Functional status at listing predicts waitlist and posttransplant mortality in pediatric liver transplant candidates.

Functional impairment is associated with mortality in adult liver transplant candidates. This has not been studied in pediatric liver transplant candidates. United Network for Organ Sharing Standard Transplant Analysis and Research files were used to investigate functional status, waitlist mortality, and posttransplant outcomes in children younger than 18 years who were waitlisted in 2006-2016 for primary liver transplant. Functional status was categorized, by using the Lansky Play-Performance Scale (LPPS), as normal/good (80-100), moderately impaired (50-70), or severely impaired (10-40) by center assessment. Among 3250 children not listed as Status 1A, 62% had an LPPS score of 80-100, 25% had a score of 50-70, and 13% had a score of 10-40 at listing. Children with an LPPS score of 10-40 at listing were more likely to die while on the waitlist (standardized hazard ratio 1.85, 95% confidence interval 1.09-3.13, P = .02) in analyses adjusting for being on a ventilator, breathing support, or dialysis and other illness severity measures. For the 2565 children transplanted, an LPPS score of 10-40 at listing drastically increased mortality risk by 1 year posttransplant (hazard ratio 5.77, 95% confidence interval 3.05-10.91, P < .0005). LPPS scores of 10-40 and 50-70 both increased the risk of graft loss by 1 year. Functional status is an independent predictor of waitlist and posttransplant mortality in pediatric liver transplant candidates. Validated tools for the assessment of functional status in these children would improve our ability to predict mortality risk-and to appropriately prioritize them for transplant.

Perito ER ，Bucuvalas J ，Lai JC 《-》

Nonstandard Exception Requests Impact Outcomes for Pediatric Liver Transplant Candidates.

Nonstandard exceptions requests (NSERs), in which transplant centers appeal on a case-by-case basis for Pediatric End-Stage Liver Disease/Mayo End-Stage Liver Disease points, have been highly utilized for pediatric liver transplant candidates. We evaluated whether NSE outcomes are associated with waitlist and posttransplant mortality. United Network for Organ Sharing (UNOS) Scientific Registry of Transplant Recipients data on pediatric liver transplant candidates listed in 2009-2014 were analyzed after excluding those granted automatic UNOS exceptions. Of 2581 pediatric waitlist candidates, 44% had an NSE request. Of the 1134 children with NSERs, 93% were approved and 7% were denied. For children 2-18 years at listing, NSER denial increased the risk of waitlist mortality or removal for being too sick (subhazard ratio 2.99, 95% confidence interval [CI] 1.26-7.07, p = 0.01 in multivariate analysis). For children younger than 2 years, NSER denial did not impact waitlist mortality/removal. Children with NSER approved had reduced risk of graft loss 3 years posttransplant in univariate but not multivariable analysis (odds ratio 0.73, 95% CI 0.53-1.01, p = 06). Those with NSER denial had a higher risk of posttransplant death than those with no NSER (hazard ratio 2.43, 95% CI 0.99-5.95, p = 0.05, multivariable analysis), but NSER approval did not impact posttransplant death. Further research on NSER utilization in pediatric liver transplant is needed to optimize organ allocation and outcomes for children.

Braun HJ ，Perito ER ，Dodge JL ，Rhee S ，Roberts JP ... -  《-》

The decreasing predictive power of MELD in an era of changing etiology of liver disease.

The field of liver transplantation has shifted considerably in the MELD era, including changing allocation, immunosuppression, and liver failure etiologies, as well as better supportive therapies. Our aim was to evaluate the predictive accuracy of the MELD score over time. The United Network for Organ Sharing provided de-identified data on 120 156 patients listed for liver transplant from 2002-2016. The ability of the MELD score to predict 90-day mortality was evaluated by a concordance (C-) statistic and corroborated with competing risk analysis. The MELD score's concordance with 90-day mortality has downtrended from 0.80 in 2003 to 0.70 in 2015. While lab MELD scores at listing and transplant climbed in that interval, score at waitlist death remained steady near 35. Listing age increased from 50 to 54 years. HCV-positive status at listing dropped from 33 to 17%. The concordance of MELD and mortality does not differ with age (>60 = 0.73, <60 = 0.74), but is lower in diseases that are increasing most rapidly-alcoholic liver disease and non-alcoholic fatty liver disease-and higher in those that are declining, particularly in HCV-positive patients (HCV positive = 0.77; negative = 0.73). While MELD still predicts mortality, its accuracy has decreased; changing etiology of disease may contribute.

Godfrey EL ，Malik TH ，Lai JC ，Mindikoglu AL ，Galván NTN ，Cotton RT ，O'Mahony CA ，Goss JA ，Rana A ... -  《-》

Impact of the Pediatric End-Stage Liver Disease (PELD) growth failure thresholds on mortality among pediatric liver transplant candidates.

The Pediatric End-Stage Liver Disease (PELD) score is intended to determine priority for children awaiting liver transplantation. This study examines the impact of PELD's incorporation of "growth failure" as a threshold variable, defined as having weight or height <2 standard deviations below the age and gender norm (z-score <2). First, we demonstrate the "growth failure gap" created by PELD's current calculation methods, in which children have z-scores <2 but do not meet PELD's growth failure criteria and thus lose 6-7 PELD points. Second, we utilized United Network for Organ Sharing (UNOS) data to investigate the impact of this "growth failure gap." Among 3291 pediatric liver transplant candidates, 26% met PELD-defined growth failure, and 17% fell in the growth failure gap. Children in the growth failure gap had a higher risk of waitlist mortality than those without growth failure (adjusted subhazard ratio [SHR] 1.78, 95% confidence interval [95% CI] 1.05-3.02, P = .03). They also had a higher risk of posttransplant mortality (adjusted HR 1.55, 95% CI 1.03-2.32, P = .03). For children without PELD exception points (n = 1291), waitlist mortality risk nearly tripled for those in the gap (SHR 2.89, 95% CI 1.39-6.01, P = .005). Current methods for determining growth failure in PELD disadvantage candidates arbitrarily and increase their waitlist mortality risk. PELD should be revised to correct this disparity.

Swenson SM ，Roberts JP ，Rhee S ，Perito ER ... -  《-》

Functional status predicts pediatric heart transplant outcomes: A united network for organ sharing (UNOS) database study.

Functional status predicts waitlist survival in adult heart transplantation and is an independent predictor of outcomes in pediatric liver transplantation. This has not been studied in pediatric heart transplantation. Study aims were to determine the association of: (1) functional status at listing with waitlist and post-transplant outcomes, and (2) functional status at transplant with post-transplant outcomes in pediatric heart transplantation. Retrospective United Network of Organ Sharing database study of pediatric patients listed for heart transplant between 2005 and 2019 with Lansky Play Performance Scale (LPPS) scores at listing. Standard statistical methods were used to assess relationships between LPPS and outcomes (waitlist and post-transplant). Negative waitlist outcome was defined as death or removal from waitlist due to clinical deterioration. There were 4,169 patients identified, including 1,080 with LPPS 80-100 (normal activity), 1,603 with LPPS 50-70 (mild limitations), and 1,486 with LPPS 10-40 (severe limitations). LPPS 10-40 correlated with negative waitlist outcomes (HR 1.69, CI 1.59-1.80, p < 0.0001). While LLPS at listing had no association with post-transplant survival, those with LPPS 10-40 at transplant had inferior 1-year post-transplant survival compared to those with LPPS ≥50 (92% vs 95%-96%, p = 0.0011). Functional status was an independent predictor of post-transplant outcomes in patients with cardiomyopathy. A functional improvement of ≥20 points between listing and transplant (N = 770, 24%) was associated with higher 1-year post-transplant survival (HR 1.63, 95% CI: 1.10-2.41, p = 0.018). Functional status is associated with waitlist and post-transplant outcomes. Interventions targeting functional impairment may improve pediatric heart transplantation outcomes.

Khan RS ，Khoury PR ，Zafar F ，Morales DL ，Chin C ，Peng DM ，Almond CS ，Burstein DS ，Odeniyi F ，Wittekind SG ... -  《-》