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

作者：Tessier Mary Elizabeth M; Harpavat Sanjiv; Shepherd Ross W; Hiremath Girish S; Brandt Mary L; Fisher Amy; Goss John A^*

来源：World Journal of Gastroenterology, 2014, 20(32): 11062-11068.

DOI：10.3748/wjg.v20.i32.11062

• 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

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.

• 出版日期2014-8-28