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Histopathology and molecular pathology confirmed a diagnosis of atypical Caroli’s syndrome: a case report - Diagnostic Pathology
Caroli’s syndrome (CS) is a rare congenital disease characterized by non-obstructive segmental saccular dilation of intrahepatic bile ducts [17]. It is often accompanied by congenital hepatic fibrosis (CHF), which presents as fibrosis in the portal tracts. In terms of pathogenesis, CS belongs to a disorder called ductal plate malformation (DPM), a congenital disorder caused by abnormal development of the embryonic biliary [18,19,20].
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