Rapid evolution of congenital hepatic fibrosis after liver transplantation for acute liver failure: The potential role of extrahepatic factors

Judith Flores, Ronen Arnon, Raffaella A. Morotti, Lisa Guay-Woodford, Sukru Emre, Benjamin L. Shneider

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

A 9-year-old male underwent deceased donor liver transplantation for idiopathic fulminant hepatic failure. In the postoperative period, a review of perioperative biopsies of the donor liver revealed mild features of congenital hepatic fibrosis. Over the ensuing year, the recipient developed severe complications of congenital hepatic fibrosis including multiple episodes of cholangitis and progressive portal hypertension. After a second transplant, the patient had no further episodes of cholangitis and/or features of portal hypertension. Examination of the explanted liver revealed remarkable progression of the congenital hepatic fibrosis, suggesting that the phenotype of this disease may be dependent in part on extrahepatic factors.

Original languageEnglish
Pages (from-to)660-664
Number of pages5
JournalLiver Transplantation
Volume14
Issue number5
DOIs
StatePublished - May 2008

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