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- W1970486521 abstract "In cases of idiopathic portal hypertension (IPH), the deposition of elastic fibers in the major portal vein branches and peripheral portal tracts is a common and characteristic histological finding, which may be related to the disease's pathogenesis. This study aimed to clarify the mechanism of this portal fibroelastosis.The expression of fibulin-5 and fibrillin-1, proteins essential for elastogenesis, was examined in IPH livers (n = 15) using immunohistochemistry. Liver specimens obtained from patients with chronic viral hepatitis (CVH)/liver cirrhosis (LC) (n = 12) and normal/subnormal livers (n = 10) were used as controls.In IPH livers, immunohistochemical labeling of fibulin-5 was observed in the major portal vein branches in eight cases (53%), and the distribution corresponded to that of elastic fibers in the vessel walls, while the peripheral portaltracts totally lacked fibulin-5 in spite of the presence of dense elastic fibers. In CVH/LC and normal livers, fibulin-5 expression was absent or faint throughout the sections. Fibrillin-1 was detected in the connective tissue of the hilar region and peripheral portal tracts in IPH, CVH/LC and normal livers, with the expression varying greatly among cases.These results suggest that fibulin-5, rather than fibrillin-1, expressed in the major portal vein branches of IPH livers is related to phlebosclerosis, leading to an increase in presinusoidal vascular resistance and portal hypertension. In addition, the mechanism of fibroelastosis may differ between the major portal vein branches and peripheral portal tracts of IPH livers." @default.
- W1970486521 created "2016-06-24" @default.
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- W1970486521 date "2007-08-15" @default.
- W1970486521 modified "2023-10-16" @default.
- W1970486521 title "Fibulin-5 is involved in phlebosclerosis of major portal vein branches associated with elastic fiber deposition in idiopathic portal hypertension" @default.
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- W1970486521 doi "https://doi.org/10.1111/j.1872-034x.2007.00253.x" @default.
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