A Case of Congenital Hepatic Fibrosis Associated With Medullary Sponge Kidney-Radiologic and Pathologic Features
| Autor: | Lei Zhu, Yan Li, Gang Zhao, Chong-Fu Jia |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2012 |
| Předmět: |
medicine.medical_specialty
Pathology medicine.diagnostic_test business.industry Hepatosplenomegaly Case Report medicine.disease Medullary sponge kidney Gastroenterology Liver biopsy Ectasia Internal medicine Congenital hepatic fibrosis medicine Portal hypertension Liver function medicine.symptom business Radiology Rare disease |
| Zdroj: | Gastroenterology Research |
| ISSN: | 1918-2813 1918-2805 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|