-term stent therapy for extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc 46 (1997) 344-347
135. Kaya M., Petersen B.T., Angulo P., Baron T.H., Andrews J.C., Gostout C.J., et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 96 (2001) 1059-1066
136. Welsh F.K., and Wigmore S.J. Roux-en-Y choledochojejunostomy is the method of choice for biliary reconstruction in liver transplantation for primary sclerosing cholangitis. Transplantation 77 (2004) 602-604
137. Gordon F. Recurrent primary sclerosing cholangitis: clinical diagnosis and long-term management issues. Liver Transpl 12 (2006) S73-S75
138. LaRusso N.F., Shneider B.L., Black D., Gores G.J., James S.P., Doo E., et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology 44 (2006) 746-764
139. Cholongitas E., Shusang V., Papatheodoridis G.V., Marelli L., Manousou P., Rolando N., et al. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl 14 (2008) 138-143
140. el-Shabrawi M., Wilkinson M.L., Portmann B., Mieli-Vergani G., Chong S.K., Williams R., et al. Primary sclerosing cholangitis in childhood. Gastroenterology 92 (1987) 1226-1235
141. Minuk G.Y., Sutherland L.R., Pappas G., Kelly J.K., and Martin S.E. Autoimmune chronic active hepatitis (lupoid hepatitis) and primary sclerosing cholangitis in two young adult females. Can J Gastroenterol 2 (1988) 22-27
142. Rabinovitz M., Demetris A.J., Bou-Abboud C.F., and Van Thiel D.H. Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 37 (1992) 1606-1611
143. Lawrence S.P., Sherman K.E., Lawson J.M., and Goodman Z.D. A 39 year old man with chronic hepatitis. Semin Liver Dis 14 (1994) 97-105
144. Debray D., Pariente D., Urvoas E., Hadchouel M., and Bernard O. Sclerosing cholangitis in children. J Pediatr 124 (1994) 49-56
145. Wilschanski M., Chait P., Wade J.A., Davis L., Corey M., St. Louis P., et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology 22 (1995) 1415-1422
146. Gohlke F., Lohse A.W., Dienes H.P., Lohr H., Marker-Hermann E., Gerken G., et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol 24 (1996) 699-705
147. McNair A.N., Moloney M., Portmann B.C., Williams R., and McFarlane I.G. Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five cases. Am J Gastroenterol 93 (1998) 777-784
148. Al-Chalabi T., Portmann B.C., Bernal W., McFarlane I.G., and Heneghan M.A. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival. Aliment Pharmacol Ther 28 (2008) 209-220
149. van Buuren H.R., van Hoogstraten H.J.E., Terkivatan T., Schalm S.W., and Vleggaar F.P. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 33 (2000) 543-548
150. Kaya M., Angulo P., and Lindor K.D. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 33 (2000) 537-542
151. Floreani A., Rizzotto E.R., Ferrara F., Carderi I., Caroli D., Blasone L., et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol 100 (2005) 1516-1522
152. Abdo A.A., Bain V.G., Kichian K., and Lee S.S. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology 36 (2002) 1393-1399
153. Stathopoulos G., Nourmand A.D., Blackstone M., Andersen D., and Baker A.L. Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor. J Clin Gastroenterol 21 (1995) 143-148
154. Erkelens G.W., Vleggaar F.P., Lesterhuis W., van Buuren H.R., and van der Werf S.D. Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet 354 (1999) 43-44
155. van Buuren H.R., Vleggaar F.P., Willemien Erkelens G., Zondervan P.E., Lesterhuis W., Van Eijck C.H., et al. Autoimmune pancreatocholangitis: a series of ten patients. Scand J Gastroenterol Suppl 243 (2006) 70-78
156. Bjornsson E., Chari S.T., Smyrk T.C., and Lindor K. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 45 (2007) 1547-1554
157. Umemura T., Zen Y., Hamano H., Kawa S., Nakanuma Y., and Kiyosawa K. Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology 46 (2007) 463-471
158. Zen Y., Fujii T., Harada K., Kawano M., Yamada K., Takahira M., et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 45 (2007) 1538-1546
159. Ghazale A., Chari S.T., Zhang L., Smyrk T.C., Takahashi N., Levy M.J., et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 134 (2008) 706-715
160. Colombo C., Battezzati P.M., Crosignani A., Morabito A., Costantini D., Padoan R., et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology 36 (2002) 1374-1382
161. Sokol R.J., and Durie P.R. Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr 28 (1999) S1-S13
162. Colombo C., Battezzati P.M., Podda M., Bettinardi N., and Giunta A. Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-blind multicenter trial. The Italian Group for the Study of Ursodeoxycholic Acid in Cystic Fibrosis. Hepatology 23 (1996) 1484-1490
163. van de Meeberg P.C., Houwen R.H., Sinaasappel M., Heijerman H.G., Bijleveld C.M., and Vanberge-Henegouwen G.P. Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease. Results of a randomized study with 1-year follow-up. Scand J Gastroenterol 32 (1997) 369-373
164. Lindblad A., Glaumann H., and Strandvik B. A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease. Hepatology 27 (1998) 166-174
165. Debray D., Lykavieris P., Gauthier F., Dousset B., Sardet A., Munck A., et al. Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension. J Hepatol 31 (1999) 77-83
166. Oude Elferink R.P., Paulusma C.C., and Groen A.K. Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseases. Gastroenterology 130 (2006) 908-925
167. de Vree J.M., Jacquemin E., Sturm E., Cresteil D., Bosma P.J., Aten J., et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci USA 95 (1998) 282-287
168. Paulusma C.C., Groen A., Kunne C., Ho-Mok K.S., Spijkerboer A.L., Rudi de Waart D., et al. Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport. Hepatology 44 (2006) 195-204
169. Strautnieks S.S., Bull L.N., Knisely A.S., Kocoshis S.A., Dahl N., Arnell H., et al. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat Genet 20 (1998) 233-238
170. Knisely A.S., Strautnieks S.S., Meier Y., Stieger B., Byrne J.A., Portmann B.C., et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 44 (2006) 478-486
171. Trauner M., Fickert P., and Wagner M. MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. Semin Liver Dis 27 (2007) 77-98
172. Jacquemin E. Role of multidrug resistance 3 deficiency in pediatric and adult liver disease: one gene for three diseases. Semin Liver Dis 21 (2001) 551-562
173. Whitington P.F., and Whitington G.L. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 95 (1988) 130-136
174. Arnell H., Bergdahl S., Papadogiannakis N., Nemeth A., and Fischler B. Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis. J Pediatr Surg 43 (2008) 1312-1320
175. van Ooteghem N.A., Klomp L.W., van Berge-Henegouwen G.P., and Houwen R.H. Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum. J Hepatol 36 (2002) 439-443
176. Stapelbroek J.M., van Erpecum K.J., Klomp L.W., Venneman N.G., Schwartz T.P., van Berge Henegouwen G.P., et al. Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis. Hepatology 43 (2006) 51-53
177. Piccoli D.A., and Spinner N.B. Alagille syndrome and the Jagged1 gene. Semin Liver Dis 21 (2001) 525-534
178. Benichou C. Criteria of drug-induced liver disorders. Report of an international consensus meeting. J Hepatol 11 (1990) 272-276
179. Andrade R.J., Lucena M.I., Fernandez M.C., Pelaez G., Pachkoria K., Garcia-Ruiz E., et al. Drug-induced liver injury: an analysis of 461 incidences submitted to the Spanish registry over a 10-year period. Gastroenterology 129 (2005) 512-521
180. Kaplowitz N. Idiosyncratic drug hepatotoxicity. Nat Rev Drug Discov 4 (2005) 489-499
181. Marschall H.U., Wagner M., Zollner G., and Trauner M. Clinical hepatotoxicity. Regulation and treatment with inducers of transport and cofactors. Mol Pharm 4 (2007) 895-910
182. Maddrey W.C. Drug-induced hepatotoxicity: 2005. J Clin Gastroenterol 39 (2005) S83-S89
183. Erlinger S. Drug-induced cholestasis. J Hepatol 26 (1997) S1-S4
184. Navarro V.J., and Senior J.R. Drug-related hepatotoxicity. N Engl J Med 354 (2006) 731-739
185. Nathwani R.A., and Kaplowitz N. Drug hepatotoxicity. Clin Liver Dis 10 (2006) 207-217
186. Aithal P.G., and Day C.P. The natural history of histologically proved drug induced liver disease. Gut 44 (1999) 731-735
187. Desmet V.J. Vanishing bile duct syndrome in drug-induced liver disease. J Hepatol 26 (1997) S31-S35
188. Lammert F., Marschall H.U., Glantz A., and Matern S. Intrahepatic cholestasis of pregnancy: molecular pathogenesis, diagnosis and management. J Hepatol 33 (2000) 1012-1021
189. Pusl T., and Beuers U. Intrahepatic cholestasis of pregnancy. Orphanet J Rare Dis 2 (2007) 26
190. Glantz A., Marschall H.U., and Mattsson L.A. Intrahepatic cholestasis of pregnancy: relationships between bile acid levels and fetal complication rates. Hepatology 40 (2004) 467-474
191. Royal College of Obstetricians and Gynaecologists. Obstetric cholestasis. RCOG Guideline No. 43, 2006:1-10.
192. Brites D. Intrahepatic cholestasis of pregnancy: changes in maternal-fetal bile acid balance and improvement by ursodeoxycholic acid. Ann Hepatol 1 (2002) 20-28
193. Palma J., Reyes H., Ribalta J., Hernandez I., Sandoval L., Almuna R., et al. Ursodeoxycholic acid in the treatment of cholestasis of pregnancy: a randomized, double-blind study controlled with placebo. J Hepatol 27 (1997) 1022-1028
194. Williamson C., Hems L.M., Goulis D.G., Walker I., Chambers J., Donaldson O., et al. Clinical outcome in a series of cases of obstetric cholestasis identified via a patient support group. BJOG 111 (2004) 676-681
195. Lee R.H., Kwok K.M., Ingles S., Wilson M.L., Mullin P., Incerpi M., et al. Pregnancy outcomes during an era of aggressive management for intrahepatic cholestasis of pregnancy. Am J Perinatol 25 (2008) 341-345
196. Hay J.E. Liver disease in pregnancy. Hepatology 47 (2008) 1067-1076
197. Glantz A., Marschall H.U., Lammert F., and Mattsson L.A. Intrahepatic cholestasis of pregnancy: a randomized controlled trial comparing dexamethasone and ursodeoxycholic acid. Hepatology 42 (2005) 1399-1405
198. Kondrackiene J., Beuers U., and Kupcinskas L. Efficacy and safety of ursodeoxycholic acid versus cholestyramine in intrahepatic cholestasis of pregnancy. Gastroenterology 129 (2005) 894-901
199. Binder T., Salaj P., Zima T., and Vitek L. Randomized prospective comparative study of ursodeoxycholic acid and S-adenosyl-l-methionine in the treatment of intrahepatic cholestasis of pregnancy. J Perinat Med 34 (2006) 383-391
200. Roncaglia N., Locatelli A., Arreghini A., Assi F., Cameroni I., Pezzullo J.C., et al. A randomised controlled trial of ursodeoxycholic acid and S-adenosyl-l-methionine in the treatment of gestational cholestasis. BJOG 111 (2004) 17-21
201. Mazzella G., Nicola R., Francesco A., Patrizia S., Luciano B., Anna M., et al. Ursodeoxycholic acid administration in patients with cholestasis of pregnancy: effects on primary bile acids in babies and mothers. Hepatology 33 (2001) 504-508
202. Kenyon A.P., Piercy C.N., Girling J., Williamson C., Tribe R.M., and Shennan A.H. Obstetric cholestasis, outcome with active management: a series of 70 cases. BJOG 109 (2002) 282-288
203. Roncaglia N., Arreghini A., Locatelli A., Bellini P., Andreotti C., and Ghidini A. Obstetric cholestasis: outcome with active management. Eur J Obstet Gynecol Reprod Biol 100 (2002) 167-170
204. Ko C.W., Beresford S.A., Schulte S.J., Matsumoto A.M., and Lee S.P. Incidence, natural history, and risk factors for biliary sludge and stones during pregnancy. Hepatology 41 (2005) 359-365
205. Kahaleh M., Hartwell G.D., Arseneau K.O., Pajewski T.N., Mullick T., Isin G., et al. Safety and efficacy of ERCP in pregnancy. Gastrointest Endosc 60 (2004) 287-292
206. Tham T.C., Vandervoort J., Wong R.C., Montes H., Roston A.D., Slivka A., et al. Safety of ERCP during pregnancy. Am J Gastroenterol 98 (2003) 308-311
207. Cohen L.B., Delegge M.H., Aisenberg J., Brill J.V., Inadomi J.M., Kochman M.L., et al. AGA Institute review of endoscopic sedation. Gastroenterology 133 (2007) 675-701
208. Mahadevan U., and Kane S. American gastroenterological association institute technical review on the use of gastrointestinal medications in pregnancy. Gastroenterology 131 (2006) 283-311
209. Poupon R., Chretien Y., Chazouilleres O., and Poupon R.E. Pregnancy in women with ursodeoxycholic acid-treated primary biliary cirrhosis. J Hepatol 42 (2005) 418-419
210. Hempfling W., Dilger K., and Beuers U. Systematic review: ursodeoxycholic acid-adverse effects and drug interactions. Aliment Pharmacol Ther 18 (2003) 963-972
211. Park-Wyllie L., Mazzotta P., Pastuszak A., Moretti M.E., Beique L., Hunnisett L., et al. Birth defects after maternal exposure to corticosteroids: prospective cohort study and meta-analysis of epidemiological studies. Teratology 62 (2000) 385-392
212. Armenti V.T., Moritz M.J., Cardonick E.H., and Davison J.M. Immunosuppression in pregnancy: choices for infant and maternal health. Drugs 62 (2002) 2361-2375
213. Benjaminov F.S., and Heathcote J. Liver disease in pregnancy. Am J Gastroenterol 99 (2004) 2479-2488
214. Kremer A.E., Beuers U., Oude-Elferink R.P., and Pusl T. Pathogenesis and treatment of pruritus in cholestasis. Drugs 68 (2008) 2163-2182
215. Datta D.V., and Sherlock S. Cholestyramine for long term relief of the pruritus complicating intrahepatic cholestasis. Gastroenterology 50 (1966) 323-332
216. Rust C., Sauter G.H., Oswald M., Buttner J., Kullak-Ublick G.A., Paumgartner G., et al. Effect of cholestyramine on bile acid patterns and synthesis during administration of ursodeoxycholic acid in man. Eur J Clin Invest 30 (2000) 135-139
217. Khurana S., and Singh P. Rifampin is safe for the treatment of pruritus die to chronic cholestasis: a meta-analysis of prospective randomized-controlled trials. Liver Int 26 (2006) 943-948
218. Tandon P., Rowe B.H., Vandermeer B., and Bain V.G. The efficacy and safety of bile acid binding agents, opioid antagonists or rifampicin in the treatment of cholestasis-associated pruritus. Am J Gastroenterol 102 (2007) 1528-1536
219. Bachs L., Pares A., Elena M., Piera C., and Rodes J. Effects of long-term rifampicin administration in primary biliary cirrhosis. Gastroenterology 102 (1992) 2077-2080
220. Prince M.I., Burt A.D., and Jones D.E.J. Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis. Gut 50 (2002) 436-439
221. Jones E.A., and Dekker R.L. Florid opioid withdrawal-like reaction precipitated by naltrexone in a patient with chronic cholestasis. Gastroenterology 118 (2000) 431-432
222. Jones E.A., Neuberger J.M., and Bergasa N.V. Opiate antagonist therapy for the pruritus of cholestasis: the avoidance of opioid withdrawal-like reactions. Q J Med 95 (2002) 547-552
223. Mayo M.J., Handem I., Saldana S., Jacobe H., Getachew Y., and Rush A.J. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology 45 (2007) 666-674
224. Pares A., Cisneros L., Salmeron J.M., Caballeria L., Mas A., Torras A., et al. Extracorporeal albumin dialysis: a procedure for prolonged relief of intractable pruritus in patients with primary biliary cirrhosis. Am J Gastroenterol 99 (2004) 1105-1110
225. Pusl T., Denk G.U., Parhofer K.G., and Beuers U. Plasma separation and anion adsorption transiently relieve intractable pruritus in primary biliary cirrhosis. J Hepatol 45 (2006) 887-891
226. Alallam A., Barth D., and Heathcote E.J. Role of plasmapheresis in the treatment of severe pruritus in pregnant patients with primary biliary cirrhosis: case reports. Can J Gastroenterol 22 (2008) 505-507
227. Beuers U., Gerken G., and Pusl T. Biliary drainage transiently relieves intractable pruritus in primary biliary cirrhosis. Hepatology 44 (2006) 280-281
228. Gross C.R., Malinchoc M., Kim W.R., Evans R.W., Wiesner R.H., Petz J.L., et al. Quality of life before and after liver transplantation for cholestatic liver disease. Hepatology 29 (1999) 356-364
229. Jones D.E.J. Fatigue in cholestatic liver disease: is it all in the mind?. J Hepatol 46 (2007) 992-994
230. Newton J.L., Pairman J., Sutcliffe K., Wilton K., and Jones D.E.J. A predictive model for fatigue and its aetiologica associations in primary biliary cirrhosis. Clin Gastroenterol Hepatol 6 (2008) 228-233
231. Newton J.L., Gibson J.G., Tomlinson M., Wilton K., and Jones D.E.J. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology 44 (2006) 91-98
232. Jones D.E., and Newton J.L. An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis. Aliment Pharmacol Ther 25 (2007) 471-476
233. 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 2008 Dec 12. [Epub ahead of print].
234. Jones D.E.J., Sutcliffe K., Pairman J., Wilton K., and Newton J.L. An integrated care pathway improves quality of life in primary biliary cirrhosis. QJM 101 (2008) 535-543
235. Pares A., and Guanabens N. Osteoporosis in primary biliary cirrhosis: pathogenesis and treatment. Clin Liver Dis 12 (2008) 407-424
236. Pereira S.P., O'Donohue J., Moniz C., Phillips M.G., Abraha H., Buxton-Thomas M., et al. Transdermal hormone replacement therapy improves veretebral bone density in primary biliary cirrhosis: results of a 1-year controlled trial. Aliment Pharmacol Therap 19 (2004) 563-570
237. Boone R.H., Cheung A.M., Girlan M., and Heathcote E.J. Osteoporosis in primary biliary cirrhosis: a randomized trial of the efficacy and feasibility of estrogen/progestin. Dig Dis Sci 51 (2006) 1103-1112
238. Guanabens N., Pares A., Ros I., Alvarez L., Pons F., Caballeria L., et al. Alendronate is more effective than etidronate for increasing bone mass in osteopenic patients with primary biliary cirrhosis. Am J Gastroenterol 98 (2003) 2268-2274
239. Musialik J., Petelenz M., and Gonciarz Z. Effects of alendronate on bone mass in patients with primary biliary cirrhosis and osteoporosis: preliminary results after one year. Scand J Gastroenterol 40 (2005) 873-874
240. Guanabens N., Pares A., del Rio L., Roca M., Gomez R., Munoz J., et al. Sodium fluoride prevents bone loss in primary biliary cirrhosis. J Hepatol 15 (1992) 345-349
241. Levy C., Harnois D.M., Angulo P., Jorgensen R., and Lindor K.D. Raloxifene improves bone mass in osteopenic women with primary biliary cirrhosis: results of a pilot study. Liver Int 25 (2005) 117-121
242. Newton J.L., Francis R., Prince M., James O.F.W., Bassendine M.F., Rawlings D., et al. Osteoporosis in primary biliary cirrhosis revisited. Gut 49 (2001) 282-287
243. Gores G.J., Wiesner R.H., Dickson E.R., Zinsmeister A.R., Jorgensen R.A., and Langworthy A. Prospective evaluation of esophageal varices in primary biliary cirrhosis: development, natural history, and influence on survival. Gastroenterology 96 (1989) 1552-1559
244. Jones D.E.J., Metcalf J.V., Collier J.D., Bassendine M.F., and James O.F.W. Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes. Hepatology 26 (1997) 1138-1142
245. Garcia-Tsao G., Sanyal A.J., Grace N.D., and Carey W. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology 46 (2007) 922-938
246. Bruix J., and Sherman M. Management of hepatocellular carcinoma. Hepatology 42 (2005) 1208-1236
247. Bressler B., Pinto R., El-Ashry D., and Heathcote E.J. Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic creeening for oesophageal varices detection. Gut 54 (2005) 407-410