Non-cirrhotic portal fibrosis (NCPF) is a chronic liver disease[1] and type of non-cirrhotic portal hypertension (NCPH).
| Non-cirrhotic portal fibrosis | |
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| Non-cirrhotic portal fibrosis is inherited in an autosomal recessive manner |
Presentation
editIt 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
editHallmark of the disease is thrombosis/sclerosis of branches of portal vein. Vessels formed are often termed as mesangiosinusoids or periportal cavernoma.[citation needed]
Treatment
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References
edit- ^ 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. S2CID 24867131.
- ^ 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.