Published online December 1, 2008
PEDIATRICS Vol. 122 No. 6 December 2008, pp. e1128-e1135 (doi:10.1542/10.1542/peds.2008-1363)
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ARTICLE

Outcomes of 5-Year Survivors of Pediatric Liver Transplantation: Report on 461 Children From a North American Multicenter Registry

Vicky Lee Ng, MD, FRCPCa,b, Annie Fecteau, MD, FRCSCa,c, Ross Shepherd, MDd,e, John Magee, MDf, John Bucuvalas, MDg, Estella Alonso, MDh, Suzanne McDiarmid, MDi, Geoff Cohen, PhDj, Ravinder Anand, PhDj and the Studies of Pediatric Liver Transplantation Research Group

c SickKids Transplant Center
a Division of Paediatric Gastroenterology, Hepatology, and Nutrition
b Division of Paediatric General Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
d Washington University School of Medicine, St Louis, Missouri
e St Louis Children's Hospital, St Louis, Missouri
f Division of Transplant Surgery, University of Michigan, Ann Arbor, Michigan
g Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
h Siragusa Transplant Center, Children's Memorial Hospital, Chicago, Illinois
i Dumont-University of California Los Angeles Liver Transplant Center, Los Angeles, California
j Studies of Pediatric Liver Transplantation, EMMES Corporation, Rockville, Maryland

OBJECTIVES. Although liver transplantation has been the standard of care therapy for life-threatening liver diseases for >20 years, data on the long-term impact of liver transplantation in children have been primarily limited to single-center experiences. The objective of this study was to characterize and evaluate the clinical course of children who have survived ≥5 years after pediatric liver transplantation in multiple centers across North America.

PATIENTS AND METHODS. Patients enrolled in the Studies of Pediatric Liver Transplantation database registry who had undergone liver transplantation at 1 of 45 pediatric centers between 1996 and 2001 and survived >5 years from liver transplantation were identified and their clinical courses retrospectively reviewed.

RESULTS. The first graft survival for 461 five-year survivors was 88%, with 55 (12%) and 10 (2%) children undergoing a second and third liver transplantation. At the 5-year anniversary clinic visit, liver function was preserved in the majority with daily use of immunosuppression therapy, including a calcineurin inhibitor and oral prednisone, reported by 97% and 25% of children, respectively. The probability of an episode of acute cellular rejection occurring within 5 years after liver transplantation was 60%. Chronic rejection occurred in 5% patients. Posttransplant lymphoproliferative disease was diagnosed in 6% children. Calculated glomerular filtration rate was <90 mL/minute per 1.73 m2 in 13% of 5-year survivors. Age- and gender-adjusted BMI >95th percentile was noted in 12%, with height below the 10th percentile in 29%.

CONCLUSIONS. Children who are 5-year survivors of liver transplantation have good graft function, but chronic medical conditions and posttransplantation complications affect extrahepatic organs. A comprehensive approach to the management of these patients' multiple unique needs requires the expertise and commitment of health care providers both beyond and within transplant centers to further optimize long-term outcomes for pediatric liver transplant recipients.

Key Words: liver transplantation • long-term outcome • renal function • school performance

Abbreviations: LT—liver transplantation • SPLIT—Studies of Pediatric Liver Transplantation • PTLD—posttransplant lymphoproliferative disease • EBV—Epstein-Barr virus • cGFR—calculated glomerular filtration rate • ADHD—attention-deficit/hyperactivity disorder • LD—learning disability

Accepted Aug 4, 2008.


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