No abstract

Our result indicates that OPN, an acidic glycoprotein from intrahepatic bile ducts and from intramural and extramural glandular epithelia, seems to be involved in lithiasis, both as a core protein in the early phase, and in the late phase.

Alagille syndrome (ALGS) is a multisystem autosomal dominant disorder attributed to defects in the Notch signaling pathway. The mutation of Jagged 1 (JAG1) gene on chromosome 20 has been identified as the underlying cause of this syndrome. In addition, the mutation of Ryanodine receptor 2 (RYR2) gene on chromosome 1 has clarified. Here described is a 7 × 5 cm nodular lesion in segment 4 in a 28-year-old man with ALGS. Through imaging studies and histopathological analysis, the nodule was diagnosed, for the first time, as iron deposit giant nodule. Additionally, a review of previous reports has been made to shed more light on this syndrome.This study describes the case of a 28-year-old man with a 7 × 5 cm nodular lesion in segment 4 compatible with iron deposit central large nodule detected by radiological and histopathological analysis. We also review previous reports that describe solitary focal hepatic nodules adjacent to the right portal vein as large regenerative hyperplasia.