Skip to main content
  • 3302 Accesses

Abstract

Cholestasis is defined as a disorder of cholepoiesis and bile secretion as well as a mechanical or functional stoppage of the bile flow in intrahepatic or extrahepatic bile ducts — with bile components passing into the blood. • Cholestasis can occur both with and without jaundice.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  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

    PubMed  CAS  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

    Article  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

    Article  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

    PubMed  CAS  Google Scholar 

  7. Chand, N, Sanyal, A.J.: Sepsis-induced cholestasis (review). Hepatology 2007; 45: 230–241

    Article  PubMed  CAS  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

    PubMed  CAS  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

    PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Article  PubMed  CAS  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.: Drug-induced prolonged cholestasis in adults: a histological semiquantitative study demonstrating progressive ductopenia. Hepatology 1992; 15: 244–251

    Article  PubMed  CAS  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

    Article  PubMed  CAS  Google Scholar 

  14. Drivdal, M., Trydal, T., Hagve, T.A., Bergstad, I., Aagenaes, O.: Prognosis with evaluation of general biochemistry of liver disease in lymphoedema cholestasis syndrome 1 (LCS 1/Aagenaes syndrome). Scand. J. Gastroenterol. 2006; 41: 465–471

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  16. 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

    Article  PubMed  CAS  Google Scholar 

  17. 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

    Article  PubMed  CAS  Google Scholar 

  18. 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

    Article  PubMed  Google Scholar 

  19. 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

    PubMed  CAS  Google Scholar 

  20. Glantz, A., Marschall, H.W., Mattsson, L.A.: Intrahepatic cholestasis of pregnancy: Relationship between bile acid levels and fetal complication rates. Hepatology 2004; 40: 467–474

    Article  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

    PubMed  CAS  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

    Article  Google Scholar 

  23. Harris, M.J., le Couteur, D.G., Arias, I.M.: Progressive familial intrahepatic cholestasis: Genetic disorders of biliary transporters (review). J. Gastroenterol. Hepatol. 2005; 20: 807–817

    PubMed  CAS  Google Scholar 

  24. 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 

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

    PubMed  CAS  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

    Article  PubMed  Google Scholar 

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

    Article  Google Scholar 

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

    Article  PubMed  CAS  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

    PubMed  CAS  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

    PubMed  CAS  Google Scholar 

  32. Kondrackiene, J., Beuers, U., Kupcinskas, L.: Efficacy and safety of ursodeoxycholic acid versus cholestyramine in ultrahepatic cholestasis of pregnancy. Gastroenterology 2005; 129: 894–901

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  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

    Article  PubMed  CAS  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

    Article  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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  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

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  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

    PubMed  CAS  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

    Article  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

    Article  PubMed  CAS  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

    Article  PubMed  CAS  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

    Article  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

    Article  Google Scholar 

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

    Article  PubMed  CAS  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

    Article  PubMed  CAS  Google Scholar 

  59. 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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  63. 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

    Article  Google Scholar 

  64. Sevastos, N., Savvas, S.P., Rafailidis, P.I., Manesis, E.K.: Cholestasis in acute stroke: An investigation on its prevalence and etiology. Scand. J. Gastroenterol. 2005; 40: 862–866

    Article  PubMed  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

    Article  PubMed  CAS  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

    PubMed  CAS  Google Scholar 

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

    Article  Google Scholar 

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

    Article  PubMed  CAS  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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  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

    Article  PubMed  CAS  Google Scholar 

  74. Vaishali, M.D., Agarwal, A.K., Upadhyaya, D.N., Chauhan, V.S., Sharma, O.P., Shukla, V.K.: Magnetic resonance cholangiopancreatography in obstructive jaundice. J. Clin. Gastroenterol. 2004; 38: 887–890

    Article  PubMed  CAS  Google Scholar 

  75. 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

    PubMed  CAS  Google Scholar 

  76. 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

    Article  CAS  Google Scholar 

  77. 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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  79. 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

    Article  PubMed  CAS  Google Scholar 

  80. Zapata, R., Sandoval, L., Palma, J., Hernandez, I., Ribalta, J., Reyes, H., Sedano, M., Toha, D., Silva, J.J.: Ursookoxycholic acid in the treatment of intrahepatic cholestasis of pregnancy. A 12-year experience. Liver Int. 2005; 25: 548–554

    Article  PubMed  CAS  Google Scholar 

  81. 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 

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

    CAS  Google Scholar 

Download references

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Medizin Verlag Heidelberg

About this chapter

Cite this chapter

(2008). Cholestasis. In: Hepatology Textbook and Atlas. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76839-5_13

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-76839-5_13

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-76838-8

  • Online ISBN: 978-3-540-76839-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics