Paediatric liver transplantation for metabolic disorders. Part 1: Liver-based metabolic disorders without liver lesions

被引:22
作者
Darwish, Ahmed A. [2 ]
McKiernan, Patrick [3 ]
Chardot, Christophe [1 ]
机构
[1] Hop Necker Enfants Malad, Serv Chirurg Pediat, F-75743 Paris 15, France
[2] Univ Geneva Childrens Hosp, Paediat Surg Unit, Geneva, Switzerland
[3] Birmingham Childrens Hosp, Liver Unit, Birmingham, W Midlands, England
关键词
SYNDROME TYPE-I; HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA; ISOLATED HEPATOCYTE TRANSPLANTATION; TRANSCARBAMYLASE DEFICIENT PATIENT; HEMOLYTIC-UREMIC SYNDROME; UREA CYCLE DEFECTS; METHYLMALONIC ACIDEMIA; PRIMARY HYPEROXALURIA; LIVING DONOR; CELL TRANSPLANTATION;
D O I
10.1016/j.clinre.2011.01.006
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Liver-based metabolic disorders account for 10 to 15% of the indications for paediatric liver transplantation. In the last three decades, important progress has been made in the understanding of these diseases, and new therapies have emerged. Concomitantly, medical and surgical innovations have lead to improved results of paediatric liver transplantation, patient survival nowadays exceeding 80% 10-year after surgery with close to normal quality of life in most survivors. This review is a practical update on medical therapy, indications and results of liver transplantation, and potential future therapies, for the main liver-based metabolic disorders in which paediatric liver transplantation may be considered. Part 1 focuses on metabolic based liver disorders without liver lesions, and part 2 on metabolic liver diseases with liver lesions. (C) 2011 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:194 / 203
页数:10
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