Non-cirrhotic portal fibrosis

Non-cirrhotic portal fibrosis
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

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

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

Liver transplantation

References

  1. ↑ Nayak, NC; Jain, D; Saigal, S; Soin, AS (July 2011). "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. 64 (7): 592–8. doi:10.1136/jcp.2010.087395. PMID 21685145.
  2. ↑ Sarin, SK (December 2002). "Non-cirrhotic portal fibrosis". Journal of Gastroenterology and Hepatology. 17 Suppl 3: S214-23. doi:10.1046/j.1440-1746.17.s3.3.x. PMC 1378468. PMID 12472939.


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