فهرست نشریات علمی دسترسی آزاد ایران

Background: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular dysplasia. Objectives: The aim is to characterize the liver involvement of HHT by multi-detector helical computed tomography. Results: (i) Three types of shunting were found in the livers, including arteriovenous (hepatic artery to hepatic vein) in 6 cases, arterioportal (hepatic artery to portal vein) in 2 cases, and portal venous (portal vein to hepatic vein) in 4 cases, (ii) Biliary diseases were detected in 8 cases, including 3 cases of bilomas accompanied by increases of alkaline phosphatase (mean 271 units, ranging from 152 to 479 units) and arteriovenous shunts, and the other 5 cases revealed slight biliary dilation with no increase of alkaline phosphates, but were instead accompanied by arteriovenous shunts (3 cases) and portal venous shunts (2 cases). (iii) Vascular anatomic variants were observed in 4 cases, including 3 with accompanying arteriovenous shunts and 1 with accompanying arterioportal shunting. Conclusion: The involved liver with HHT typically shows vascular shunting and biliary diseases. Also, arteriovenous shunts may be vulnerable to biliary diseases.