The pathology of noncirrhotic portal fibrosis: a review of 32 autopsy cases.

摘要: Abstract A wide spectrum of clinical and morphologic changes in 32 autopsy cases noncirrhotic portal fibrosis have been described. The disease frequently occurs younger patients with a long history splenomegaly, usually hematemesis. Females are affected almost equally as often males contrast to cirrhosis. tolerate the bleeding episodes well. Death is due massive hemorrhage. diagnosis achieved through process exclusion. critical analysis hemodynamic data, splenoportogram, liver function tests (particularly Bromsulphalein retention) angiographic data mandatory. Needle biopsy appears limited value making diagnosis. gross anatomic findings vary from nearly normal nodularity, seen particularly on posteroinferior surface. In some these nodules physically impede blood flow contribute hypertension. Phlebosclerosis smaller radicles vein irregular scarring outstanding features disease. These associated dilatation larger intrahepatic branches well fibroelastosis or without occluding organizing thrombi both intra- extrahepatic vein. hepatic venous characterized by sclerosis, which seems significantly toward postsinusoidal block advanced cases. probable mode evolution discussed.