title: A Case of Congenital Hepatic Fibrosis Associated With Medullary Sponge Kidney-Radiologic and Pathologic Features.
abstract: Congenital hepatic fibrosis is an exceedingly rare disease in China, where only very few cases with sufficient evidences and clinical data have been reported up to now. Here we reported a young patient, onset of hematemesis and melena, who had striking portal hypertension but without liver function damage. Computer tomography scans showed hepatosplenomegaly, intra-hepatic bile ducts dilation, thickening portal vein and tortuous spleen vein, and medullary sponge kidney. Liver biopsy found significant fibrosis in the portal area and ectasia of bile ductules. With sufficient radiologic and pathologic data, our case revealed the features of congenital hepatic fibrosis associated with medullary sponge kidney.
authors: Department of Gastroenterology, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China.
authors: Lei
authors: L
authors: Zhu
authors: Department of Gastroenterology, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China.
authors: Gang
authors: G
authors: Zhao
authors: Chong-Fu Jia, Cardiovascular Radiology Center, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China.
authors: Chong-Fu
authors: CF
authors: Jia
authors: Department of Gastroenterology, Shengjing Hospital, China Medical University, Shenyang City, 110001, Liaoning Province, China.
authors: Yan
authors: Y
authors: Li
keywords: Congenital hepatic fibrosis; Medullary sponge kidney; Pathology; Radiology