IDIOPATHIC PORTAL-HYPERTENSION - AN UPDAT E WITH REFERENCE TO A CASE-REPORT

Idiopathic portal hypertension is common in Japan and India but exceed ingly rare in Europe and North America. Its pathogenesis remains unelu cidated, but may involve phlebosclerosis associated with protein C def iciency or fibrosis upstream from the sinusoids with consequent sinuso id obstruction. Possible etiologic factors include abdominal infection s, exposure to toxic substances, and disorders characterized by immune dysfunction. Onset can occur at any age. Digestive bleeding is the pr esenting manifestation in most instances, although enlargement of the spleen or hematologic disorders are inaugural in some cases. Portograp hy and liver histology (which often requires a surgical biopsy) demons trate fairly specific venous abnormalities. Bleeding from esophageal v arices is the main complication. In the long term, some patients devel op liver failure with manifestations similar to those of advanced cirr hosis. The indications of surgery, recently developed decompression pr ocedures (ligations and transjugular intrahepatic portosystemic shunts ), and sclerosis are discussed.