| Autor: |
Zhu L; Department of Gastroenterology, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China., Zhao G; Department of Gastroenterology, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China., Jia CF; Chong-Fu Jia, Cardiovascular Radiology Center, The First Affiliated Hospital, Dalian Medical University, Dalian City, 116011, Liaoning Province, China., Li Y; Department of Gastroenterology, Shengjing Hospital, China Medical University, Shenyang City, 110001, Liaoning Province, China. |
| Abstrakt: |
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. |
