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

被引：5

作者：

Black, D

Reutter, J

Johnson, M

Fair, J

Woosley, J

Gerber, D

机构：

[1] Univ N Carolina Hosp, Dept Gen Surg, Div Transplantat, Chapel Hill, NC 27599 USA

[2] Univ N Carolina Hosp, Dept Pathol, Chapel Hill, NC USA

来源：

PEDIATRIC TRANSPLANTATION | 2002年 / 6卷 / 03期

关键词：

Ellis-van Creveld syndrome; liver transplantation; paucity of intra-hepatic bile ducts;

D O I：

10.1034/j.1399-3046.2002.01085.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：255 / 259

页数：5

共 13 条

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