Ductal plate malformation and congenital hepatic fibrosis Clinical and histological findings in four patients

Hepatology Research : the Official Journal of the Japan Society of Hepatology
Olga I GioulemeNikolaos Eugenidis

Abstract

Congenital hepatic fibrosis belongs to the fibrocystic diseases of the liver and represents ductal plate malformation of interlobular bile ducts, along with a destructive cholangiopathy associated with fibrosis. Four patients with congenital hepatic fibrosis are described. Their median age at presentation was 25 years; none of them had a family history of liver or renal disease. Variceal bleeding was the initial manifestation in three patients. All of them required frequent endoscopic variceal ligation sessions and distal splenorenal shunting was also performed in two, almost obviating the need for further variceal ligation. Variceal bleeding did not recur during follow-up. One of these three patients rarely exhibited acute cholangitis; administration of ursodeoxycholic acid resulted in complete remission. In contrast, the fourth patient showed frequent severe episodes of acute cholangitis but normal cholangiographic findings. He underwent liver transplantation but died 2 months later. Laboratory findings disclosed pancytopenia in all patients whereas hepatic synthetic capacity was well preserved. Renal function was unaffected despite the presence of polycystic kidneys in two patients. In summary, congenital hepatic fibrosis ca...Continue Reading

Citations

Jan 19, 2010·Veterinary Pathology·D L BrownJ M Cullen
Jan 13, 2015·Der Radiologe·S Pötter-LangA Ba-Ssalamah
Oct 5, 2019·American Journal of Clinical Pathology·Mohammed I AlsomaliWei Chen
Jan 28, 2016·The Cochrane Database of Systematic Reviews·Lars G HemkensMatthias Briel
Jan 28, 2015·World Journal of Gastroenterology : WJG·Silvia BerardisEtienne Marc Sokal

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