Caroli disease

(redirected from Caroli's syndrome)

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.
Farlex Partner Medical Dictionary © Farlex 2012

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.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

Caroli,

Jacques, French gastroenterologist, 1902-1979.
Caroli disease - congenital cystic dilation of the intrahepatic bile ducts.
Medical Eponyms © Farlex 2012
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References in periodicals archive ?
Bayraktar, "Clinical characteristics of Caroli's syndrome," World Journal of Gastroenterology, vol.
But in CHF, the smaller and peripheral bile ducts are involved.19 We had two patients diagnosed as Caroli's syndrome on the basis of CT scan and both were excluded from the study as we included only biopsy-proven patients of CHF.
Caroli's syndrome which has autosomal recessive or dominant inheritance occurs more frequently than in Caroli's disease.
Estimates suggest that 25% to 50% of cases could be autosomal recessive, the balance being sporadic, although no clear genetic basis for the disease has been identified.[sup.5] Caroli's disease is characterized by segmental dilatation of the intrahepatic bile ducts without hepatic fibrosis as compared to Caroli's syndrome, which is defined as ectasia of the intrahepatic bile ducts with hepatic fibrosis.[sup.6,7] Clinically, patients with Caroli's disease often develop portal hypertension, fat-soluble vitamin deficiency (vitamin K in particular) and are at increased risk for intrahepatic stones and severe septic cholangitis.
Caroli's syndrome and adult polycystic kidney disease.
Thi s can take several forms: (1) cysts in the main trunk (choledochal cysts), (2) cysts (or lakes) in the small branches within the liver (Caroli's Syndrome) or (3) cysts in the liver separate from the biliary tree (polycystic liver disease).