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Hepatology Principles and Practice pp 227–242Cite as

Cholestasis

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Keywords

  • Bile Duct
  • Bile Acid
  • Ursodeoxycholic Acid
  • Choledochal Cyst
  • Intrahepatic Cholestasis

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References

  1. Aagenaes, O., van der Hagen, C.B., Refsum, S.: Hereditary recurrent intrahepatic cholestasis from birth. Arch. Dis. Childh. 1968; 43:646–657

    CAS  PubMed  Google Scholar 

  2. Aagenaes, O.: Hereditary recurrent cholestasis with lymphoedema. — Two new families. Acta Paed. Scand. 1974; 63: 465–471

    CAS  Google Scholar 

  3. Alonso, E.M., Snover, D.C., Montag, A., Freese, D.K., Whitington, P.F.: Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J. Pediatr. Gastroenterol. Nutr. 1994; 18: 128–133

    CAS  PubMed  Google Scholar 

  4. Arkenau, H.-T., Widjaja, A.: An unusual case of cholestasis and macrohematuria in a 52-year-old patient. Med. Klin. 2002; 97: 480–483

    CrossRef  Google Scholar 

  5. Bergasa, N.V., Alling, D.W., Talbot, T.L., Wells, M.C., Jones, E.A.: Oral nalmefene therapy reduces scratching activity due to the pruritus of cholestasis: a controlled study. J. Amer. Acad. Derm. 1999; 41: 431–434

    CAS  Google Scholar 

  6. Bowen, P., Lee, C.S.N., Zellweger, H., Lindenberg, R.: A familial syndrome of multiple congenital defects. Bull. Hopkins Hosp. 1964; 114:402–414

    CAS  PubMed  Google Scholar 

  7. Chitturi, S., George, J., Ranjitkumar, S., Kench, J., Benson, W.: Exchange transfusion for severe intrahepatic cholestasis associated with sickle cell disease? J. Clin. Gastroenterol. 2002; 35: 362–363

    CrossRef  PubMed  Google Scholar 

  8. Cissarek, T., Schumacher, B., Schwöbel, H., Sarbia, M., Neuhaus, H.: Verlauf einer benignen rekurrierenden intrahepatischen Cholestase (Summerskill-Walshe-Tygstrup-Syndrom) über 46 Jahre. Z. Gastroenterol. 1998; 36: 379–383

    CAS  PubMed  Google Scholar 

  9. Clayton, R.J., Iber, F.L., Ruebner, B.H., McKusick, V.M.: Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Amer. J. Dis. Child. 1969; 117: 112–124

    CAS  PubMed  Google Scholar 

  10. Clayton, P.T.: Inborn errors of bile acid metabolism. J. Inher. Metabol. Dis. 1991; 14: 478–496

    CAS  Google Scholar 

  11. Colombo, C., Okolicsanyi, L., Strazzabosco, M.: Advances in familial and congenital cholestatic diseases. Clinical and diagnostic implications. Dig. Liver Dis. 2000; 32: 152–159

    CrossRef  CAS  PubMed  Google Scholar 

  12. Degott, C., Feldmann, G., Larrey, D., Durand-Schneider, A.-M., Grange, D., Machayekhi, J.-P., Moreau, A, Potet, F., Benhamou, J.-P.: Druginduced prolonged cholestasis in adults: a histological semiquantitative study demonstrating progressive ductopenia. Hepatology 1992; 15:244–251

    CAS  PubMed  Google Scholar 

  13. Deleuze, J.-F., Jacquemin, E., Dubuisson, C., Cresteil, D., Dumont, M., Erlinger, S., Bernard, O., Hadchouel, M.: Defect of multidrug-resistance 3 gene expression in a subtype of progressive intrahepatic cholestasis. Hepatology 1996; 23: 904–908

    CAS  PubMed  Google Scholar 

  14. Dulk, den, M., Valentijn, R.M., Welten, C.A.H.C., Beyer, G.P.J.: Intrahepatic cholestasis of pregnancy (case report). Netherl. J. Med. 2002; 60: 366–369

    Google Scholar 

  15. Desmet, V.J.: Modulation of the liver in cholestasis. J. Gastroenterol. Hepatol. 1992; 7: 313–323

    CAS  PubMed  Google Scholar 

  16. De Vos, R., de Wolf-Peeters, C., Desmet, V., Eggermont, E., van Acker, K.: Progressive intrahepatic cholestasis (Byler’s disease): case report. Gut 1975; 16: 943–950

    PubMed  Google Scholar 

  17. Eppens, E.F., van Mil, S.W.C., de Vree, J.M.L., Mok, K.S., Juijn, J.A., Oude Elferink, R.P.J., Berger, R., Houwen, R.H.J., Klomp, L.W.J.: FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte. J. Hepatol. 2001; 35: 436–443

    CrossRef  CAS  PubMed  Google Scholar 

  18. Floreani, A., Guido, M., Bortolami, M., della Zentil, G., Venturi, C., Pennelli, N., Naccarato, R.: Relationship between apoptosis, tumour necrosis factor, and cell proliferation in chronic cholestasis. Dig. Liver Dis. 2001; 33: 570–575

    CrossRef  CAS  PubMed  Google Scholar 

  19. Forrest, E.H., Oien, K.A., Dickson, S., Galloway, D., Mills, P.R.: Improvement in cholestasis associated with total parenteral nutrition after treatment with an antibody against tumour necrosis factor alpha. Liver 2002; 22: 317–320

    PubMed  Google Scholar 

  20. Frezza, M., Surrenti, C., Manzillo, G., Fiaccadori, F., Bortolini, M., di Padova, C.: Oral S-adenosylmethionine in the symptomatic treatment of intrahepatic cholestasis. A double-blind, placebo-controlled study. Gastroenterology 1990; 99: 211–215

    CAS  PubMed  Google Scholar 

  21. Goez, H., Meiron, D., Horowitz, J., Schutgens, R.H., Wanders, R.J.A., Berant, M., Mandel, H.: Infantile Refsum disease: neonatal cholestatic jaundice. Presentation of a peroxismal disorder. J. Pediatr. Gastroenterol. Nutr. 1995; 20: 98–101

    CAS  PubMed  Google Scholar 

  22. Gores, G.J.: Mechanisms of cell injury and death in cholestasis and hepatoprotection by ursodeoxycholic acid. J. Hepatol. 2000; 32(Suppl. 2): 11–13

    Google Scholar 

  23. Herzog, D., Chessex, P., Martin, S., Alvarez, F.: Transient cholestasis in newborn infants with perinatal asphyxia. Canad. J. Gastroenterol. 2003;17: 179–182

    CAS  Google Scholar 

  24. Hofmann, A.F.: Cholestatic liver disease: Pathophysiology and therapeutic options. Liver 2002; 22(Suppl. 2): 14–19

    CAS  PubMed  Google Scholar 

  25. Hopf, U., Möller, B., Lobeck, H., Klein, R., Berg, P.A.: Immunserologische Differenzierung chronisch-cholestatischer Leberentzündungen. Dtsch. Med. Wschr. 1985; 110: 1924–1929

    CAS  PubMed  Google Scholar 

  26. Huster, D., Schubert, C., Achenbach, H., Caca, K., Moessner, J., Beer, F.: Successful clinical application of extracorporal albumine dialysis in a patient with benign recurrent intrahepatic cholestasis (BRIC). Z. Gastroenterol. 2001; 39(Suppl. 2): 13–14

    PubMed  Google Scholar 

  27. Jacquemin, E.: Progressive familial intrahepatic cholestasis. Genetic basis and treatment. Clin. Liver Dis. 2000; 47: 753–763

    Google Scholar 

  28. Jansen, P.L.M., Müller, M.: The molecular genetics of familial intrahepatic cholestasis. Gut 2000; 47: 1–5

    CrossRef  CAS  PubMed  Google Scholar 

  29. Jones, E.A., Neuberger, J., Bergasa, N.V.: Opiate antagonist therapy for the pruritus of cholestasis: the avoidance of opioid withdrawal-like reactions. Quart. J. Med. 2002; 95: 547–552

    CAS  Google Scholar 

  30. Juberg, R.C., Holland-Moritz, R.M., Henley, K.S., Gonzalez, C.F.: Familial intrahepatic cholestasis with mental and growth retardation. Pediatrics 1966; 38: 819–836

    CAS  PubMed  Google Scholar 

  31. Kaaden, R., Zwack, K., Wienbeck, M., Klotz, E.: Ikterische Cholestase als Frühsymptom bei Morbus Hodgkin. Dtsch. Med. Wschr. 1990;115: 1670–1673

    CAS  PubMed  Google Scholar 

  32. Kruiswijk, T., Holtkamp, H.C., Diaz, D., Seidel, B., Skakaly, M.: The relevance of some laboratory tests in the diagnosis of cholestasis and the differentiation between intra-and extrahepatic obstruction. Clin. Chim. Acta 1981; 112: 285–291

    CrossRef  CAS  PubMed  Google Scholar 

  33. Kullak-Ublick, G.A., Meier, P.J.: Mechanisms of cholestasis. Clin. Liver Dis. 2000; 4: 357–385

    CAS  PubMed  Google Scholar 

  34. Kumar, D., Tandon, R.K.: Fatigue in cholestatic liver disease — A perplexing symptom (review). Postgrad. Med. J. 2002; 78: 404–407

    CAS  PubMed  Google Scholar 

  35. Kuntz, H.-D.: Rezidivierende intrahepatische Cholestase. Münch. Med. Wschr. 1986; 128: 86–90

    Google Scholar 

  36. Lazarow, P.B., Black, V., Shio, H., Fujiki, Y., Hajra, A.K., Datta, N.S., Bangaru, B.S., Dancis, J.: Zellweger syndrome: biochemical and morphological studies on two patients treated with clofibrate. Pediatr. Res. 1985; 19: 1356–1364

    CAS  PubMed  Google Scholar 

  37. Linarelli, L.G., Williams, G.N., Philipps, M.J.: Byler’s disease: fatal intrahepatic cholestasis. J. Pediatr. 1972; 81: 484–492

    CAS  PubMed  Google Scholar 

  38. Lorette, G., Vaillant, I.: Pruritus. Current concepts in pathogenesis and treatment. Drugs 1990; 39: 218–223

    CAS  PubMed  Google Scholar 

  39. Lovisetto, P., Raviolo, P., Rizzetto, M., Marchi, L., Actis, G.C., Verme, G.: Benign recurrent intrahepatic cholestasis. A clinico-pathologic study. Res. Clin. Lab. 1990; 20: 19–27

    CAS  Google Scholar 

  40. Macia, M., Aviles, J., Navarro, J., Morales, S., Garcia, J.: Efficacy of molecular adsorbent recirculating system for the treatment of intractable pruritus in cholestasis. Amer. J. Med. 2003; 114: 62–64

    CAS  PubMed  Google Scholar 

  41. Maeda, K., Kimura, A., Yamato, Y., Nittono, H., Takei, H., Sato, T., Mitsubuchi, H., Murai, T., Kurosawa, T.: Oral bile acid treatment in two Japanese patients with Zellweger syndrome. J. Pediatr. Gastroenterol. Nutr. 2002; 35: 227–230

    CrossRef  PubMed  Google Scholar 

  42. Marzioni, M., Glaser, S.S., Francis, H., Phinizy, J.L., LeSage, G., Alpini, G.: Functional heterogeneisy of cholangiocytes. Semin. Liver Dis. 2002; 22: 227–240

    CrossRef  PubMed  Google Scholar 

  43. McDonald, J.A.: Cholestasis of pregnancy. J. Gastroenterol. Hepatol. 1999; 14: 515–518

    CrossRef  CAS  PubMed  Google Scholar 

  44. McGill, J.M., Kwiatkowski, A.P.: Cholestatic liver disease in adults. Amer. J. Gastroenterol. 1998; 93: 684–691

    CAS  Google Scholar 

  45. Messing, B., Colombel, J.F., Heresbach, D., Chazouillres, O., Galian, A.: Chronic cholestasis and macronutrient excess in patients treated with prolonged parenteral nutrition. Nutrition 1992; 8: 30–36

    CAS  PubMed  Google Scholar 

  46. Moseley, R.H.: Sepsis associated cholestasis. Gastroenterology 1997;112: 302–306

    CrossRef  CAS  PubMed  Google Scholar 

  47. Moss, R.L., Das, J.B., Raffensperges, J.G.: Total parenteral nutritionassociated cholestasis: clinical and histopathologic correlation. J. Pediatr. Surg. 1993; 28: 1270–1275

    CrossRef  CAS  PubMed  Google Scholar 

  48. Nagore, N., Howe, S., Boxer, L., Scheuer, P.J.: Liver cell rosettes: structural differences in cholestasis and hepatitis. Liver 1989; 9: 43–51

    CAS  PubMed  Google Scholar 

  49. Nakamuta, M., Sakamoto, S., Miyata, Y., Sato, M., Nawata, H.: Benign recurrent intrahepatic cholestasis: a long-term follow-up. Hepat. Gastroenterol. 1994; 41: 287–289

    CAS  Google Scholar 

  50. Neff, G.W., O’Brien, C.B., Reddy, K.R., Bergasa, N.V., Regev, A., Molina, E., Amaro, R., Rodriguez, M.J., Chase, V., Jeffers, L., Schiff, E.: Preliminary observation with dronabinol in patients with intractable pruritus secondary to cholestatic liver disease. Amer. J. Gastroenterol. 2002; 97: 2117–2119

    Google Scholar 

  51. Nishida, M., Tamakuma, S., Idei, T., Mochizuki, H.: A research on the cholestasis caused by continuous endotoxemia. J. Jap. Surg. Soc. 1990;91: 184–190

    CAS  Google Scholar 

  52. Nissenbaum, M., Chedid, A., Mendenhall, Ch., Gartside, P.: Prognostic significance of cholestatic alcoholic hepatitis. Dig. Dis. Sci. 1990; 35:891–896

    CrossRef  CAS  PubMed  Google Scholar 

  53. Paumgartner, G., Beuers, U.: Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 2002; 36: 525–531

    CrossRef  CAS  PubMed  Google Scholar 

  54. Paus, T.C., Schneider, G., van de Vondel, P., Sauerbruch, T., Reichel, C.: Diagnosis and therapy of intrahepatic cholestasis of pregnancy (review). Zschr. Gastroenterol. 2004; 42: 623–628

    CAS  Google Scholar 

  55. Puttermann, C., Keidar, S., Brook, J.G.: Benign recurrent intrahepatic cholestasis—25 years of follow-up. Postgrad. Med. J. 1987; 63: 295–296

    Google Scholar 

  56. Qureshi, W.A.: Intrahepatic cholestasic syndromes: pathogenesis, clinical features and management. Dig. Dis. 1999; 17: 49–59

    CrossRef  CAS  PubMed  Google Scholar 

  57. Refsum, S.: Heredoataxia hemeralopica polyneuritiformis — et tigligere ikke beskrevet familiaer syndrom? En forelöbig meddelelse. Nord. Med. 1945; 28: 2682–2686

    Google Scholar 

  58. Reyes, H.: Review: Intrahepatic cholestasis. A puzzling disorder of pregnancy. J. Gastroenterol. Hepatol. 1997; 12: 211–216

    CAS  PubMed  Google Scholar 

  59. Röllinghoff, W., Braun, H.J., Schad, F.J.: Intrahepatische Cholestase als Leitsymptom einer primären Amyloidose. Dtsch. Med. Wschr. 1976;101: 1838–1841

    PubMed  Google Scholar 

  60. Rubinow, A., Koff, R.S., Cohen, A.S.: Severe intrahepatic cholestasis in primary amyloidosis. A report of four cases and a review of the literature. Amer. J. Med. 1978; 64: 937–946

    CAS  PubMed  Google Scholar 

  61. Rudzki, C., Ishak, K.G., Zimmermann, H.J.: Chronic intrahepatic cholestasis of sarcoidosis. Amer. J. Med. 1975; 59: 373–387

    CAS  PubMed  Google Scholar 

  62. Schiraldi, O., Modugno, A., Miglietta, A., Fera, G.: Prolonged viral hepatitis type A with cholestasis: case report. Ital. J. Gastroenterol. 1991; 23: 364–366

    CAS  PubMed  Google Scholar 

  63. Schirrmacher, S., Blumenstein, I., Stein, J.: Pathogenesis and treatment of pruritus in patients with cholestasis (review). Zschr. Gastroenterol. 2003; 41: 259–262

    CAS  Google Scholar 

  64. Schworer, H., Hartmann, H., Ramadori, G.: Relief of cholestatic pruritus by a novel class of drugs: 5-hydroxytryptamine type 3 (5-HT3) receptor antagonist: effectiveness of ondansetron. Pain 1995; 61: 31–37

    CrossRef  Google Scholar 

  65. Socha, P., Koletzko, B., Jankowska, I., Pawlowska, J., Demmelmair, H., Stolarczyk, A., Swiatkowska, E., Socha, J.: Long-chain PUFA supplementation improves PUFA profile in infants with cholestasis. Lipids 2002; 37: 953–957

    CAS  PubMed  Google Scholar 

  66. Sturm, E., Franssen, C.F.M., Gouw, A., Staels, B., Boverhof, R., de Knegt, R.J., Stellaard, F., Bijleveld, C.M.A., Kuipers, F.: Extracorporal albumin dialysis (MARS) improves cholestasis and normalizes low apo A-I levels in a patient with benign recurrent intrahepatic cholestasis (BRIC). Liver 2002; 22(Suppl. 2), 72–75

    CAS  PubMed  Google Scholar 

  67. Summerskill, W.H.J., Walshe, J.M.: Benign recurrent intrahepatic “obstructive” jaundice. Lancet 1959/II: 686–690

    Google Scholar 

  68. Summerskill, W.H.J.: The syndrome of benign recurrent cholestasis. Amer. J. Med. 1965; 38: 298–305

    CAS  PubMed  Google Scholar 

  69. Terg, R., Coronel, E., Sorda, J., Munoz, A.E., Findor, J.: Efficacy and safety of oral naltrexone treatment for pruritus of cholestasis, a crossover, double blind, placebo-controlled study. J. Hepatol. 2002; 37:717–722

    CrossRef  CAS  PubMed  Google Scholar 

  70. Trauner, M., Fickert, P., Stauber, R.E.: Inflammatory-induced cholestasis. J. Gastroenterol. Hepatol. 1999; 14: 946–959

    CrossRef  CAS  PubMed  Google Scholar 

  71. Trauner, M., Boyer, J.L.: Cholestatic syndromes. Curr. Opin. Gastroenterol. 2003; 19: 216–231

    CrossRef  CAS  PubMed  Google Scholar 

  72. Tygstrup, N.: Intermittent possibly familial intrahepatic cholestatic jaundice. Lancet 1960/I: 1171–1172

    Google Scholar 

  73. Ujhazy, P., Ortiz, D., Misra, S., Li, S., Moseley, J., Jones, H., Arias, I.M.: Familiale intrahepatic cholestasis 1: studies of localization and function. Hepatology 2001; 34: 768–775

    CrossRef  CAS  PubMed  Google Scholar 

  74. Wang, L., Soroka, C.J., Boyer, J.L.: The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type II. J. Clin. Invest. 2002; 110: 965–972

    CAS  PubMed  Google Scholar 

  75. Westermann, G., Lügering, N., August, C., Rahn, K.H., Kisters, K.: A classic case of benign recurrent intrahepatic cholestasis (Summerskill-Walshe-Tygstrup syndrome). Med. Klin. 2000; 95: 349–354

    CAS  Google Scholar 

  76. Whitington, P.F., Freese, D.K., Alonso, E.M.: Clinical and biochemical findings in progressive familial intrahepatic cholestasis. J. Pediatr. Gastroenterol. Nutr. 1994; 18: 134–141

    CAS  PubMed  Google Scholar 

  77. Witzleben, C.L., Piccoli, D.A., Setchell, K.: Case 3.A new category of causes of intrahepatic cholestasis. Pediatr. Pathol. 1992; 12 269–274

    CAS  PubMed  Google Scholar 

  78. Yerushalmi, B., Sokol, R.J., Narkewicz, M.R., Smith, D., Karrer, F.M.: Use of rifampin for severe pruritus in children with chronic cholestasis. J. Pediatr. Gastroenterol. Nutr. 1999; 29: 442–447

    CAS  PubMed  Google Scholar 

  79. Zeijen, R.N.M., Sels, J.P.J.F., Flendrig, J.A., Arends, J.W.: Portal hypertension and intrahepatic cholestasis in hepatic amyloidosis. Netherl. J. Med. 1991; 38: 257–261

    CAS  Google Scholar 

  80. Zimniak, P., Radominska, A., Lester, R.: The pathogenesis of cholestasis. Hosp. Pract. 1990; 25: 107–125

    CAS  Google Scholar 

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(2006). Cholestasis. In: Hepatology Principles and Practice. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-28977-1_13

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