Portosinusoidal vascular disease

定义 英文原文（暂无中文）

A rare vascular liver disease characterized by the absence of cirrhosis with or without portal hypertension and histological lesions involving portal venules or sinusoids. Patients may present either with specific clinical signs of portal hypertention (including gastric esophageal or ectopic varices, portal hypertensive bleeding or porto-systemic collaterals at imaging) or a histological lesion specific to portosinusoidal vascular disease (including obliterative portal venopathy, nodular regenerative hyperplasmia or incomplete septal fibrosis or cirrhosis) or they may manifest clinical signs that are not specific to portal hypertention (such as ascites, platelet count <150.000/µl, increased spleen size &#8805;13&nbsp;cm in the largest axis) together with a histological lesion including portal tract abnormalities, irregular distribution of the portal tracts and central veins, non-zonal sinusoidal dilation, mild perisinusoidal fibrosis. Possible associations with altered immunity, hematological diseases and prothrombotic conditions, infections, congenital or familial defects, or drug exposure are reported.

别名

PSVD

基本事实

遗传方式

不适用

发病年龄

成年期

外部标识与链接