Familial cholestasis: Progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy

Best Practice and Research: Clinical Gastroenterology

Volumn 24, Issue 5, 2010, Pages 541-553

  Van Der Woerd, Wendy L ^a,b   Van Mil, Saskia W C ^b   Stapelbroek, Janneke M ^a,b   Klomp, Leo W J ^b   Van De Graaf, Stan F J ^b   Houwen, Roderick H J ^a  

^a UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^b UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

ABCB11; ABCB4; ATP8B1; Benign recurrent intrahepatic cholestasis; BRIC; ICP; Intrahepatic cholestasis of pregnancy; PFIC; Progressive familial intrahepatic cholestasis

Indexed keywords

ALANINE AMINOTRANSFERASE; ASPARTATE AMINOTRANSFERASE; COLESTYRAMINE; PROTEIN; PROTEIN ABCB11; PROTEIN ABCB4; PROTEIN ATP8B1; RIFAMPICIN; UNCLASSIFIED DRUG; URSODEOXYCHOLIC ACID;

ALANINE AMINOTRANSFERASE BLOOD LEVEL; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS; CLINICAL FEATURE; FAMILIAL DISEASE; HETEROZYGOSITY; HUMAN; INTRAHEPATIC CHOLESTASIS; LIVER TRANSPLANTATION; OUTCOME ASSESSMENT; PREGNANCY; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS; RECURRENT DISEASE; REVIEW;

EID: 77958090115     PISSN: 15216918     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bpg.2010.07.010     Document Type: Article

References (97)

1. E. Jacquemin, J.M. de Vree, D. Cresteil, E.M. Sokal, E. Sturm, and M. Dumont The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood Gastroenterology 120 2001 1448 1458
2. S.W. van Mil, R.H. Houwen, and L.W. Klomp Genetics of familial intrahepatic cholestasis syndromes J Med Genet 42 2005 449 463
3. A. Davit-Spraul, M. Fabre, S. Branchereau, C. Baussan, E. Gonzales, and B. Stieger ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): phenotypic differences between PFIC1 and PFIC2 and natural history Hepatology 51 2010 1645 1655
4. S.W. van Mil, L.W. Klomp, L.N. Bull, and R.H. Houwen FIC1 disease: a spectrum of intrahepatic cholestatic disorders Semin Liver Dis 21 2001 535 544
5. N.A. van Ooteghem, L.W. Klomp, G.P. van Berge-Henegouwen, and R.H. Houwen Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum J Hepatol 36 2002 439 443
6. S.W. van Mil, W.L. van der Woerd, G. van der Brugge, E. Sturm, P.L. Jansen, and L.N. Bull Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11 Gastroenterology 127 2004 379 384
7. P. Ujhazy, D. Ortiz, S. Misra, S. Li, J. Moseley, and H. Jones Familial intrahepatic cholestasis 1: studies of localization and function Hepatology 34 2001 768 775
8. S.W. van Mil, M.M. van Oort, I.T. van den Berg, R. Berger, R.H. Houwen, and L.W. Klomp Fic1 is expressed at apical membranes of different epithelial cells in the digestive tract and is induced in the small intestine during postnatal development of mice Pediatr Res 56 2004 981 987
9. P. Verhulst, L. van der Velden, V. Oorschot, E.E. van Faassen, J. Klumperman, and R.H. Houwen A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells Hepatology 51 2010 2049 2060
10. S.Y. Cai, S. Gautam, T. Nguyen, C.J. Soroka, C. Rahner, and J.L. Boyer ATP8B1 deficiency disrupts the bile canalicular membrane bilayer structure in hepatocytes, but FXR expression and activity are maintained Gastroenterology 136 2009 1060 1069
11. C.C. Paulusma, D.R. de Waart, C. Kunne, K.S. Mok, and R.P. Oude Elferink Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content J Biol Chem 284 2009 9947 9954
12. N. Tygstrup, B.A. Steig, J.A. Juijn, L.N. Bull, and R.H. Houwen Recurrent familial intrahepatic cholestasis in the Faeroe Islands. Phenotypic heterogeneity but genetic homogeneity Hepatology 29 1999 506 508
13. P. Lykavieris, S. van Mil, D. Cresteil, M. Fabre, M. Hadchouel, and L. Klomp Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: no catch-up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation J Hepatol 39 2003 447 452
14. J.M. Stapelbroek, T.A. Peters, D.H. van Beurden, J.H. Curfs, A. Joosten, and A.J. Beynon ATP8B1 is essential for maintaining normal hearing Proc Natl Acad Sci U S A 106 2009 9709 9714
15. L.N. Bull, M.J. van Eijk, L. Pawlikowska, J.A. DeYoung, J.A. Juijn, and M. Liao A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis Nat Genet 18 1998 219 224
16. L.W. Klomp, J.C. Vargas, S.W. van Mil, L. Pawlikowska, S.S. Strautnieks, and M.J. van Eijk Characterization of mutations in ATP8B1 associated with hereditary cholestasis Hepatology 40 2004 27 38
17. L.Y. Liu, X.H. Wang, Z.L. Wang, Q.R. Zhu, and J.S. Wang Characterization of ATP8B1 gene mutations and a hot-linked mutation found in Chinese children with progressive intrahepatic cholestasis and low GGT J Pediatr Gastroenterol Nutr 50 2010 179 183
18. D.E. Folmer, V.A. van der Mark, K.S. Ho-Mok, R.P. Oude Elferink, and C.C. Paulusma Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1 Hepatology 50 2009 1597 1605
19. L.M. van der Velden, J.M. Stapelbroek, E. Krieger, P.V. van den Berghe, R. Berger, and P.M. Verhulst Folding defects in P-type ATP8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate Hepatology 51 2010 286 296
20. R. Brenard, A.P. Geubel, and J.P. Benhamou Benign recurrent intrahepatic cholestasis. A report of 26 cases J Clin Gastroenterol 11 1989 546 551
21. M. Vallejo, O. Briz, M.A. Serrano, M.J. Monte, and J.J. Marin Potential role of trans-inhibition of the bile salt export pump by progesterone metabolites in the etiopathogenesis of intrahepatic cholestasis of pregnancy J Hepatol 44 2006 1150 1157
22. H. Egawa, T. Yorifuji, R. Sumazaki, A. Kimura, M. Hasegawo, and K. Tanaka Intractable diarrhea after liver transplantation for Byler's disease: successful treatment with bile adsorptive resin Liver Transpl 8 2002 714 716
23. L. Pawlikowska, S. Strautnieks, I. Jankowska, P. Czubkowski, K. Emerick, and A. Antoniou Differences in presentation and progression between severe FIC1 and BSEP deficiencies J Hepatol 53 2010 170 178
24. P.F. Whitington, D.K. Freese, E.M. Alonso, S.J. Schwarzenberg, and H.L. Sharp Clinical and biochemical findings in progressive familial intrahepatic cholestasis J Pediatr Gastroenterol Nutr 18 1994 134 141
25. L.N. Bull, V.E. Carlton, N.L. Stricker, S. Baharloo, J.A. DeYoung, and N.B. Freimer Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity Hepatology 26 1997 155 164
26. A.S. Knisely Progressive familial intrahepatic cholestasis: a personal perspective Pediatr Dev Pathol 3 2000 113 125
27. S.S. Strautnieks, L.N. Bull, A.S. Knisely, S.A. Kocoshis, N. Dahl, and H. Arnell A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis Nat Genet 20 1998 233 238
28. T. Gerloff, B. Stieger, B. Hagenbuch, J. Madon, L. Landmann, and J. Roth The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver J Biol Chem 273 1998 10046 10050
29. S.S. Strautnieks, J.A. Byrne, L. Pawlikowska, D. Cebecauerová, A. Rayner, and L. Dutton Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families Gastroenterology 134 2008 1203 1214
30. S. Treepongkaruna, A. Gaensan, P. Pienvichit, O. Luksan, A.S. Knisely, and P. Sornmayura Novel ABCB11 mutations in a Thai infant with progressive familial intrahepatic cholestasis World J Gastroenterol 15 2009 4339 4342
31. L. Wang, H. Dong, C.J. Soroka, N. Wei, J.L. Boyer, and M. Hochstrasser Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II Hepatology 48 2008 1558 1569
32. T. Kagawa, N. Watanabe, K. Mochizuki, A. Numari, Y. Ikeno, and J. Itoh Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells Am J Physiol Gastrointest Liver Physiol 294 2008 G58 G67
33. J.A. Byrne, S.S. Strautnieks, G. Ihrke, F. Pagani, A.S. Knisely, and K.J. Linton Missense mutations and single nuclotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing Hepatology 49 2009 553 567
34. C.W. Lam, K.M. Cheung, M.S. Tsui, M.S. Yan, C.Y. Lee, and S.F. Tong A patient with novel ABCB11 gene mutations with phenotypic transition between BRIC2 and PFIC2 J Hepatol 44 2006 240 242
35. A.S. Knisely, S.S. Strautnieks, Y. Meier, B. Stieger, J.A. Byrne, and B.C. Portmann Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency Hepatology 44 2006 478 486
36. A.O. Scheimann, S.S. Strautnieks, A.S. Knisely, J.A. Byrne, R.J. Thompson, and M.J. Finegold Mutations in bile salt export pump (ABCB11) in two children with progressive familial cholestasis and cholangiocarcinoma J Pediatr 150 2007 556 559
37. V. Keitel, M. Burdelski, U. Warskulat, T. Kühlkamp, D. Keppler, and D. Häussinger Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis Hepatology 41 2005 1160 1172
38. R.P. Oude Elferink, and C.C. Paulusma Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein) Pflugers Arch 453 2007 601 610
39. J.M. de Vree, E. Jacqemin, E. Sturm, D. Cresteil, P.J. Bosma, and J. Aten Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis Proc Natl Acad Sci U S A 95 1998 282 287
40. J.F. Deleuze, E. Jacquemin, C. Dubuisson, D. Cresteil, M. Dumont, and S. Erlinger Defect of multidrug-resistance 3 gene expression in a subtype of progressive familial intrahepatic cholestasis Hepatology 23 1996 904 908
41. D. Degiorgio, C. Colombo, M. Seia, L. Porcaro, L. Costantino, and L. Zazzeron Molecular characterization and structural implications of 25 new ABCB4 mutations in progressive familial intrahepatic cholestasis type 3 (PFIC3) Eur J Hum Genet 15 2007 1230 1238
42. J.L. Delaunay, A.M. Durand-Schneider, D. Delautier, A. Rada, J. Gautherot, and E. Jacquemin A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature Hepatology 49 2009 1218 1227
43. E. Gonzales, A. Davit-Spraul, C. Baussan, C. Buffet, M. Maurice, and E. Jacquemin Liver diseases related to MDR3 (ABCB4) gene deficiency Front Biosci 14 2009 4242 4256
44. J.F. Lucena, J.I. Herrero, J. Quiroga, B. Sangro, J. Garcia-Foncillas, and N. Zabalegui A multidrug resistance 3 gene mutation causing cholelithiasis, cholestasis of pregnancy, and adulthood biliary cirrhosis Gastroenterology 124 2003 1037 1042
45. G. Maggiore, O. Bernard, M. Hadchouel, A. Lemonnier, and D. Alagille Diagnostic value of serum gamma-glutamyl transpeptidase activity in liver diseases in children J Pediatr Gastroenterol Nutr 12 1991 21 26
46. A.C. Kurbegov, K.D. Setchell, J.E. Haas, G.W. Mierau, M. Narkewicz, and J.D. Bancroft Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile Gastroenterology 125 2003 1227 1234
47. S. Uegaki, A. Tanaka, Y. Mori, H. Kodama, T. Fukusato, and H. Takikawa Successful treatment with colestimide for a bout of cholestasis in a Japanese patient with benign recurrent intrahepatic cholestasis caused by ATP8B1 mutation Intern Med 47 2008 599 602
48. E.L. Cancado, R.M. Leitao, F.J. Carrilho, and A.A. Laudanna Unexpected clinical remission of cholestasis after rifampicin therapy in patients with normal or slightly increased levels of gamma-glutamyl transpeptidase Am J Gastroenterol 93 1998 1510 1517
49. E. Jacquemin, D. Hermans, A. Myara, D. Habes, D. Debray, and M. Hadchouel Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis Hepatology 25 1997 519 523
50. P. Fickert, M. Wagner, H.U. Marschall, A. Fuchsbichler, G. Zollner, and O. Tsybrovskyy 24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice Gastroenterology 130 2006 465 481
51. P.F. Whitington, and G.L. Whitington Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis Gastroenterology 95 1988 130 136
52. J. Bustorff-Silva, L. Sbraggia Neto, H. Olimpio, R.V. de Alcantara, E. Matsushima, and A.M. De Tommaso Partial internal biliary diversion through a cholecystojejunocolonic anastomosis - a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report J Pediatr Surg 42 2007 1337 1340
53. H. Ismail, P. Kalicinski, M. Markiewicz, I. Jankowska, J. Pawlowska, and P. Kluge Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion Pediatr Transplant 3 1999 219 224
54. J.C. Emond, and P.F. Whitington Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease) J Pediatr Surg 30 1995 1635 1641
55. S. Ekinci, I. Karnak, F. Gurakan, A. Yüce, M.E. Senocak, and F. Cahit Tanyel Partial external biliary diversion for the treatment of intractable pruritus in children with progressive familial intrahepatic cholestasis: report of two cases Surg Today 38 2008 726 730
56. H. Arnell, S. Bergdahl, N. Papadogiannakis, A. Nemeth, and B. Fischler 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
57. H. Yang, R.J. Porte, H.J. Verkade, Z.J. De Langen, and J.B. Hulscher Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease J Pediatr Gastroenterol Nutr 49 2009 216 221
58. C. Wanty, R. Joomye, N. van Hoorebeek, K. Paul, J.B. Otte, and R. Reding Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy Acta Gastroenterol Belg 67 2004 313 319
59. P.J. Kalicinski, H. Ismail, I. Jankowska, A. Kaminski, J. Pawlowska, and T. Drewniak Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass Eur J Pediatr Surg 13 2003 307 311
60. J.M. Stapelbroek, K.J. van Erpecum, L.W. Klomp, N.G. Venneman, T.P. Schwartz, and G.P. van Berge Henegouwen Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis Hepatology 43 2006 51 53
61. E. Torri, A. Lucianetti, D. Pinelli, V. Corno, M. Guizzetti, and G. Maldini Orthotopic liver transplantation for Byler's disease Transplant Proc 37 2005 1149 1150 (Pubitemid 40590819)
62. C. Englert, E. Grabhorn, A. Richter, X. Rogiers, M. Burdelski, and R. Ganschow Liver transplantation in children with progressive familial intrahepatic cholestasis Transplantation 84 2007 1361 1363
63. V. Keitel, M. Burdelski, Z. Vojnisek, L. Schmitt, D. Häussinger, and R. Kubitz De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: a novel mechanism of cholestasis Hepatology 50 2009 510 517
64. P. Jara, L. Hierro, P. Martinez-Fernandez, R. Alvarez-Doforno, F. Yánez, and M.C. Diaz Recurrence of bile salt export pump deficiency after liver transplantation N Engl J Med 361 2009 1359 1367
65. J.M. Stapelbroek, K.J. van Erpecum, L.W. Klomp, and R.H. Houwen Liver disease associated with canalicular transport defects: current and future therapies J Hepatol 52 2010 258 271
66. J.M. de Vree, R. Ottenhoff, P.J. Bosma, A.J. Smith, J. Aten, and R.P. Oude Elferink Correction of liver disease by hepatocyte transplantation in a mouse model of progressive familial intrahepatic cholestasis Gastroenterology 119 2000 1720 1730
67. G. Zollner, and M. Trauner Nuclear receptors as therapeutic targets in cholestatic liver diseases Br J Pharmacol 156 2009 7 27
68. E.M. Welch, E.R. Barton, J. Zhuo, Y. Tomizawa, W.J. Friesen, and P. Trifillis PTC124 targets genetic disorders caused by nonsense mutations Nature 447 2007 87 91
69. H. Hayashi, and Y. Sugiyama 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps Hepatology 45 2007 1506 1516
70. C. Williamson, L.M. Hems, D.G. Goulis, I. Walker, J. Chambers, and O. Donaldson Clinical outcome in a series of cases of obstetric cholestasis identified via a patient support group BJOG 111 2004 676 681
71. B. Stieger, K. Fattinger, J. Madon, G.A. Kullak-Ublick, and P.J. Meier Drug- and estrogen-induced cholestasis through inhibition of the hepatocellular bile salt export pump (Bsep) of rat liver Gastroenterology 118 2000 422 430
72. S. Abu-Hayyeh, P. Martinez-Becerra, S.H. Sheik Abdul Kadir, C. Selden, M.R. Romero, and M. Rees Inhibition of Na+-taurocholate co-transporting polypeptide mediated bile acid transport by cholestatic sulphated progesterone metabolites J Biol Chem 285 2010 16504 16512
73. E. Jacquemin, D. Cresteil, S. Manouvrier, O. Boute, and M. Hadchouel Heterozygous non-sense mutation of the MDR3 gene in familial intrahepatic cholestasis of pregnancy Lancet 353 1999 210 211
74. P.H. Dixon, N. Weerasekera, K.J. Linton, O. Donaldson, J. Chambers, and E. Egginton Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking Hum Mol Genet 9 2000 1209 1217
75. R. Mullenbach, K.J. Linton, S. Wiltshire, N. Weerasekera, J. Chambers, and E. Elias ABCB4 gene sequence variation in women with intrahepatic cholestasis of pregnancy J Med Genet 40 2003 e70
76. C. Pauli-Magnus, T. Lang, Y. Meier, T. Zidan-Marin, D. Jung, and C. Breymann Sequence analysis of bile salt export pump (ABCB11) and multi drug resistance p-glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy Parmacogenetics 14 2004 91 102
77. H.E. Wasmuth, A. Glantz, H. Keppeler, E. Simon, C. Bartz, and W. Rath Intrahepatic cholestasis of pregnancy: the severe form is associated with common variants of the hepatobiliary phospholipid transporter ABCB4 gene Gut 56 2007 265 270
78. A. Floreani, I. Carderi, D. Paternoster, G. Soard, F. Azzaroli, and W. Esposito Hepatobiliary phospholipid transporter ABCB4, MDR3 gene variants in a large cohort of Italian women with intrahepatic cholestasis of pregnancy Dig Liver Dis 40 2008 366 370
79. Y. Bacq, C. Gendrot, F. Perrotin, L. Lefrou, S. Chrétien, and V. Vie-Buret ABCB4 gene mutations and single-nucleotide polymorphisms in women with intrahepatic cholestasis of pregnancy J Med Genet 46 2009 711 715
80. R. Mullenbach, A. Bennett, N. Tetlow, N. Patel, G. Hamilton, and F. Cheng ATP8B1 mutations in British cases with intrahepatic cholestasis of pregnancy Gut 54 2005 829 834
81. J.N. Painter, M. Savander, A. Ropponen, N. Nupponen, S. Riikonen, and O. Ylikorkala Sequence variation in the ATP8B1 gene and intrahepatic cholestasis of pregnancy Eur J Hum Genet 13 2005 435 439
82. Y. Meier, T. Zodan, C. Lang, R. Zimmermann, G.A. Kullak-Ublick, and P.J. Meier Increased susceptibility for intrahepatic cholestasis of pregnancy and contraceptive-induced cholestasis in carriers of the 1331T > C polymorphism in the bile salt export pump World J Gastroenterol 14 2008 38 45
83. P.H. Dixon, S.W. van Mil, J. Chambers, S. Strautnieks, R.J. Thompson, and F. Lammert Contribution of variant alleles of ABCB11 to susceptibility to intrahepatic cholestasis of pregnancy Gut 58 2009 537 544
84. S.W. van Mil, A. Milona, P.H. Dixon, R. Mullenbach, V. Geenes, and J. Chambers Functional variants of the bile acid sensor FXR identified in intrahepatic cholestasis of pregnancy Gastroenterology 133 2007 507 516
85. S. Sookoian, G. Castano, A. Burgueno, T.F. Gianotti, and C.J. Pirola Association of the multidrug-resistance-associated protein gene (ABCC2) variants with intrahepatic cholestasis of pregnancy J Hepatol 48 2008 125 132
86. W. Hardikar, S. Kansal, R.P. Oude Elferink, and P. Angus Intrahepatic cholestasis of pregnancy: when should you look further? World J Gastroenterol 15 2009 1126 1129
87. S.H. Sheikh Abdul Kadir, M. Miragoli, S. Abu-Hayyeh, A.V. Moshkov, Q. Xie, and V. Keitel Bile acid-induced arrhythmia is mediated by muscarinic M2 receptors in neonatal rat cardiomyocytes PLoS One 15 2010 e9689
88. Y. Bacq, T. Sapey, M.C. Brechot, F. Pierre, A. Fignon, and F. Dubois Intrahepatic cholestasis of pregnancy: a French prospective study Hepatology 26 1997 358 364
89. M.H. Davies, R.C. da Silva, S.R. Jones, J.B. Weaver, and E. Elias Fetal mortality associated with cholestasis of pregnancy and the potential benefit of therapy with ursodeoxycholic acid Gut 37 1995 580 584
90. A. Glantz, H.U. Marschall, and L.A. Mattson Intrahepatic cholestasis of pregnancy: relationships between bile acid levels and fetal complication rates Hepatology 40 2004 467 474
91. V. Geenes, and C. Williamson Intrahepatic cholestasis of pregnancy World J Gastroenterol 15 2009 2049 2066
92. A. Diaferia, P.L. Nicastri, M. Tartagni, P. Loizzi, C. Iacovizzi, and A. Di Leo Ursodeoxycholic acid therapy in pregnant women with cholestasis Int J Gynaecol Obstet 52 1996 133 140
93. J. Palma, H. Reyes, J. Ribalta, I. Hernández, L. Sandoval, and R. Almuna Ursodeoxycholic acid in the treatment of cholestasis of pregnancy: a randomized, double-blind study controlled with placebo J Hepatol 27 1997 1022 1028
94. R. Zapata, L. Sandoval, J. Palma, I. Hernández, J. Ribalta, and H. Reyes Ursodeoxycholic acid in the treatment of intrahepatic cholestasis of pregnancy. A 12-year experience Liver Int 25 2005 548 554
95. P.L. Nicastri, A. Diaferia, M. Tartagni, P. Loizzi, and M. Fanelli A randomised placebo-controlled trial of ursodeoxycholic acid and S-adenosylmethionine in the treatment of intrahepatic cholestasis of pregnancy Br J Obstet Gynaecol 105 1998 1205 1207
96. J.K. Mays The active management of intrahepatic cholestasis of pregnancy Curr Opin Obstet Gynecol 22 2010 100 103
97. A. Ropponen, R. Sund, S. Riikonen, O. Ylikorkala, and K. Aittomäki Intrahepatic cholestasis of pregnancy as an indicator of liver and biliary diseases: a population-based study Hepatology 43 2006 723 728