NCPF is the abbreviation of Non-Cirrhotic Portal Fibrosis. This liver disease arises from an issue called Non-cirrhotic portal hypertension. It is an autosomal recessive disorder. NCPF is also a chronic liver disease which means that the condition stays in for a long time, approximately for 6 months. The main symptoms of this disease include portal hypertension, which is the abnormal increase in the portal venous pressure that drains most of the blood to the liver. Another prominent symptom is splenomegaly, which means that the spleen is enlarged, which results in the reduction of the number of active blood cells. Another common symptom is variceal bleeding, where submucosal veins in the oesophagus are dilated.
NCPF can be identified primarily through thrombosis and sclerosis of portal veins. Thrombosis means the formation of blood clots restricting the flow of blood throughout the body. At the same time, sclerosis is the stiffening of the tissue. Such a deformed structure of the portal vein is known as mesangial sinusoids, or periportal cavernoma. Usually, patients with this case are often misdiagnosed with liver cirrhosis.
Treatment of the bleeding occurring is one way to control the disease. Frequent endotherapy and surveillance will help with an updated status of the condition. Surgery is the permanent solution for those coping well with the treatment method. Although sometimes, this shows little success.
The below mentioned are the most common symptoms observed during the disease. Not all of the below mentioned are required in every case.
Splenomegaly
Anaemia
Portal Hypertension
Absence of cirrhosis
Absence of blood disease
Absence of parasites in hepatobiliary system
Normal to near normal liver function tests
Varices showing endoscopy
Low level of formed elements in blood
Elevated portal pressure
Scanning of liver not showing typical cirrhosis
Presence of extrahepatic portal vein with frequent collateral vessels
During portal hypertension occurring during NCPF, the central vein that leads to the liver is dilated.
Although very rare, Idiopathic non-cirrhotic portal hypertension (INCPH) may occur without cirrhosis.
NCPF is more commonly reported in India and Japan
The aetiology is unclear, but the reasons may be chronic infections, exposure to toxins, thrombophilia, and genetic or immunological disorders.
NCPF is a benign disorder and shows an overall good prognosis.