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Liver and biliary system

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Caroli disease


communicating, cavernous ectasia of the intrahepatic bile ducts

  • rare, autosomal recessive
  • usually detected in childhood or early adulthood

  • no cirrhosis or portal HTN

  • predisposed to calculus formation
  • recurrent cholangitis ==> liver abscesses ==> death
  • increased risk of cholangiocarcinoma

  • a/w medullary sponge kidney (renal tubular ectasia) in 80%


Charles E. Kahn, Jr., MD  -  2 February 1995
Last updated:  1 October 2013


 

Related CHORUS documents:
liver cyst
medullary sponge kidney
hepatoblastoma
Ehlers-Danlos syndrome
choledochal cyst
hypertrophic pyloric stenosis

 

Copyright © 2013, Charles E. Kahn, Jr.