Abstract：
BACKGROUND: Acute-on-chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse.
METHODS: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra-hepatic organ failure in children with decompensated cirrhosis.
RESULTS: Of 107 patients (65% female; median age 14 [9-31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4-8] vs. 9 [6-24] months; p < .001) and at LT (8 [8-11] vs. 16 [10-40] months, p < .001) compared to no-ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20-53] vs. 10 [6-19] mL/kg; p < .001) and blood transfusion requirements (33 [21-69] vs. 18 [7-25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45-151] vs. 13 [7-30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1-year post-LT survival rates were comparable between the groups.
CONCLUSIONS: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT.
METHODS: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra-hepatic organ failure in children with decompensated cirrhosis.
RESULTS: Of 107 patients (65% female; median age 14 [9-31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4-8] vs. 9 [6-24] months; p < .001) and at LT (8 [8-11] vs. 16 [10-40] months, p < .001) compared to no-ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20-53] vs. 10 [6-19] mL/kg; p < .001) and blood transfusion requirements (33 [21-69] vs. 18 [7-25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45-151] vs. 13 [7-30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1-year post-LT survival rates were comparable between the groups.
CONCLUSIONS: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT.
摘要：
背景：慢性急性肝衰竭（ACLF）与胆道闭锁（BA）患者死亡率和发病率增加相关。ACLF对术后结局影响的数据，然而,是稀疏的。
方法：我们对2011年至2021年在我们机构接受LT的年龄<18岁的BA患者进行了回顾性分析。使用小儿ACLF标准定义ACLF：失代偿性肝硬化患儿的肝外器官衰竭≥1。
结果：在接受LT的107例患者中（65％为女性；中位年龄14[9-31]个月），13人（12%）在LT之前的索引入院期间患有ACLF。2名(15%)为1级;4名(30%)为2级;7名(55%)为≥3级ACLF。ACLF队列在上市时更年轻（5[4-8]vs.9[6-24]个月；p<.001）和LT（8[8-11]vs.16[10-40]个月，p<.001）与非ACLF组相比。术中,ACLF患者失血量较高（40[20-53]vs.10[6-19]mL/kg；p<.001）和输血要求（33[21-69]vs.18[7-25]mL/kg;p=.004)。术后,他们需要更高的血管加压药支持(31%vs.10.6%；p=.04），总住院时间较高（106[45-151]vs.13[7-30]天；p=.023）。手术室的回报率，医院再入院率,两组间LT术后1年生存率相当.
结论：尽管围手术期并发症较高，LT后BA中ACLF的生存结局是有利的，与没有ACLF的生存结局相当。这些令人鼓舞的数据重申了这些重症患儿在LT器官分配过程中的优先次序。
方法：我们对2011年至2021年在我们机构接受LT的年龄<18岁的BA患者进行了回顾性分析。使用小儿ACLF标准定义ACLF：失代偿性肝硬化患儿的肝外器官衰竭≥1。
结果：在接受LT的107例患者中（65％为女性；中位年龄14[9-31]个月），13人（12%）在LT之前的索引入院期间患有ACLF。2名(15%)为1级;4名(30%)为2级;7名(55%)为≥3级ACLF。ACLF队列在上市时更年轻（5[4-8]vs.9[6-24]个月；p<.001）和LT（8[8-11]vs.16[10-40]个月，p<.001）与非ACLF组相比。术中,ACLF患者失血量较高（40[20-53]vs.10[6-19]mL/kg；p<.001）和输血要求（33[21-69]vs.18[7-25]mL/kg;p=.004)。术后,他们需要更高的血管加压药支持(31%vs.10.6%；p=.04），总住院时间较高（106[45-151]vs.13[7-30]天；p=.023）。手术室的回报率，医院再入院率,两组间LT术后1年生存率相当.
结论：尽管围手术期并发症较高，LT后BA中ACLF的生存结局是有利的，与没有ACLF的生存结局相当。这些令人鼓舞的数据重申了这些重症患儿在LT器官分配过程中的优先次序。
