Non-cirrhotic portal fibrosis

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Non-cirrhotic portal fibrosis
Autosomal recessive - en.svg
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).

Presentation[edit]

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]

Diagnosis[edit]

Hallmark of the disease is thrombosis/sclerosis of branches of portal vein. Vessels formed are often termed as mesangiosinusoids or periportal cavernoma.

Treatment[edit]


References[edit]

  1. "Non-cirrhotic portal fibrosis: one disease with many names? An analysis from morphological study of native explant livers with end stage chronic liver disease.".Journal of Clinical Pathology.July 2011;64(7)
    592–8.doi:10.1136/jcp.2010.087395.PMID:21685145.
  2. "Non-cirrhotic portal fibrosis.".Journal of Gastroenterology and Hepatology.December 2002;17 Suppl 3
    S214-23.PMID:12472939.

External links[edit]