Tomita Hirofumi, Shimojima Naoki, Sasaki Hideyuki, Shimotakahara Akihiro, Yamada Yohei, Kuroda Tatsuo, Nio Masaki, Hirobe Seiichi
Department of Surgery, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Aoba-ku, Sendai, Miyagi, Japan.
Ann Surg. 2024 Apr 1;279(4):692-698. doi: 10.1097/SLA.0000000000006075. Epub 2023 Aug 7.
To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis.
Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified.
The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity.
The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation.
Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx.
使用婴儿胆汁淤积性肝纤维化（iBALF）评分（一种基于标准血液分析的肝纤维化标志物）来识别胆汁引流手术预后极差的胆道闭锁（BA）患者。
尽管原发性肝移植开始被视为BA患者胆汁引流手术的替代方案，但最有可能从该手术中获益的患者尚未确定。
收集了2015年至2019年间60家参与研究医院的380例行胆汁引流手术的BA患者的病历进行回顾性分析。为预测1岁时的自体肝存活率，绘制了iBALF评分的受试者工作特征曲线。将临界值确定为灵敏度>99%的点。
手术时的中位年龄为56天（范围：4 - 183天），258例（67.9%）患者实现了1岁时的自体肝存活。选择iBALF评分为5.27作为临界值，发现18例（4.7%）患者的iBALF评分>5.27；其中，只有2例（95%CI：1.4% - 34.7%）在1岁时实现了自体肝存活，这表明其预后明显比其他患者差（95%CI：65.7% - 75.4%）。此外，iBALF评分>5.27的患者在挽救性肝移植时的死亡率显著更高且年龄更小。
术前iBALF评分>5.27的BA患者胆汁引流手术预后极差，可考虑作为原发性肝移植的候选者。
