Non Cirrhotic Portal Fibrosis: Prognosis and Life Expectancy with NCPF

Question: I am 26 years old and I have problem of Non Cirrhotic Portal Fibrosis. Can physical exercise reduce this problem. I am taking tablet for this. Can I lead long life? Give some more details about this disease and what is the Prognosis and Life Expectancy with NCPF?

Answer:

Non Cirrhotic Portal Fibrosis

It is a distinct variety of portal hypertension which exists in adults between 25 to 35 years, and is characterized by splenomegaly, anemia, episodes of bleeding from gastrointestinal varices, hepatic encephalopathy and absence of ascites. You can ask your doctor regarding doing physical exercise, to my opinion it is not advisable to do heavy exercise.

Do you have varices? And if present take care of the varices in consultation with your doctor and you will not have any problem to lead a long life. The incidence of NCPF is 3-5% in the patients who suffer of portal hypertension.

Causes Of Non Cirrhotic Portal Fibrosis

The precise aetiology of NCPF remains an enigma, because similar lesions during histological review were known to be produced by other stimuli. This include

  • Exposure to chemicals such as vinyl chloride, copper, arsenic,
  • Bacterial infection of the intestine,
  • When the blood flow of the portal system increases
  • Abnormal immunological conditions
  • Abnormal genetic factors.

Clinical Features and Symptoms Of Non Cirrhotic Portal Fibrosis

Gastrointestinal bleeding due to varices is a common symptom in most of the patients suffering from NCPF.

  • Anemia is present in this patients
  • Swelling in the left side of the upper abdomen due to enlarged spleen.

Laboratory findings: low Hb, low WBC count, low platelet count are frequently seen. Liver function tests are normal like SGPT, bilirubin. Endoscopy will reveal esophageal varices in 90% of patients. Ultrasonography reveals dilatation of portal and splenic veins, with thickening of the portal veins. Treatment: Beta blocker tablets and band ligation, are recommended by many physicians as primary prophylaxis of bleeding due to varices, as majority of patients come with variceal bleeding. Do not consume alcohol or tobacco in any form. Prognosis: NCPF runs a benign course and if variceal bleeding is taken care of and controlled, the long term prognosis is very good.

2 Comments

  1. D R said:

    A female friend of mine has with massive Splenomegaly,
    – Normal liver n liver function tests, negative viral markers
    – No febrile illness, ogd-normal, portal doppler-dilated portal n splenic vein s/o portal HT,
    – She has thrombocytopenia and bleeding manifestations
    – No KF ring…coombs test negativ, ana-negativ, serum ceruloplasmin-mild reduction

    She has hypo-functioning goiter too..What could be the cause?

    November 17, 2010
    Reply
    • PUP said:

      Your friend needs an expert opinion by a gastroenterologist; there are several causes for enlarged spleen and portal hypertension. The cause may be pre-hepatic, post-hepatic or intra-hepatic. It may be an idiopathic to more serious like polycythemia vera or non cirrhotic portal fibrosis condition or a sequel of immunologic presentation such as hypothyroidism. A detailed history, examination and evaluation will make the diagnoses easier.

      November 19, 2010
      Reply

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