Anderson Linda, Hukkinen Maria, Nyholm Iiris, Niemi Mikko, Pakarinen Mikko P
Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Pediatric Research Center, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
J Pediatr Gastroenterol Nutr. 2025 Mar;80(3):462-470. doi: 10.1002/jpn3.12451. Epub 2024 Dec 29.
To compare the predictive value of serum bile acids on native liver survival (NLS) and portal hypertension (PH) at various time points early after portoenterostomy (PE) in biliary atresia (BA).
This was a retrospective observational study. Serum bilirubin and bile acid concentrations were defined by enzymatic spectrophotometry 1, 3, and 6 months after PE. After defining optimal bilirubin and bile acids cutoffs by area under the receiver operating characteristic (AUROC) curves, cutoffs were compared with other predictors of NLS and PH in Cox regression.
Out of 56 patients, 42 (75%) achieved clearance of jaundice (COJ, bilirubin <20 µmol/L at 6 months). Both bilirubin and bile acids at 3 and 6 months were accurate predictors of NLS among all patients (AUROC 0.82-0.91, p < 0.001). In COJ patients, bile acids (AUROC 0.82, p = 0.003), but not bilirubin, at 1 month also predicted NLS. Among all patients, the strongest predictors of NLS were bilirubin >18.5 µmol/L and bile acids >150 µmol/L at 3 months, increasing the risk of transplantation/death seven- and eightfold, respectively (p < 0.001 for both). In COJ patients, the strongest predictor of NLS was bile acids >119 µmol/L at 3 months, increasing the risk of transplantation/death 12-fold (p = 0.014). Bile acids and bilirubin at 3 and 6 months predicted PH development in COJ patients with moderate accuracy (AUROC 0.72-0.78, p = 0.004-0.019). Bilirubin >8.5 µmol/L and bile acids >78 µmol/L at 6 months increased PH risk 13-fold (p < 0.001) and 4-fold (p = 0.006).
Serum bile acids offer a simple and useful additional tool to predict PE outcomes in BA, particularly after COJ.
比较胆汁闭锁（BA）患者行门肠吻合术（PE）后早期不同时间点血清胆汁酸对自体肝生存（NLS）和门静脉高压（PH）的预测价值。
这是一项回顾性观察研究。通过酶分光光度法测定PE术后1、3和6个月时的血清胆红素和胆汁酸浓度。通过受试者操作特征曲线下面积（AUROC）确定最佳胆红素和胆汁酸临界值后，在Cox回归分析中，将这些临界值与NLS和PH的其他预测指标进行比较。
56例患者中，42例（75%）实现黄疸清除（COJ，6个月时胆红素<20µmol/L）。在所有患者中，3个月和6个月时的胆红素和胆汁酸都是NLS的准确预测指标（AUROC 0.82 - 0.91，p<0.001）。在COJ患者中，1个月时的胆汁酸（AUROC 0.82，p = 0.003）而非胆红素也可预测NLS。在所有患者中，3个月时胆红素>18.5µmol/L和胆汁酸>150µmol/L是NLS的最强预测指标，分别使移植/死亡风险增加7倍和8倍（两者p均<0.001）。在COJ患者中，3个月时胆汁酸>119µmol/L是NLS的最强预测指标，使移植/死亡风险增加12倍（p = 0.014）。3个月和6个月时的胆汁酸和胆红素对COJ患者PH的发生有中等准确性的预测作用（AUROC 0.72 - 0.78，p = 0.004 - 0.019）。6个月时胆红素>8.5µmol/L和胆汁酸>78µmol/L使PH风险分别增加13倍（p<0.001）和4倍（p = 0.006）。
血清胆汁酸为预测BA患者PE术后结局提供了一种简单且有用的辅助工具，尤其是在COJ之后。
