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Vascular Complications of Fibrocystic Liver Disease

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Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

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Summary

Vascular complications of fibrocystic liver disease can involve the hepatic or the extrahepatic vasculature. The hepatic vascular complications of fibrocystic liver disease involve the venous structures of the liver. Portal hypertension is common in congenital hepatic fibrosis, often manifesting as variceal bleeding. In autosomal dominant polycystic liver disease, enlarging hepatic cysts can obstruct the hepatic veins or the inferior vena cava, or both, resulting in the corresponding venous obstruction syndrome. Extrahepatic vascular complications of fibrocystic liver disease are related to abnormalities at the level of the arterial elastic lamina associated with aneurysm formation, most commonly at the level of the intracranial vessels. Rupture of intracranial aneurysms results in the devastating complication of subarachnoid hemorrhage. Autosomal dominant polycystic liver disease is also associated with cardiac valvular anomalies.

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References

  1. Qian Q, Li A, King BF, Kamath PS, Lager DJ et al. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 2003;37(1), 164–171.

    Article  PubMed  Google Scholar 

  2. Everson GT, Taylor MR, Doctor RB. Polycystic disease of the liver. Hepatology 2004;40(4), 774–782.

    CAS  PubMed  Google Scholar 

  3. Griffin MD, Torres VE, Grande JP, Kumar R. Vascular expression of polycystin. J Am Soc Nephrol 1997;8, 616–626.

    CAS  PubMed  Google Scholar 

  4. Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol 2007;37, 920–924.

    Article  PubMed  Google Scholar 

  5. Bayraktar Y, Balkanci F, Kayhan B et al. Congenital hepatic fibrosis associated with cavernous transformation of the portal vein. Hepatogastroenterology 1997;44, 1588–1594.

    CAS  PubMed  Google Scholar 

  6. Bergmann C, Senderek J, Windelen E et al. Clinical consequences of PKHD1 mutations in 164 patients with autosomal-recessive polycystic kidney disease (ARPKD). Kidney Int 2005;67, 829–848.

    Article  CAS  PubMed  Google Scholar 

  7. Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: The clinical experience in North America. Pediatrics 2003;111, 1072–1080.

    Article  PubMed  Google Scholar 

  8. Capisonda R, Phan V, Traubuci J et al. Autosomal recessive polycystic kidney disease: Outcomes from a single-center experience. Pediatr Nephrol 2003;18, 119–126.

    PubMed  Google Scholar 

  9. Zeitoun D, Brancatelli G, Colombat M et al. Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 2004;231, 109–116.

    Article  PubMed  Google Scholar 

  10. Nagral A, Nabi F, Humar A et al. Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation. Indian J Gastroenterol 2007;26, 88–89.

    PubMed  Google Scholar 

  11. Khuroo MS, Khuroo NS, Farahat KL et al. Meta-analysis: Endoscopic variceal ligation for primary prophylaxis of oesophageal variceal bleeding. Aliment Pharmacol Ther 2005;21, 347–361.

    Article  CAS  PubMed  Google Scholar 

  12. Garcia-Pagan JC, Bosch J. Endoscopic band ligation in the treatment of portal hypertension. Nat Clin Pract Gastroenterol Hepatol 2005;2, 526–535.

    Article  PubMed  Google Scholar 

  13. Bernard B, Grange JD, Khac EN et al. Antibiotic prophylaxis for the prevention of bacterial infections in cirrhotic patients with gastrointestinal bleeding: A meta-analysis. Hepatology 1999;29, 1655–1661.

    Article  CAS  PubMed  Google Scholar 

  14. Hou MC, Lin HC, Liu TT et al. Antibiotic prophylaxis after endoscopic therapy prevents rebleeding in acute variceal hemorrhage: A randomized trial. Hepatology 2004;39, 746–753.

    Article  CAS  PubMed  Google Scholar 

  15. Fernandez J, Ruiz del Arbol L, Gomez C et al. Norfloxacin vs ceftriaxone in the prophylaxis of infections in patients with advanced cirrhosis and hemorrhage. Gastroenterology 2006;131, 1049–1056.

    Article  CAS  PubMed  Google Scholar 

  16. Gonzalez R, Zamora J, Gomez-Camarero J et al. Meta-analysis: Combination endoscopic and drug therapy to prevent variceal rebleeding in cirrhosis. Ann Intern Med 2008;149, 109–122.

    PubMed  Google Scholar 

  17. Ryckman FC, Alonso MH. Causes and management of portal hypertension in the pediatric population. Clin Liver Dis 2001;5, 789–818.

    Article  CAS  PubMed  Google Scholar 

  18. Torres VE, Rastogi S, King BF et al. Hepatic venous outflow obstruction in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1994;5, 186–1192.

    Google Scholar 

  19. Grams J, Teh SH, Torres VE et al. Inferior vena cava stenting: A safe and effective treatment for intractable ascites in patients with polycystic liver disease. J Gastrointest Surg 2007;11, 985–990.

    Article  PubMed  Google Scholar 

  20. Torres VE, Cai Y, Chen X et al. Vascular expression of polycystin-2. J Am Soc Nephrol 2001;12, 1–9.

    CAS  PubMed  Google Scholar 

  21. Grantham JJ. Autosomal dominant polycystic kidney disease. N Engl J Med 2008;359, 1477–1485.

    Article  CAS  PubMed  Google Scholar 

  22. Rinkel GJE. Intracranial aneurysm screening: Indications and advice for practice. Lancet Neurol 2005;4, 122–128.

    Article  PubMed  Google Scholar 

  23. Rinkel GJ, Djibuti M, Algra A, van Gijn J. Prevalence and risk of rupture of intracranial aneurysms: A systematic review. Stroke 1998;29, 251–256.

    CAS  PubMed  Google Scholar 

  24. International Study of Unruptured Intracranial Aneurysms Investigators. Unruptured intracranial aneurysms – risk of rupture and risks of surgical intervention. N Engl J Med 1998;339, 1725–1733.

    Article  Google Scholar 

  25. Hughes PDV, Becker GJ. Screening for intracranial aneurysms in autosomal dominant polycystic kidney disease. Nephrology 2003;8, 163–170.

    Article  PubMed  Google Scholar 

  26. Schievink WI, Torres VE, Wiebers DO, Huston JIII. Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1997;8, 1298–1303.

    CAS  PubMed  Google Scholar 

  27. Lee CC, Chang WT, Fang CC, Tsai IL, Chen WJ. Sudden death caused by dissecting thoracic aortic aneurysm in a patient with autosomal dominant polycystic kidney disease. Resuscitation 2004;63, 93–96.

    Article  PubMed  Google Scholar 

  28. Kato A, Takita T, Furuhashi M et al. Abdominal aortic aneurysms in hemodialysis patients with autosomal dominant polycystic kidney disease. Nephron 2001;88, 185–186.

    Article  CAS  PubMed  Google Scholar 

  29. Griffin MD, Torres VE, Grande JP, Kumar R. Immunolocalization of polycystin in human tissues and cultured cells. Proc Assoc Am Physicians 1996;108, 185–197.

    CAS  PubMed  Google Scholar 

  30. Lumiaho A, Iheimo R, Miettinen R et al. Mitral valve prolapse and mitral regurgitation are common in patients with polycystic kidney disease type 1. Am J Kidney Dis 2001;38, 1208–1216.

    Article  CAS  PubMed  Google Scholar 

  31. Bardaji A, Vea AM, Gutierrez C et al. Left ventricular mass and diastolic function in normotensive young adults with autosomal dominant polycystic kidney disease. Am J Kidney Dis 1998;32, 970–975.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic, Rochester, MN, USA

    Maarouf Hoteit MD

  2. Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, Rochester, MN, USA

    Patrick S. Kamath MD

Authors
  1. Maarouf Hoteit MD
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  2. Patrick S. Kamath MD
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Editor information

Editors and Affiliations

  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

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Hoteit, M., Kamath, P.S. (2010). Vascular Complications of Fibrocystic Liver Disease. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_19

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  • DOI: https://doi.org/10.1007/978-1-60327-524-8_19

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  • Publisher Name: Humana Press

  • Print ISBN: 978-1-60327-523-1

  • Online ISBN: 978-1-60327-524-8

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