title: [Hepatoportal sclerosis: apropos of a familial case].
abstract: BACKGROUND
  Hepatoportal sclerosis is uncommon in European countries and its diagnosis is difficult. The etiology remains unknown; its familial origin is probably exceptional.
  
  
  METHODS
  An 18-month-old girl born to a mother with hepatoportal sclerosis had hepatomegaly. She also had a moderate splenomegaly and mild increase transaminase and gamma GT activities. Ultrasound examination failed to show portal hypertension. Histological study of liver showed changes quite similar to those seen in her mother. Two members of the mother's family had portal hypertension.
  
  
  CONCLUSIONS
  Familial forms of portal hypertension have been reported: a familial form of incomplete septal cirrhosis and a familial occurrence of cavernous transformation of the portal vein. Obstruction of the portal vessels was not found in our patient, the youngest in whom hepatoportal sclerosis is documented.
authors.affiliation: Service de pédiatrie, centre hospitalier Louis-Pasteur, Cherbourg, France.
authors.forename: T
authors.initials: T
authors.lastname: Maugard
authors.forename: A
authors.initials: A
authors.lastname: David
authors.forename: M F
authors.initials: MF
authors.lastname: Nomballais
authors.forename: D
authors.initials: D
authors.lastname: Golfain
authors.forename: M
authors.initials: M
authors.lastname: Le Bideau
authors.forename: A
authors.initials: A
authors.lastname: Mouzard
mesh_terms: D001706:Biopsy; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000235:genetics* / Q000473:pathology; D007223:Infant; D008099:Liver / Q000473:pathology*; D011169:Portal Vein / Q000473:pathology*; D012598:Sclerosis