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Caroli's disease
(redirected from Caroli disease)

   Also found in: Wikipedia 0.01 sec.
Caroli's disease
Hepatology A condition characterized by a diffuse distribution of intrahepatic biliary cysts and other cystic lesions–eg, medullary sponge kidneys Clinical Childhood onset–eg, jaundice, episodic fever, pain Complications Hepatic fibrosis, cirrhosis, portal HTN, esophageal bleeding, death in childhood


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5-8) More than 300 cases of Caroli disease and Caroli syndrome (Caroli disease associated with congenital hepatic fibrosis) have been reported in the world literature, (9) and cholangiocarcinoma has been found in 7% to 14% of the cases.
1) An association with congenital abnormalities of the biliary tree, such as a choledochal cyst or Caroli disease, and with polyposis cull and ulcerative colitis has previously been reported.
This leads to different congenital bile duct disorders, such as Caroli disease and syndrome, autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, congenital hepatic fibrosis, and bile duct hamartomas (BDHs, also known as von Meyenburg complexes).
 
 
 
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