title: Liver transplantation in Ellis-van Creveld syndrome: a case report.
abstract: This case represents a rare association of Ellis-van Creveld (EvC) syndrome, a chondroectodermal disorder, with congenital paucity of bile ducts. Sequential liver biopsies during the patient's childhood demonstrated progressive fibrosis that can occur in other chondrodysplastic malformations. However, this EvC case is the first report to demonstrate paucity of intra-hepatic bile ducts progressing to cirrhosis and subsequently requiring transplant.
authors.affiliation: Division of Transplantation, Department of General Surgery, The University of North Carolina Hospital, Chapel Hill, North Carolina 27559, USA.
authors.forename: Dalliah
authors.initials: D
authors.lastname: Black
authors.forename: Jason
authors.initials: J
authors.lastname: Reutter
authors.forename: Mark
authors.initials: M
authors.lastname: Johnson
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Fair
authors.forename: John
authors.initials: J
authors.lastname: Woosley
authors.forename: David
authors.initials: D
authors.lastname: Gerber
mesh_terms: D004613:Ellis-Van Creveld Syndrome / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male