Skip to main content
SpringerLink
Log in

Erkrankungen der Leber und der Gallenwege

  • Chapter
Cystische Fibrose

  • 153 Accesses

Der klinische Verlauf der cystischen Fibrose wird bei einer Vielzahl von Patienten durch eine Beteiligung der Leber und des extrahepatischen Gallengangssystems kompliziert [39,52,61]. Fettige Degeneration der Leber, intrahepatische Cholestase, fokal biliäre Zirrhose, multilobuläre biliäre Zirrhose, portale Hypertension, Gallenblasenatresie, Mikro-gallenblase, Gallenblasenhydrops, Cholelithiasis, Choledocholithiasis, Gallengangsstenosierungen, Cholangitiden, sklerosierende Cholangitis und Cholangiokarzinome wurden bei Patienten mit cystischer Fibrose in unterschiedlicher Häufigkeit beschrieben

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

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.

Literatur

  1. Andersen DH (1938) Cystic fibrosis of the pancreas and its relation to celiac disease. Am J Dis Child 56:344–399

    Google Scholar 

  2. Angelico M, Gandin C, Canuzzi P (1991) Gallstones in cystic fibrosis: a critical reappraisal. Hepatology 14:768–775

    Article  PubMed  CAS  Google Scholar 

  3. Balistreri WF (1997) Bile acid therapy in pediatric hepatobiliary disease: the role of ursodeoxycholic acid. JPGN 24: 573–589

    PubMed  CAS  Google Scholar 

  4. Bergasa NV, Jones EA (1995) The pruritus of cholestasis: potential pathogenic and therapeutic implications of opioids. Gastroenterology 108:1582

    Article  PubMed  CAS  Google Scholar 

  5. Bernard B, Lebrec D, Mathurin P, Opolon P, Poynard T (1997) Beta adrenergic antagonists in the prevention of gastrointestinal rebleeding in patients with cirrhosis: A meta-analysis. Hepatology 25:63–70

    Article  PubMed  CAS  Google Scholar 

  6. Besson I, Ingrand P, Person B, Boutroux D, Heresbach D, Bernard P, Hochhain P, Larricq J, Gourlaouen A, Ribard D, Kara MN, Legoux JL, Pillegand B, Becker MC, Costanzo J, Metreau JM, Silvain C, Beauchant M (1995) Sclerotherapy with or without octreotide for acute variceal bleeding. N Engl J Med 333:555–560

    Article  PubMed  CAS  Google Scholar 

  7. Beuers U, Boyer JL, Paumgartner G (1998) Ursodeoxycholic acid in cholestasis: potential mechanisms of action and therapeutic applications. Hepatology 28:1449–1453

    Article  PubMed  CAS  Google Scholar 

  8. Beuers U, Nathanson MH, Isales CM, Boyer JL (1993) Tauroursodeoxycholic acid stimulates hepatocellular exocytosis and mobilizes extracellular Ca mechanisms defective in cholestasis. J Clin Invest 92:2984–2993

    Article  PubMed  CAS  Google Scholar 

  9. Borg J, Warter JM, Schlienger JL et al. (1982) Neurotransmitter modifications in human cerebrospinal fluid and serum during hepatic encephalopathy. J Neurol Sci 57:343

    Article  PubMed  CAS  Google Scholar 

  10. Boyer TD, Henderson JM (1996) Portal hypertension and bleeding esophagela varices. In: Zakim D, Boyer TD (eds) Hepatology. W.B. Saunders, Philadelphia, pp 720–763

    Google Scholar 

  11. Calmus Y, Gane P, Rouger P, Poupon R (1990) Hepatic expression of class I and class II major histocompatibility complex molecules in primary biliary cirrhosis: effect of ursodeoxycholic acid. Hepatology 11:12–15

    Article  PubMed  CAS  Google Scholar 

  12. Carbera J, Maynar M, Granados R, Gorriz E, Reyes R, Pulido-Duque JM, San Roman JLR, Guerra C, Kravetz D (1996) Transjugular intrahepatic portosystemic shunt versus sclerotherapy in the elective treatment or variceal he-morrhage. Gastroenterology 110:832–839

    Article  Google Scholar 

  13. Charlton GPJ, Buchanan E, Holden CE, Preece MA, Green A, Booth IW, Tarlow MJ (1992) Intensive enteral feeding in advanced cirrhosis: reversal of malnutrition without precipitation of hepatic encephalopathy. Arch Dis Child 67: 603–607

    Article  PubMed  CAS  Google Scholar 

  14. Colombo C, Apostolo MG, Ferrari M, Seia M, Giunta A, Sereni LP (1994) Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 124:393–399

    Article  PubMed  CAS  Google Scholar 

  15. Colombo C, Battezzati PM, Podda M, Bettinardi N, Giunta A (1996) Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double blind multicenter trial. Hepatology 23:1484–1490

    Article  PubMed  CAS  Google Scholar 

  16. Colombo C, Bertolini E, Assaisso ML, Bettinardi N, Giunta A, Podda M (1993) Failure of ursodeoxycholic acid to dissolve radiolucent gallstones in patients with cystic fibrosis. Acta Paediatr 82:562–565

    Article  PubMed  CAS  Google Scholar 

  17. Cotting J, Lentze MJ, Reichen J (1990) Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. Gut 31:918–921

    Article  PubMed  CAS  Google Scholar 

  18. Cox KL, Freese DK (1996) Tacrolimus (FK506) the pros and cons of its use as an immunosuppressant in pediatric liver transplantation. Clin Invest Med 19:389–392

    PubMed  CAS  Google Scholar 

  19. Cynamon HA, Andres JM, Iafrate RP (1990) Rifampicin relieves pruritus in children with cholestatic liver disease. Gastroenterology 98:1013–1016

    Article  PubMed  CAS  Google Scholar 

  20. Dawson AM, McLaren J, Sherlock S (1957) Neomycin in the treatment of hepatic coma. Lancet 2:263–1268

    Google Scholar 

  21. Di Sant’ Agnese PA, Blanc WA (1956) A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas. Pediatrics 18:387–409

    Google Scholar 

  22. Duthie A, Doherty DG, Williams C et al. (1992) Genotype analysis for DF508, G551D, and R553X mutations in children and young adults with cystic fibrosis with and without chronic liver disease. Hepatology 15:660–664

    Article  PubMed  CAS  Google Scholar 

  23. Elkington SG, Floch MH, Conn HO (1969) Lactulose in the treatment of chronic portal-systemic encephalopathy. A Double-blind clinical trial. N Engl J Med 281:408–412

    Article  PubMed  CAS  Google Scholar 

  24. Eriksson LS, Person A, Wahren J (1982) Branched chain amino acids in the therapy of chronic hepatic encephalopathy. Gut 23:801–806

    Article  PubMed  CAS  Google Scholar 

  25. Faloon WW, Evans GL (1970) Precipitating factors in the genesis of hepatic coma. NY State J Med 70:2891–2896

    CAS  Google Scholar 

  26. Farber S (1944) Pancreatic function and disease in early life. Arch Pathol 37:238–250

    Google Scholar 

  27. Feu F, Garcia-Pagan JC, Bosch J, Luca A, Teres J, Escorsell A, Rodes J (1995) Relation between portal pressure response to pharmacotherapy and risk of recurrent variceal haemorhage in patients with cirrhosis. Lancet 346: 1056–1059

    Article  PubMed  CAS  Google Scholar 

  28. Floras JS, Legault L, Morah GA et al. (1991) Increased sym-pathetic outflow in cirrhosis and ascites: direct evidence from intraneural recordings. Ann Intern Med 114: 373–380

    Article  PubMed  CAS  Google Scholar 

  29. Forstner GG, Durie PR (1996) Cystic fibrosis. In: Walker AW, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB (eds) Pediatric gastrointestinal disease. Mosby, St. Louis, pp 1466–1487

    Google Scholar 

  30. Fox VL, Carr-Locke DL, Connors PJ, Leichtner AM (1995) Endoscopic ligation of esophageal varices in children. J Ped Gastroenterol Nutr 20:202–208

    Article  CAS  Google Scholar 

  31. Gaskin K (1994) The liver and biliary tract in cystic fibrosis. In: Suchy FJ (ed) Liver disease in children. Mosby, St. Louis, pp 705–719

    Google Scholar 

  32. Gaskin KJ, Waters DLM, Howman-Giles R et al. (1988) Liver disease and common bile duct Stenosis in cystic fibrosis. N Engl J Med 318:340–346

    Article  PubMed  CAS  Google Scholar 

  33. Gillespie DA, Vickens CR (1993) Pruritus and cholestasis: therapeutic options. J Gastroenterol Hepatol 8:168–173

    Article  PubMed  CAS  Google Scholar 

  34. Gyr K, Meier R (1991) Flumazenil in the treatment of porto-systemic encephalopathy — an overview. Intensive Care Med l7:S39–S42

    Article  Google Scholar 

  35. Hardy SC, Kleinman RE (1994) Cirrhosis and chronic liver failure. In: Suchy FJ (eds) Liver disease in children. Mosby, St. Louis, pp 214–248

    Google Scholar 

  36. Hayes PC, Davis JM, Lewis JA, Boucher IAD (1990) Meta-analysis of value of propanolol in prevention of variceal hemorrhage. Lancet 336:153–156

    Article  PubMed  CAS  Google Scholar 

  37. Hofmann AF (1990) Bile acid hepatotoxicity and the rationale for UDCA therapy in chronic cholestatic liver diseases: some hypotheses. In: Paumgartner G, Stiehl A, Barbara L, Roda E (eds) Strategies for the treatment of hepatobiliary diseases. Kluwer Academic, Dordrecht, pp 12–23

    Google Scholar 

  38. Imperial T, Teran JC, McCullough AJ (1995) A meta-analysis of somatostatin versus Vasopressin in the management of acute esophageal variceal hemorrhage. Gastroenterology 109:1289–1294

    Article  Google Scholar 

  39. Isenberg JN (1982) Cystic fibrosis: its influence on the liver, biliary tree and bile salt metabolism. Semin Liver Dis 2: 302–313

    Article  PubMed  CAS  Google Scholar 

  40. Isenberg JN, L’Heureux PR, Warwick WJ (1976) Clinical Observation on the biliary System in cystic fibrosis. Am J Gastroenterol 65:134–141

    PubMed  CAS  Google Scholar 

  41. Jalan R, Hayes PC (1997) Hepatic encephalopathy and ascites. Lancet 350:1309–1315

    Article  PubMed  CAS  Google Scholar 

  42. Johnston DE, Kaplan MM (1993) Pathogenesis and treatment of gallstones. N Engl J Med 328:412–421

    Article  PubMed  CAS  Google Scholar 

  43. Knudsen GM, Schmid J, Almdal T et al. (1993) Passage of amino acids and glucose accross the blood-brain barrier in patients with hepatic encephalopathy. Hepatology 17:987

    Article  PubMed  CAS  Google Scholar 

  44. Lang T (1997) Gallensteine bei Kindern und Jugendlichen. Monatsschr Kinderheilkd 145:1102–1114

    Article  Google Scholar 

  45. Lindblad A, Glaumann H, Strandvik B (1998) 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 27:166–174

    Article  PubMed  CAS  Google Scholar 

  46. Lykavieris P, Bernard O, Hadchouel M (1996) Neonatal cholestasis as the presenting feature in cystic fibrosis. Arch Dis Child 75:67–70

    Article  PubMed  CAS  Google Scholar 

  47. Mack DR, Traystman MD, Colombo JL, Sammut PH, Kaufman SS, Vanderhoof JA, Antonson DL, Markin RS, Shaw BW, Langnas AN (1995) Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis. J Pediatr 127:881–887

    Article  PubMed  CAS  Google Scholar 

  48. Morgan MY, Hawley KE (1987) Lactitol vs lactulose in the treatment of acute hepatic encephalopathy in cirrhotic patients: a double-blind randomized trial. Hepatology 7: 1278–1284

    Article  PubMed  CAS  Google Scholar 

  49. Noble-Jamieson G, Barnes N, Jamieson N, Friend P, Calne R (1996) Liver transplantation for hepatic cirrhosis in cystic fibrosis. J R Soc Med 89:31–37

    PubMed  Google Scholar 

  50. Noble-Jamieson G, Valente J, Barnes ND et al. (1994) Liver transplantation for hepatic cirrhosis in cystic fibrosis. Arch Dis Child 71:349–352

    Article  PubMed  CAS  Google Scholar 

  51. Oppenheimer EH, Esterley JR (1975) Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. J Pediatr 86:683–689

    Article  PubMed  CAS  Google Scholar 

  52. Park RW, Grand RJ (1981) Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 81: 1143–1161

    PubMed  CAS  Google Scholar 

  53. Patrick MK, Howman-Giles R, de Silva M et al. (1986) Common bile duct obstruction causing right Upper quadrant pain in cystic fibrosis. J Pediatr 108:101–102

    Article  PubMed  CAS  Google Scholar 

  54. Pratschke J, Steinmüller T, Bechstein WO, Neuhaus R, Tullius SG, Jonas S, Schumacher G, Luck W, Becker M, Neuhaus P (1998) Orthotopic liver transplantation for hepatic associated metabolic disorders. Clin Transplant 12: 228–232

    PubMed  CAS  Google Scholar 

  55. Price MR, Sartorelli KH, Karrer FM, Narkewicz MR, Sokol RJ, Lilly JR (1996) Management of esophageal varices in children by endoscopic variceal ligation. J Pediatr Surg 31: 1056–1059

    Article  PubMed  CAS  Google Scholar 

  56. Psacharopoulos HAT, Portmann B, Howard ER et al. (1981) Hepatic complications of cystic fibrosis. Lancet 2:78–80

    Article  PubMed  CAS  Google Scholar 

  57. Reif S, Sloven DG, Lebenthal E (1991) Gallstones in children. AJDC 145:105–108

    PubMed  CAS  Google Scholar 

  58. Rescorla FJ (1997) Cholelithiasis, Cholecystitis, and common bile duct stones. Curr Opin Pediatr 9:276–282

    Article  PubMed  CAS  Google Scholar 

  59. Roberts WC (1962) The hepatic cirrhosis of cystic fibrosis of the pancreas. Am J Med 32:324–328

    Article  PubMed  CAS  Google Scholar 

  60. Rossle M, Haag K, Ochs A, Sellinger M, Noldge G, Perarnau JM (1994) The transjugular intrahepatic portosystemic stent-shunt procedure for variceal bleeding. N Engl J Med 330:165–171

    Article  PubMed  CAS  Google Scholar 

  61. Roy CC, Weber AM, Morin CL et al. (1977) Abnormal biliary lipid composition in cystic fibrosis: effect of pancreatic enzymes. N Engl J Med 297:1301–1305

    Article  PubMed  CAS  Google Scholar 

  62. Roy CC, Weber AM, Morin CL, et al. (1982) Hepatobiliary disease in cystic fibrosis: a survey of current issues and concepts. J Pediatr Gastroenterol Nutr 1:469–478

    Article  PubMed  CAS  Google Scholar 

  63. Schrier RW (1988) Pathogenesis of sodium and water retention in high Output and low Output cardiac failure, nephrotic sydrome, cirrhosis, and pregnancy, parts I-II. N Engl J Med 319:1065–1072, 1127–1134

    Article  PubMed  CAS  Google Scholar 

  64. Scott-Jupp R, Lama M, Tanner MS (1991) Prevalence of liver disease in cystic fibrosis. Arch Dis Child 66:698–701

    Article  PubMed  CAS  Google Scholar 

  65. Sharp HL (1995) Cystic fibrosis liver disease and transplantation. J Pediatr 127:944–946

    Article  PubMed  CAS  Google Scholar 

  66. Shneider BL, Groszmann RJ (1994) Portal hypertension. In: Suchy FJ (ed) Liver disease in children. Mosby, St. Louis, pp 249–266

    Google Scholar 

  67. Sokol JR (1990) Medical management of neonatal cholestasis. In: Balistreri WF, Stocker JT (eds) Pediatric hepatology. Hemisphere Publishing, pp 48–49

    Google Scholar 

  68. Sokol JR, Durie PR (1999) Recommendations for management of liver and biliary tract disease in cystic fibrosis. J Pediatr Gastroenterol Nutr 28:S1–S13

    Article  PubMed  Google Scholar 

  69. Stanley AJ, Hayes PC (1997) Portal hypertension and variceal haemorrhage. Lancet 350:1235–1239

    Article  PubMed  CAS  Google Scholar 

  70. Stern RC, Stevens DP, Boat TF et al. (1976) Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic shunting. Gastroenterology 70:645–649

    PubMed  CAS  Google Scholar 

  71. Strandvik B, Lultcramtz R (1994) Liver function and morphology during long term fatty acid supplementation in cystic fibrosis. Liver 14:32–36

    PubMed  CAS  Google Scholar 

  72. Strauss RM, Boyer TD (1996) Diagnosis and management of cirrhotic ascites. In: Zakim D, Boyer TD (eds) Hepatology. W.B. Saunders, Philadelphia, pp 764–788

    Google Scholar 

  73. Tanner MS (1986) Current clinical management of hepatic Problems in cystic fibrosis. J R Soc Med 79:38–43

    PubMed  Google Scholar 

  74. Teran JC, Imperiale TF, Mullen KD, Tavill AS, McCullough S (1997) Primary Prophylaxis of variceal bleeding in cirrhosis: A cost-effectiveness analysis. Gastroenterology 112:473–482

    Article  PubMed  CAS  Google Scholar 

  75. Teres J, Bosch J, Bordas J, Garcia-Pagan JC, Feu F, Cirera I, Rodes J (1993) Propanolol versus sclerotherapy in preventing variceal rebleeding: a randomized controlled trial. Gastroenterology 105:1508–1514

    Article  PubMed  CAS  Google Scholar 

  76. The US Multicenter FK506 Liver Study Group (1994) A comparison of tracrolimus (FK506) and cyclosporine for immunosuppression in liver transplantation. N Engl J Med 331:1110–1115

    Article  Google Scholar 

  77. Thornton JR, Losowky MS (1988) Opoid peptides and primary biliary cirrhosis. Br J Med 297:1501

    Article  CAS  Google Scholar 

  78. Treem WR, Stanley CA (1989) Massive hepatomegaly, steatosis and secundary plasma carnitine deficiency in an Infant with cystic fibrosis. Pediatrics 83:993–997

    PubMed  CAS  Google Scholar 

  79. Tyson KRT, Schuster SR, Shwachman HR (1968) Portal hypertension in cystic fibrosis. J Pediatr Surg 3:271–277

    Article  PubMed  CAS  Google Scholar 

  80. Valletta EA, Loreti S, Cipolli M, Cazzola G, Zanolla L (1993) Portal hypertension and esophageal varices in cystic fibrosis. Unreliability of echo-Doppler flowmetry. Scand J Gastroenterol 28:1042–1046

    Article  PubMed  CAS  Google Scholar 

  81. Vawter GF, Shwachman H (1979) Cystic fibrosis in adults: An autopsy study. Pathol Ann 14:357–382

    Google Scholar 

  82. Vlavianos P, Gimson AES, Westaby D, Williams R (1989) Balloon tamponade for the management of variceal bleeding: uses and misuses. Br Med J 298:1158

    Article  CAS  Google Scholar 

  83. Walker CO, Schenker S (1970) Pathogenesis of hepatic encephalopathy with specific reference to the role of ammonia. Am J Clin Nutr 23:619–632

    PubMed  CAS  Google Scholar 

  84. Warwick WJ, L’Heureux PR, Sharp HL (1976) Gallstones and cystic fibrosis. In: Proceedings of the Seventh International Cystic Fibrosis Congress, Paris, pp 484–491

    Google Scholar 

  85. Westaby D, Hayes P, Gimson A et al. (1989) Controlled clinical trial of injection sclerotherapy for active variceal bleeding. Hepatology 9:276–277

    Article  Google Scholar 

  86. Williams SGJ, Westaby D (1995) Recent advances in the endoscopic management of variceal bleeding. Gut 36: 647–653

    Article  PubMed  CAS  Google Scholar 

  87. Willie UV, Reddish JM, Littlewood TR (1980) Cystic fibrosis: Its characteristic appearance on abdominal sonography. Am J Radiol 134:1005–1010

    Google Scholar 

  88. Wilroy RS, Crawford SE, Johnson WW (1966) Cystic fibrosis with extensive fat replacement of the liver. J Pediatr 68: 67–73

    Article  PubMed  Google Scholar 

  89. Wong F, Blendis L (1993) Ascites and portal-systemic encephalopathy as complications of cirrhosis. Curr Opin Gastroenterol 9:391

    Google Scholar 

Download references

Authors
  1. T. Lang
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Kinderklinik und Poliklinik im Dr. von Haunerschen Kinderspital der Ludwig-Maximilians-Universität, Lindwurmstr. 4, 80337, München, Deutschland

    Dietrich Reinhardt

  2. Abteilung für Kinder- und Jugendheilkunde, Wilhelminenspital der Stadt Wien, Montlearstr. 37, 1171, Wien, Österreich

    Manfred Götz

  3. Inselspital, Medizinische Universitäts-Kinderklinik, Freiburgstr. 23, 3010, Bern, Schweiz

    Richard Kraemer  & Martin H. Schöni  & 

Rights and permissions

Reprints and permissions

Copyright information

© 2001 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Lang, T. (2001). Erkrankungen der Leber und der Gallenwege. In: Reinhardt, D., Götz, M., Kraemer, R., Schöni, M.H. (eds) Cystische Fibrose. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-56796-4_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-56796-4_10

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-63172-6

  • Online ISBN: 978-3-642-56796-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation