NON-CIRRHOTIC PORTAL FIBROSIS (NCPF)
Non-cirrhotic portal fibrosis is a chronic liver disease. It is characterized by perivenular fibrosis of the small and medium branches of the portal vein resulting in portal hypertension. During this liver function still remain normal.
Causes
The exact cause of NCPF is unclear. Though, it is associated with the following:
- Chronic infections
- Exposure to trace metals or toxins
- Immunological disorders
- Genetic disorders
Symptoms
The most common symptoms include:
- Gastrointestinal bleeding
- Enlarged spleen.
- Upper abdominal pain
- Ascites
Diagnosis
Starting with analyzing the symptoms, the most common diagnosis methods used are:
- Imaging: Ultra sonography with hepatoportal doppler is the most common imaging procedure used.
- Liver biopsy: It is sometimes obtained to differentiate NCPF from cryptogenic cirrhosis
- Endoscopy: Endoscopy may also be used to screen esophageal varices and treat them.
Treatment and Management
The treatment involves control of acute bleeding and prevention of rebleeding by medical treatment.
In cases of emergency, endoscopic therapy such as Endoscopic variceal band ligation or Endoscopic sclerotherapy could be opted. Other options are Pharmacotherapy which includes administrating drugs for emergency situations. Balloon tamponade may also be used only in massive bleeding as a temporary measure.
In other management techniques, regular medication is prescribed. These may include b blockers to reduce blood flow to control bleeding. There are also preventive procedures such as EST and ELV that could be elected as per patient’s condition.
Surgery
The surgical management for bleeding involves use of portosystemic shunt and devascularization procedure when shunt cannot be performed.
NCPF has been reported from all over the world, with maximum cases reported from India. As per data, in India NCPF accounts for almost one third of variceal bleeds in non-cirrhotic portal hypertension. Also, the patients are usually young and healthy. This presents a critical need of evidence backed solutions to the condition.