title: Rapid evolution of congenital hepatic fibrosis after liver transplantation for acute liver failure: the potential role of extrahepatic factors.
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.
authors.affiliation: Department of Gastroenterology, Hospital de Pediatria, Centro Medico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico.
authors.forename: Judith
authors.initials: J
authors.lastname: Flores
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.forename: Raffaella A
authors.initials: RA
authors.lastname: Morotti
authors.forename: Lisa
authors.initials: L
authors.lastname: Guay-Woodford
authors.forename: Sukru
authors.initials: S
authors.lastname: Emre
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Shneider
mesh_terms: D002648:Child; D002761:Cholangitis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D018450:Disease Progression; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D008099:Liver / Q000473:pathology* / Q000601:surgery; D008103:Liver Cirrhosis / Q000150:complications* / Q000151:congenital / Q000473:pathology / Q000601:surgery; D017114:Liver Failure, Acute / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D012086:Reoperation; D018805:Sepsis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D013997:Time Factors; D016896:Treatment Outcome