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Beyond the Pediatric end-stage liver disease system: Solutions for infants with biliary atresia requiring liver transplant

Beyond the Pediatric end-stage liver disease system: Solutions for infants with biliary atresia requiring liver transplant

作     者:Mary Elizabeth M Tessier Sanjiv Harpavat Ross W Shepherd Girish S Hiremath Mary L Brandt Amy Fisher John A Goss 

作者机构:Department of Pediatrics Baylor College of Medicine and Texas Children’s Hospital Department of Pediatrics Baylor College of Medicine and Texas Children’s Hospital Houston Department of Surgery Baylor College of Medicine and Texas Children’s HospitalHouston 

出 版 物:《World Journal of Gastroenterology》 (世界胃肠病学杂志(英文版))

年 卷 期:2014年第20卷第32期

页      面:11062-11068页

核心收录:

学科分类:1002[医学-临床医学] 100202[医学-儿科学] 10[医学] 

基  金:National Institutes of Health, NIH National Institute of Diabetes and Digestive and Kidney Diseases, NIDDK: T32DK007664 

主  题:Biliary atresia Liver transplantation Pediatric liver disease Pediatric end-stage liver disease Kasai operation Newborn screening Surgical outcomes Living-related donor transplantation Split liver transplantation ABO-incompatible liver transplantation 

摘      要:Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.

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