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

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

doi:10.1034/j.1399-3046.2002.01085.x

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 journal › Article › peer-review

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 language	English (US)
Pages (from-to)	255-259
Number of pages	5
Journal	Pediatric Transplantation
Volume	6
Issue number	3
DOIs	https://doi.org/10.1034/j.1399-3046.2002.01085.x
State	Published - 2002
Externally published	Yes

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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10.1034/j.1399-3046.2002.01085.x

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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.",

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T2 - A case report

AU - Black, Dalliah

AU - Reutter, Jason

AU - Johnson, Mark

AU - Fair, Jeffrey

AU - Woosley, John

AU - Gerber, David

PY - 2002

Y1 - 2002

N2 - 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.

AB - 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.

KW - Ellis-van Creveld syndrome

KW - Liver transplantation

KW - Paucity of intra-hepatic bile ducts

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JO - Pediatric Transplantation

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ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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