CHORUS
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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
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Copyright © 2013, Charles E. Kahn, Jr.