Liver transplantation in Ellis-van Creveld syndrome: A case report

Dalliah Black, Jason Reutter, Mark Johnson, Jeffrey Fair, John Woosley, David Gerber

Research output: Contribution to journalArticle

8 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)255-259
Number of pages5
JournalPediatric Transplantation
Volume6
Issue number3
DOIs
StatePublished - Aug 13 2002
Externally publishedYes

Keywords

  • Ellis-van Creveld syndrome
  • Liver transplantation
  • Paucity of intra-hepatic bile ducts

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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