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肝移植在儿童肝腺瘤病中的作用:病例报告及文献综述

The role of liver transplantation for hepatic adenomatosis in the pediatric population: case report and review of the literature.

作者信息

Wellen J R, Anderson C D, Doyle M, Shenoy S, Nadler M, Turmelle Y, Shepherd R, Chapman W C, Lowell J A

机构信息

Section of Transplantation, Washington University School of Medicine, St. Louis, MO 63110, USA.

出版信息

Pediatr Transplant. 2010 May;14(3):E16-9. doi: 10.1111/j.1399-3046.2008.01123.x. Epub 2009 Mar 10.

Abstract

Hepatic adenomas are benign lesions often found in young women during childbearing age. These tumors are often solitary but can also be multiple in which case this is referred to as hepatic adenomatosis (HA). HA is defined as having greater than or equal to ten adenomas within an otherwise normal liver. We present a case of a teenager with HA who underwent an orthotopic liver transplant for complications of her HA. To date there are only four reports of teenagers, without an underlying glycogen storage disease, who have undergone a liver transplant for HA. Liver transplantation within the pediatric population is an acceptable treatment for HA that are deemed unresectable.

摘要

肝腺瘤是一种良性病变,常见于育龄期年轻女性。这些肿瘤通常为单发,但也可能多发,多发时称为肝腺瘤病(HA)。HA的定义是在肝脏基本正常的情况下腺瘤数量大于或等于10个。我们报告一例患有HA的青少年患者,因HA的并发症接受了原位肝移植。迄今为止,仅有4例无潜在糖原贮积病的青少年因HA接受肝移植的报道。对于被认为无法切除的HA,肝移植在儿童群体中是一种可接受的治疗方法。

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