Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: A new association

Beatrice Goilav, Karen I. Norton, Lisa M. Satlin, Lisa Guay-Woodford, Frank Chen, Margret S. Magid, Sukru Emre, Benjamin L. Shneider

Research output: Contribution to journalArticlepeer-review

20 Scopus citations


Autosomal recessive polycystic kidney disease (ARPKD) is characterized by dilation of ectatic renal collecting ducts, intrahepatic biliary dysgenesis, and portal fibrosis. Portal hypertension and recurrent bacterial cholangitis can dominate the clinical picture in long-term survivors. Predominant extrahepatic bile duct disease was revealed in four patients who underwent magnetic resonance cholangiopancreatography. All four patients had portal hypertension, although liver biochemistries did not suggest biliary disease. In two of the patients, cholangitis was clinically ascribed to the bile duct disease. Western blot analysis of plasma membranes from normal rat extrahepatic bile duct and kidney revealed the presence of polyductin as a single ∼440 kDa protein. Although the exact function of polyductin in the extrahepatic duct is unknown, it may have a role in the development and control of lumenal size. Clinical management of patients with ARPKD should include consideration of potential problems related to extrahepatic bile duct disease.

Original languageEnglish (US)
Pages (from-to)294-298
Number of pages5
JournalPediatric Transplantation
Issue number3
StatePublished - May 2006
Externally publishedYes


  • Bile duct
  • Kidney
  • Liver
  • Polycystic kidney
  • Transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation


Dive into the research topics of 'Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: A new association'. Together they form a unique fingerprint.

Cite this