"Prior terms for this entity include non-cirrhotic portal fibrosis, idiopathic portal hypertension, idiopathic portal fibrosis, and Banti syndrome 5,6. Idiopathic non-cirrhotic portal hypertension was proposed in 2011 as the uniform term 5."
"Prior terms for this entity include non-cirrhotic portal fibrosis, idiopathic portal hypertension, idiopathic portal fibrosis, and Banti syndrome 5,6. Idiopathic non-cirrhotic portal hypertension was proposed in 2011 as the uniform term 5."
"Prior terms for this entity include non-cirrhotic portal fibrosis, idiopathic portal hypertension, idiopathic portal fibrosis, and Banti syndrome 5,6. Idiopathic non-cirrhotic portal hypertension was proposed in 2011 as the uniform term 5."
"As a clinical diagnosis, idiopathic non-cirrhotic portal hypertension encompasses a spectrum of histopathologic entities: nodular regenerative hyperplasia, incomplete septal cirrhosis, obliterative portal venopathy, and, less commonly, partial nodular transformation. The term hepatoportal sclerosis has also been used in a manner overlapping with obliterative portal venopathy. However, these morphologic alterations can be seen without portal hypertension. The Vascular Liver Disease Interest Group proposed in 2019 the term portosinusoidal vascular disease to encompass all those patients with histologic features historically associated with idiopathic non-cirrhotic portal hypertension, regardless of whether they actually have portal hypertension 6."
"As a clinical diagnosis, idiopathic non-cirrhotic portal hypertension encompasses a spectrum of histopathologic entities: nodular regenerative hyperplasia, incomplete septal cirrhosis, obliterative portal venopathy, and, less commonly, partial nodular transformation. The term hepatoportal sclerosis has also been used in a manner overlapping with obliterative portal venopathy. However, these morphologic alterations can be seen without portal hypertension. The Vascular Liver Disease Interest Group proposed in 2019 the term portosinusoidal vascular disease to encompass all those patients with histologic features historically associated with idiopathic non-cirrhotic portal hypertension, regardless of whether they actually have portal hypertension 6."
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"Associations"
"As a clinical diagnosis, idiopathic non-cirrhotic portal hypertension encompasses a spectrum of histopathologic entities: nodular regenerative hyperplasia, incomplete septal cirrhosis, obliterative portal venopathy, and, less commonly, partial nodular transformation. The term hepatoportal sclerosis has also been used in a manner overlapping with obliterative portal venopathy. However, these morphologic alterations can be seen without portal hypertension. The Vascular Liver Disease Interest Group proposed in 2019 the term portosinusoidal vascular disease to encompass all those patients with histologic features historically associated with idiopathic non-cirrhotic portal hypertension, regardless of whether they actually have portal hypertension 6."
"Non-specific histological lesions in portosinusoidal vascular disease include portal tract abnormalities (multiplication, arterial dilation, periportal vascular channels, aberrant vessels), architectural disturbance (irregular distribution of portal tracts and central veins), non-zonal sinusoidal dilation, and mild perisinusoidal fibrosis 6."
"Patent large hepatic and portal veins have been cited as a requirement for diagnosis 5, but intrahepatic and extrahepatic portal vein abnormalities are common in obliterative portal venopathy, including thrombosis, diminished calibre, lack of visibility, and wall thickening 7,8. The hepatic artery may be enlarged 6."
"obstruction of the hepatic or portal veins (e.g. Budd-Chiari syndrome, portal vein thrombosis)"
"immunological disorders (e.g. systemic sclerosis, systemic lupus erythematosus, primary hypogammaglobulinemia, Felty syndrome)"
"chronic infections (e.g. HIV, bacterial infections of the gut)"
"medications and toxins (e.g. azathioprine, 6-thioguanine, arsenic)"
"genetic disorders (e.g. Turner syndrome, Adams-Oliver syndrome)"
"Thought to have a more favourable outcome than cirrhotic portal hypertension, but treatment strategies currently attempt to control the varices rather than treat the portal hypertension."