title: [A rare etiology of portal hypertension in an adult: congenital hepatic fibrosis. Review of current literature in connection with an observation (author's transl)].
abstract: On the basis of a personal observation the authors review the current literature on the hypertensive forms of congenital hepatic fibrosis found in an adult subject during G.I. haemorrhage through the rupture of gastro-oesophageal varices. These observations seem to be exceptional and 17 cases were recorded between 1956 and 1976. A complete hemodynamic portal study was carried out only in six cases. It shows a presinusoïdal portal hypertension in three cases, post-sinusoïdal or sinusoïdal portal hypertension in two cases, mixed hypertension in one case. Our observation is outstanding because of the existence of a rise of the wedged hepatic venous pressure varying from one hepatic vein to the other. The prognosis seems to be relatively bad for the short-term as the mortality rate of 2 years reaches at least 29%.
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D008107:Liver Diseases / Q000150:complications* / Q000151:congenital; D008297:Male; D008875:Middle Aged; D011167:Portacaval Shunt, Surgical; D011379:Prognosis
vernacular_title: Une étiologie rare d'hypertension portale chez l'adulte: la fibrose hépatique congénitle. Revue de la littérature à propos d'une observation.