Leung Daniel H, Narang Amrita, Minard Charles G, Hiremath Girish, Goss John A, Shepherd Ross
Department of Pediatrics, Baylor College of Medicine, Houston, TX.
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Houston, TX.
Liver Transpl. 2016 Nov;22(11):1584-1592. doi: 10.1002/lt.24605.
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.
2岁以下患有慢性终末期肝病的幼儿(YC2)是等待肝移植的特别脆弱群体,其主要特征为胆道闭锁(BA)占多数。为了调查YC2的等待名单死亡率、相关风险因素及预后,我们评估了器官共享联合网络2003年4月至2013年3月登记的等待已故供体移植的YC2数据(BA = 994例;其他慢性肝病[CLD] = 221例)。总体而言,YC2的等待名单死亡率为12.4%,移植后死亡率为8%,总体登记后死亡率为19.6%。YC2在90天、180天和270天的等待名单死亡率分别为12.2%、18.7%和20.6%。与YC2中的BA相比,患有CLD的YC2等待名单死亡率显著更高(23.9%对9.8%;P < 0.05)。多因素分析显示,列出的儿童终末期肝病(PELD)评分>21(风险比[HR],3.2;95%置信区间[CI],1.6 - 6.5)、无例外情况(HR,5.8;95%CI,2.8 - 11.8)、列出时身高<60.6 cm(HR,2.1;95%CI,1.4 - 3.1)、列出时体重>10 kg(HR,3.8;95%CI,1.5 - 9.2)以及初始肌酐>0.5(HR,6.8;95%CI,3.4 - 13.5)是YC2等待名单死亡率的独立风险因素(所有P < 0.005)。对所有变量进行校正后,CLD患者的死亡风险比BA + 手术(推测为葛西手术)患者高2倍(95%CI,1.3 - 3.1)。此外,未接受手术的BA患者的死亡风险比推测接受葛西手术的BA患者高1.9倍(95%CI,1 - 3.4)。我们的数据突出显示了YC2中等待名单和肝移植后早期死亡率高得令人无法接受,而PELD评分无法预测这些情况,并提示了该年龄组中值得进一步研究的关键风险因素。《肝脏移植》22 1584 - 1592 2016美国肝脏病研究协会