Long-term outcome of pediatric non-cirrhotic portal fibrosis from the viewpoint of endoscopic profile

Excerpt

Non-cirrhotic portal fibrosis (NCPF) presents with non-cirrhotic portal hypertension characterized by moderate to severe splenomegaly, evidence of varices and/or collaterals, patent splenoportal axis and hepatic veins, normal liver functions, normal or near-normal hepatic venous pressure gradient (HVPG), and no evidence of cirrhosis or parenchymal injury in the liver histology [1]. The disease occurs worldwide but is common in socioeconomically disadvantaged countries, including India, and patients are generally young in their third and fourth decades of life. A similar disease group called idiopathic portal hypertension (IPH) in Japan has female preponderance and presents in the fourth and fifth decades of life [2]. This condition is known as idiopathic non-cirrhotic portal hypertension (INCPH) in the western countries [3]. …