Non-cirrhotic portal fibrosis | |
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Non-cirrhotic portal fibrosis is inherited in an autosomal recessive manner |
Non-cirrhotic portal fibrosis (NCPF) is a chronic liver disease [1] and type of non-cirrhotic portal hypertension (NCPH).
It is characterized by 'obliterative portovenopathy', which leads to various problems such as portal hypertension, massive splenomegaly, and variceal bleeding. It is estimated that about 85% of people with NCPF have repeated episodes of variceal bleeding. [2]
Hallmark of the disease is thrombosis/ sclerosis of branches of portal vein. Vessels formed are often termed as mesangiosinusoids or periportal cavernoma.[ citation needed]
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