Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant.
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.
['Tessier|Mary Elizabeth M|ME|', 'Harpavat|Sanjiv|S|', 'Shepherd|Ross W|RW|', 'Hiremath|Girish S|GS|', 'Brandt|Mary L|ML|', 'Fisher|Amy|A|', 'Goss|John A|JA|']
D000367:Age Factors D001656:Biliary Atresia D003661:Decision Support Techniques D058625:End Stage Liver Disease D006801:Humans D007223:Infant D016031:Liver Transplantation D011171:Portoenterostomy, Hepatic D012307:Risk Factors D061665:Time-to-Treatment D014019:Tissue Donors D016896:Treatment Outcome D014850:Waiting Lists
Pediatric end-stage liver disease Biliary atresia Liver transplantation Kasai operation ABO-incompatible liver transplantation Living-related donor transplantation Newborn screening Pediatric liver disease Split liver transplantation Surgical outcomes