The association of congenital hepatic fibrosis (CHF) with autosomal recessive polycystic kidney disease (ARPKD) is well known and occurs in approximately 50% of cases. However the association of CHF with autosomal dominant polycystic kidney disease (ADPKD) is less well known and less well documented. We report a child with neonatal onset of hypertension due to ADPKD who later develops portal hy...

Background Ventricular fibrosis in adult congenital heart disease (ACHD) has been shown to relate to adverse clinical markers.[1,2] Novel free-breathing, motion-corrected late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) has been shown to be advantageous in quantifying LV LGE, but has not been evaluated for RV LGE. [3] It may therefore also be beneficial in ACHD patients, ...

Corresponding author : Haeryoung Kim Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro 173-beon-gil, Bundang-gu, Seongnam 463-707, Korea Tel. +82 31-787-7715, Fax. +82 31-787-4012 E-mail; [email protected] Abbreviations: BilIN, biliary intraepithelial neoplasia; CD, Caroli's disease; CHF, congenital hepatic fibrosis...

Liver fibrosis is the consequence of chronic or repeated liver injury caused by hepatotoxic agents like alcohol and viruses, as well as immune and congenital metabolic disorders. Nonalcoholic fatty liver disease (NAFLD), caused by obesity and abnormal lipid metabolism, may be the latest known cause of liver fibrosis and cirrhosis. Furthermore, NAFLD with obesity can provide a terrain in which a...