Banc-Husu Anna M, Hall Matt, Thurm Cary, Whitehead Bridget A, Godown Justin A, Alonso Estella M
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.
Children's Hospital Association, Lenexa, Kansas, USA.
Liver Transpl. 2023 May 1;29(5):485-496. doi: 10.1097/LVT.0000000000000077. Epub 2023 Feb 3.
Acute-on-chronic liver failure (ACLF) occurs in children with biliary atresia (BA) awaiting liver transplantation (LT). However, data on transplant outcomes in ACLF are limited. Our aim was to characterize ACLF and determine its effect on transplant outcome and resource utilization. Using a linkage of the Scientific Registry of Transplant Recipients and Pediatric Health Information System, we identified children with BA between 3 months and 18 years at the time of listing who received a transplant from 2003 to 2018 and were hospitalized while waiting. ACLF was defined by the presence of at least 1 extra-hepatic organ failure during a pre-LT hospitalization. In all, 1044 patients (58% female, median age at listing 7.0 months IQR 5.0-14.0) were included. Thirty-four percent (351/1044) of the patients had at least 1 ACLF hospitalization. Patients with ACLF had longer waitlist times (114 [54-231] vs. 81 [35-181] days, p < 0.001), and were more likely to be listed as Status 1 (8% vs. 4%, p = 0.02). Pre-LT resource utilization was significantly higher in ACLF patients. There were no differences in mortality at 30 days (ACLF 3% vs. No ACLF 2%, p = 0.17), 90 days (ACLF 3% vs. No ACLF 2%, p = 0.24), 1 year (ACLF 3% vs. No ACLF 2%, p =0.23), 3 years (ACLF 4% vs. No ACLF 3%, p = 0.58), or 5 years (ACLF 5% vs. No ACLF 4%, p = 0.38) after LT. ACLF status was not associated with increased post-transplant mortality (adjusted HR 1.51, 95% CI 0.76-3.0, p =0.25). ACLF is an important morbidity in children with BA awaiting LT as it is associated with higher resource utilization and longer waitlist times. Further studies are needed to help understand the spectrum of ACLF and better prioritize critically ill children awaiting LT, as our study shows successful post-LT outcomes in children with BA and ACLF.
急性-on-慢性肝衰竭（ACLF）发生在等待肝移植（LT）的胆道闭锁（BA）儿童中。然而，关于ACLF移植结局的数据有限。我们的目的是描述ACLF的特征，并确定其对移植结局和资源利用的影响。通过将移植受者科学登记处和儿科健康信息系统相链接，我们确定了2003年至2018年期间在列入名单时年龄在3个月至18岁之间、接受移植且在等待期间住院的BA儿童。ACLF定义为在LT前住院期间至少出现1种肝外器官衰竭。总共纳入了1044例患者（58%为女性，列入名单时的中位年龄为7.0个月，四分位间距为5.0 - 14.0）。34%（351/1044）的患者至少有1次ACLF住院。ACLF患者的等待名单时间更长（114 [54 - 231]天对81 [35 - 181]天，p < 0.001），并且更有可能被列为1级状态（8%对4%，p = 0.02）。ACLF患者LT前的资源利用率显著更高。LT后30天（ACLF为3%对无ACLF为2%，p = 0.17）、90天（ACLF为3%对无ACLF为2%，p = 0.24）、1年（ACLF为3%对无ACLF为2%，p = 0.23）、3年（ACLF为4%对无ACLF为3%，p = 0.58）或5年（ACLF为5%对无ACLF为4%，p = 0.38）的死亡率无差异。ACLF状态与移植后死亡率增加无关（调整后的风险比为1.51，95%置信区间为0.76 - 3.0，p = 0.25）。ACLF是等待LT的BA儿童中的一种重要发病情况，因为它与更高的资源利用率和更长的等待名单时间相关。需要进一步研究以帮助了解ACLF的范围，并更好地对等待LT的重症儿童进行优先排序，因为我们的研究显示BA和ACLF儿童LT后有成功的结局。
