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Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: A new association
Author(s) -
Goilav Beatrice,
Norton Karen I.,
Satlin Lisa M.,
GuayWoodford Lisa,
Chen Frank,
Magid Margret S.,
Emre Sukru,
Shneider Benjamin L.
Publication year - 2006
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2005.00456.x
Subject(s) - medicine , autosomal recessive polycystic kidney disease , gastroenterology , ectasia , intrahepatic bile ducts , bile duct diseases , congenital hepatic fibrosis , portal hypertension , bile duct , pathology , magnetic resonance cholangiopancreatography , autosomal dominant polycystic kidney disease , disease , endoscopic retrograde cholangiopancreatography , cirrhosis , pancreatitis
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.