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World J Gastroenterol. Aug 28, 2014; 20(32): 11062-11068
Published online Aug 28, 2014. doi: 10.3748/wjg.v20.i32.11062
Beyond the Pediatric end-stage liver disease system: Solutions for infants with biliary atresia requiring liver transplant
Mary Elizabeth M Tessier, Sanjiv Harpavat, Ross W Shepherd, Girish S Hiremath, Mary L Brandt, Amy Fisher, John A Goss
Mary Elizabeth M Tessier, Sanjiv Harpavat, Ross W Shepherd, Girish S Hiremath, Department of Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX 77030, United States
Mary L Brandt, Amy Fisher, John A Goss, Department of Surgery, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX 77030, United States
Author contributions: Tessier MEM researched, analyzed, and wrote the initial draft; Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, and Goss JA researched, analyzed, and edited the paper.
Correspondence to: John A Goss, MD, Department of Surgery, Baylor College of Medicine and Texas Children’s Hospital, 6620 Main Street, Suite 1450, Houston, TX 77030, United States. jgoss@bcm.tmc.edu
Telephone: +1-832-3551400 Fax: +1-713-6102482
Received: February 13, 2014
Revised: May 13, 2014
Accepted: June 21, 2014
Published online: August 28, 2014
Abstract

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.

Keywords: 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

Core tip: Infants with biliary atresia (BA) can benefit by maximizing the Kasai operation’s success, through diagnosing the disease earlier, employing experienced surgeons, ensuring adequate nutrition, and administering certain medications. If they require a liver transplant despite these interventions, infants with BA can also benefit by expediting the transplant process, through the use of living-related donor, split or ABO-incompatible liver transplants.