Nephrology pp 1537-1543 | Cite as

Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease

  • Dominique Chauveau
  • Yves Pirson
  • Dominique Droz
  • Joseph Rosenfeld
  • Jean-Pierre Grünfeld


Among extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD), liver abnormalities are the most frequent and intracranial aneurysms, the most severe. Liver cysts develop later than renal cysts, and are more prevalent in females, most particularly in those who have had multiple pregnancies. The treatment of massive polycystic liver is difficult. In very rare families, congenital hepatic fibrosis may be associated with ADPKD. Rupture of an intracranial aneurysm (ICA) is a life-threatening complication. The mean age at rupture is 39 years. This accident may occur in normotensive patients with normal renal function. Routine screening is indicated when there is a positive family history of ICA or when there has been a previous accident of ICA rupture.


Abdominal Aortic Aneurysm Intracranial Aneurysm Autosomal Dominant Polycystic Kidney Disease Polycystic Kidney Disease Mitral Valve Prolapse 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Japan 1991

Authors and Affiliations

  • Dominique Chauveau
  • Yves Pirson
    • 2
  • Dominique Droz
  • Joseph Rosenfeld
    • 3
  • Jean-Pierre Grünfeld
    • 1
  1. 1.Département de NéphrologieHôpital NeckerParisFrance
  2. 2.Service de NéphrologieCliniques Universitaires Saint-LucBrusselsBelgium
  3. 3.Beilinson Medical CenterTel Aviv University Medical SchoolTel AvivIsrael

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