A 10-Year united network for organ sharing review of mortality and risk factors in young children awaiting liver transplantation.

Young children < 2 years of age with chronic end-stage liver disease (YC2) are a uniquely vulnerable group listed for liver transplantation, characterized by a predominance of biliary atresia (BA). To investigate wait-list mortality, associated risk factors, and outcomes of YC2, we evaluated United Network for Organ Sharing registry data from April 2003 to March 2013 for YC2 listed for deceased donor transplant (BA = 994; other chronic liver disease [CLD] = 221). Overall, wait-list mortality among YC2 was 12.4% and posttransplant mortality was 8%, accounting for an overall postlisting mortality of 19.6%. YC2 demonstrated 12.2%, 18.7%, and 20.6% wait-list mortality by 90, 180, and 270 days, respectively. YC2 with CLD demonstrated significantly higher wait-list mortality compared with BA among YC2 (23.9% versus 9.8%; P < 0.05). Multivariate analyses revealed that listing Pediatric End-Stage Liver Disease [PELD] > 21 (hazard ratio [HR], 3.2; 95% confidence interval [CI], 1.6-6.5), lack of exception (HR, 5.8; 95% CI, 2.8-11.8), listing height < 60.6 cm (HR, 2.1; 95% CI, 1.4-3.1), listing weight  > 10 kg (HR, 3.8; 95% CI, 1.5-9.2), and initial creatinine > 0.5 (HR, 6.8; 95% CI, 3.4-13.5) were independent risk factors for YC2 wait-list mortality (P < 0.005 for all). Adjusting for all variables, the risk of death among CLD patients was 2 (95% CI, 1.3-3.1) times greater than patients with BA + surgery (presumed Kasai). Furthermore, the risk of death in BA without surgery was 1.9 (95% CI, 1‐3.4) times greater than BA with presumed Kasai. Our data highlight unacceptably high wait-list and early post-liver transplant mortality in YC2 not predicted by PELD and suggest key risk factors deserving of further study in this age group. Liver Transplantation 22 1584-1592 2016 AASLD.

Leung DH ，Narang A ，Minard CG ，Hiremath G ，Goss JA ，Shepherd R ... -  《-》

Wait-list mortality of young patients with Biliary atresia: Competing risk analysis of a eurotransplant registry-based cohort.

Liver transplantation (LT) is the standard treatment for biliary atresia (BA) patients with end-stage liver disease. The prognosis after LT has steadily improved, but overall prognosis of BA patients is also determined by mortality before LT. We aimed to quantify mortality in young BA patients on the Eurotransplant waiting list and to determine the effect of disease severity and age at time of listing on pretransplant mortality. We used a cohort study design, which incorporated data from the Eurotransplant registry. Participants were 711 BA patients who were below 5 years of age from 5 countries and listed for LT between 2001 and 2014. We applied a competing risk analysis to evaluate simultaneously the outcomes death, LT, and still waiting for a suitable organ. We used Cox proportional hazards regression to assess 2-year mortality. In a subcohort of 416 children, we performed multivariate analyses between 2-year mortality and disease severity or age, each at listing. Disease severity at listing was quantified by the Model for End-Stage Liver Disease (MELD) score, which assesses bilirubin, creatinine, albumin, and international normalized ratio as continuous variables. Two-year wait-list mortality was 7.9%. Age below 6 months and MELD score above 20 points, each at listing, were strongly and independently associated with 2-year mortality (each P < 0.001). A total of 21% of infants who fulfilled both criteria did not survive the first 6 months on the waiting list. In conclusion, our findings quantify mortality among young BA patients on the waiting list and the relative importance of risk factors (age and severity of disease at listing). Our results provide both an evidence base to rationally address high mortality in subgroups and a methodology to assess effects of implemented changes, for example, in allocation rules. Liver Transplantation 24 810-819 2018 AASLD.

van der Doef HPJ ，van Rheenen PF ，van Rosmalen M ，Rogiers X ，Verkade HJ ，for pediatric liver transplantation centers of Eurotransplant ... -  《-》

Variability in acceptance of organ offers by pediatric transplant centers and its impact on wait-list mortality.

Recent data have suggested that pediatric patients wait-listed for a liver transplantation frequently have liver offers declined. However, factors associated with liver offer decisions and center-level variability in practice patterns have not been explored. We evaluated United Network for Organ Sharing data on all match runs from May 1, 2007 to December 31, 2015 in which the liver was offered to ≥1 pediatric patient; the transplant recipient was ranked in the first 40 positions for the organ offer; and the donor was brain-dead and <50 years of age. We used multilevel mixed effects models to evaluate factors associated with organ offer acceptance, among-center variability, and the association between center-level acceptance and wait-list mortality. There were 4088 unique pediatric patients during the study period, comprising 27,094 match runs. Initial Model for End-Stage Liver Disease or Pediatric End-Stage Liver Disease score, history of exception points, recipient region, rank on match run, and geographic share type were all associated with probability of offer acceptance. There was significant among-center variation (P < 0.001) in adjusted liver offer acceptance rates, accounting for donor, recipient, and match-related factors (adjusted acceptance rates: median, 8.9%; range, 5.1%-14.6%). Center-level acceptance rates were associated with wait-list mortality, with a >10% increase in the risk of wait-list mortality for every 1% decrease in a center's adjusted liver offer acceptance rate (odds ratio, 1.10; 95% confidence interval, 1.01-1.19). In conclusion, there is significant among-center variability in liver offer acceptance rates for pediatric patients that is not explained by donor and recipient factors. A center's liver acceptance behavior significantly impacts whether a pediatric patient will be transplanted or die on the waiting list. Liver Transplantation 24 803-809 2018 AASLD.

Mitchell E ，Loomes KM ，Squires RH ，Goldberg D ... -  《-》

Living donor liver transplantation for post-Kasai biliary atresia: Analysis of pretransplant predictors of outcomes in infants.

After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into 3 groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants. Outcome was relatively worse in infants than older children, with overall survival at 1 and 5 years of 94.5% and 93.2%, respectively, and graft survival at 1 and 5 years of 91.1% each. Incidence of vascular complications was not significantly higher in infants. High Pediatric End-Stage Liver Disease (PELD) score (odds ratio [OR], 3.72; 95% confidence interval [CI], 1.30-10.67; P = 0.02) and portal vein (PV) hypoplasia (OR, 3.23; 95% CI, 1.10-9.52; P = 0.03) were independent risk factors for morbidity. Low weight-for-age z score (hazard ratio, 5.76; 95% CI, 1.05-31.47; P = 0.03) was identified as a significant risk factor for mortality after LDLT, but not age or absolute body weight (BW). Infants with BW deficit had a significantly smaller PV diameter (P = 0.005), greater blood loss (P = 0.001), and higher incidence of postoperative bacteremia (P = 0.01). In conclusion, high PELD score and PV hypoplasia were independent risk factors for morbidity, and BW deficit was associated with poor survival in infants with post-Kasai BA after LDLT. However, LDLT in these infants at the earliest possible time after referral is a feasible option with excellent patient survival in an experienced center. Liver Transplantation 23 1199-1209 2017 AASLD.

Kitajima T ，Sakamoto S ，Sasaki K ，Uchida H ，Narumoto S ，Fukuda A ，Teramukai S ，Uemoto S ，Kasahara M ... -  《-》

Outcomes for liver transplant candidates listed with low model for end-stage liver disease score.

Kwong AJ ，Lai JC ，Dodge JL ，Roberts JP ... -  《-》