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Fig. 1. —7-month-old male infant with Caroli's syndrome (Caroli's disease accompanied by congenital hepatic fibrosis). Photomicrograph of histopathologic specimen shows portal bile duct surrounded by chronic inflammation. Lumen is dilated and contains soft, friable bilirubin calculi (asterisk). Fibrous portal expansion with bile ductules along septa (arrows) is consistent with congenital hepatic fibrosis. (H and E, x20)

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