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Meta Description: Caroli’s disease is characterized by progressive cystic dilatation of the intrahepatic bile ducts, and belongs to an entity of fibropolycystic liver disease. It is often accompanied by congenital hepatic fibrosis.
Caroli’s disease is characterized by progressive cystic dilatation of the intrahepatic bile ducts, and belongs to an entity of fibropolycystic liver disease. It is often accompanied by congenital hepatic fibrosis (CHF), and is also known as a hepatic manifestation of autosomal recessive polycystic kidney disease. Autosomal recessive polycystic kidney disease has been estimated to have an incidence of 1 in 20,000 live births and a significant proportion of cases (up to 30%) die by the neonatal period, primarily of respiratory insufficiency.
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