Noncirrhotic intrahepatic portal hypertension: Towards a unifying definition and etiology

Excerpt

Noncirrhotic portal fibrosis (NCPF) is a condition that was recognized in India in the middle of the last century principally by Basu, Das and Aikat in Kolkata [1‐3]. Their investigations identified patients with portal hypertension who appeared to have portal fibrosis with intrahepatic portal hypertension and who did not meet the histopathological definition of cirrhosis. A large number of studies in the 1970s further described the clinical syndrome of NCPF outside of Bengal, particularly in northern India. Clinically, the disease was defined by repeated episodes of variceal bleeding that are well tolerated, moderate to large splenomegaly and absence of evidence of portal or splenic vein thrombosis. Although never documented by comparative studies, the impression of many academic gastroenterologists was that the disease was much more commonly diagnosed in the northern than in the southern part of India. …