医学
先天性肝纤维化
门脉高压
肝内胆管
胃肠病学
胃肠道出血
胆管
内科学
罕见病
胆管疾病
疾病
肝硬化
作者
Julia del Río Izquierdo,Clara Ramos Belinchón,Rafael Bañares,Laura Carrión Martín
标识
DOI:10.17235/reed.2022.8813/2022
摘要
Caroli's disease is a congenital disease characterized by saccular or cystic dilatations of intrahepatic bile ducts. It is called Caroli's syndrome (CS) when intrahepatic dilations are associated with congenital hepatic fibrosis. CS is an infrequent cause of gastrointestinal bleeding in young people that should be considered when saccular dilatations of the bile duct are associated with portal hypertension (PHT). We present a rare case with gastrointestinal bleeding as first manifestation of CS: A 13-year-old woman with no relevant medical history was admitted to the hospital with an episode of hematemesis.
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