Objectives To determine clinical and health-related quality of life outcomes, and to derive an “ideal” composite profile of children alive 10 years after pediatric liver transplantation (LT) performed in the US and Canada. Study design This was a multicenter cross-sectional analysis characterizing patients enrolled in the Studies of Pediatric Liver Transplantation database registry who have survived >10 years from LT. Results A total of 167 10-year survivors were identified, all of whom received daily immunosuppression therapy. Comorbidities associated with the post-LT course included post-transplantation lymphoproliferative disease (in 5% of patients), renal dysfunction (9%), and impaired linear growth (23%). Health-related quality of life, as assessed by the PedsQL 4.0 Generic Core Scales, revealed lower patient self-reported total scale scores for 10-year survivors compared with matched healthy children (77.2 ± 12.9 vs 84.9 ± 11.7; P < .001). At 10 years post-LT, only 32% of patients achieved an ideal profile of a first allograft stable on immunosuppression monotherapy, normal growth, and absence of common immunosuppression-induced sequelae. Conclusion Success after pediatric LT has moved beyond patient survival. Availability of an ideal composite profile at follow-up provides opportunities for patients, families, and healthcare providers to identify broader sets of outcomes at earlier stages, ultimately contributing to improved outcomes after pediatric LT.

A model using five objective parameters can accurately predict death or death-moved to ICU in children awaiting liver transplantation.

Nonadherence to immunosuppressant medications is a leading cause of poor long-term outcomes in transplant recipients. The Medication Level Variability Index (MLVI) provides a vehicle for transplant outcome risk-stratification through continuous assessment of adherence. The MALT (Medication Adherence in children who had a Liver Transplant) prospective multi-site study evaluated whether MLVI predicts Late Acute Rejection (LAR). 400 pediatric (1–17 year old) liver transplant recipients were enrolled and followed for 2 years. The a-priori hypothesis was that a higher MLVI predicts LAR. Pre-defined secondary analyses evaluated other outcomes such as liver enzyme levels, and sensitivity analyses compared adolescent to pre-adolescents. In the primary analysis sample of 379 participants, a higher pre-rejection MLVI predicted LAR [mean pre-rejection MLVI with LAR: 2.4 (3.6 SD) vs. without LAR, 1.6 (1.1); p=0.026]. 53% of the adolescents with MLVI>2 in year 1 had LAR by the end of year 2, as compared with 6% of those with year 1 MLVI≤2. A higher MLVI was significantly associated with all secondary outcomes. MLVI, a marker of medication adherence that uses clinically-derived information, predicts LAR in pediatric liver transplant recipients.

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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.

Studies of Pediatric Liver Transplantation (SPLIT) is a cooperative research network comprising 38 pediatric liver transplant centers in North America. Data from the 1092 patients who have received a first liver transplant since 1995 were analyzed for factors influencing patient survival, graft survival and acute rejection. The 3, 12, 24 and 36 month Kaplan-Meier estimates of patient/graft survival were 90.9/85.5, 86.3/80.2, 84.3/76.0, and 83.8/75.3% respectively. Univariate analysis identified initial diagnosis, type of graft (whole vs. living and cadaveric technical variant), growth failure and continuous hospitalization or ICU admission prior to transplantation as significantly influencing patient and graft survival. Subsequent multivariate analysis identified as risk factors for death: fulminant liver failure (RR = 3.05, p < 0.05), cadaveric technical variant grafts (RR = 1.95, p < 0.05), continuous hospitalization pre-transplant (RR = 1.79, p < 0.05), height deficit >2 s.d. from mean (RR = 3.22, p < 0.05). Risk factors for graft loss included: fulminant liver failure (RR = 2.27, p < 0.05), cadaveric technical variant grafts, (RR = 1.97, p < 0.05). Eleven percent of the 1092 patients were re-transplanted; vascular complications, particularly hepatic artery thrombosis (8.3% overall; 36.3% of graft failures), were responsible for the majority of re-transplants. Infection was the single most important cause of death (40 of 141, 28.4%) and was a contributing cause in 55 (39%), particularly with bacterial or fungal organisms. The cumulative Kaplan-Meier estimates of first rejection at 3, 12, 24 and 36 months were 44.8, 52.9, 59.1, and 60.3%. Initial immunosuppression with tacrolimus reduced the probability of rejection (RR = 0.62, p < 0.05). Eleven percent of rejections were steroid-resistant; chronic rejection led to 7 of 121 (5.8%) re-transplants. The SPLIT registry, in compiling data from a large number of centers, reflects the current outcomes for pediatric liver transplants in North America.

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We conclude that the most significant factors predicting patient and graft loss at 6 months in children listed for transplant are posttransplant surgical complications.