Caroli disease


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Ca·ro·li dis·ease

(kah-rō'lē), [MIM*263200]
congenital cystic dilation of the intrahepatic bile ducts, sometimes associated with intrahepatic stones and biliary obstruction; may be a part of the phenotype of infantile polycystic kidney disease.

Ca·ro·li dis·ease

, Caroli syndrome (kah-rō-lē' di-zēz', sin'drōm)
Congenital cystic dilation of the intrahepatic bile ducts, sometimes associated with intrahepatic stones and biliary obstruction.

Caroli,

Jacques, French gastroenterologist, 1902-1979.
Caroli disease - congenital cystic dilation of the intrahepatic bile ducts.
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References in periodicals archive ?
The Middlesbrough youngster suffers from a variety of conditions including portal hypertension, congenital hepatic fibrosis, polycystic kidney and Caroli disease.
Liver involvement is a result of congenital hepatic fibrosis or Caroli disease, which may progress to portal hypertension, hypersplenism with resultant thrombocytopenia and recurrent ascending cholangitis (Caroli syndrome).
Of the fibropolycystic liver diseases, patients with congenital hepatic fibrosis and Caroli disease often develop recurrent acute cholangitis and portal hypertension secondary to peribiliary fibrosis (Figure 1).
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.
Last year, Marley Gill was diagnosed with portal hypertension, congenital hepatic fibrosis, polycystic kidney disease and Caroli disease - serious genetic conditions affecting his liver and kidneys.
Caroli disease represents the Type V Todani choledochal cyst; communicating cavernous ectasia of the IBDs.
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.
In early 2013 Marley, from Middlesbrough, was diagnosed with congenital hepatic fibrosis, polycystic kidney disease and Caroli disease, a genetic conditions affecting his liver and kidneys.
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).
At the beginning of last year Marley, from Middlesbrough, was diagnosed with congenital hepatic fibrosis, polycystic kidney disease and Caroli disease - genetic conditions affecting his liver and kidneys.
Marley has congenital hepatic fibrosis, polycystic kidney disease and Caroli disease - genetic conditions affecting his liver and kidneys.
Four-year-old Marley Gill, also from Middlesbrough, must undergo surgery every eight weeks as he suffers with congenital hepatic fibrosis, polycystic kidney disease and Caroli disease - genetic conditions which affect his liver and kidneys.