Dong Hao, Liu Jing-Yi, Jiang Yi-Zhou, Sun Li-Ying, Wang You-Xin
Beijing Key Laboratory of Clinical Epidemiology, School of Public Health, Capital Medical University, Beijing 100069, China.
Department of Critical Liver Diseases, Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 101100, China.
J Clin Med. 2025 May 10;14(10):3328. doi: 10.3390/jcm14103328.
: Biliary atresia (BA) is the most common etiology for pediatric liver transplantation (LT). However, whether a previous Kasai hepatoportoenterostomy (KP) and its timing influence the outcomes of BA patients who undergo LT remains controversial. : Pediatric patients with BA who underwent LT at Beijing Friendship Hospital, Capital Medical University, between June 2013 and November 2022 were recruited. The patients were divided into non-KP, early-KP (before 90 days of life), and late-KP subgroups. The clinical data were compared among the groups. A nomogram to predict the 1-, 3-, and 5-year graft survival probabilities based on a multivariate Cox model was constructed and validated. : Among the 475 BA patients, the no-KP group accounted for 31.8%, the early KP for 60.4%, and the late KP for 7.8%, respectively. The incidences of LT complications were comparable among the groups. From the multivariate Cox analyses, an intensive care unit (ICU) stay and bleeding were identified as the independent risk factors for postoperative patient survival, and the LT type, graft type, vascular complications, and biliary complications were those for graft survival. A nomogram for graft survival was constructed, with a C-index of 0.82, and areas under the curves (AUCs) of 0.829, 0.824, and 0.824 for the 1-, 3-, and 5-year survival nomograms, respectively. The calibration and decision curve analysis (DCA) curves showed good discrimination ability and clinical applicability. A risk classification system was further developed, and the Kaplan-Meier curves demonstrated high discrimination between the high- and low-risk groups ( < 0.0001). : A previous KP has no impact on patients or graft survival after LT in BA patients. The established nomogram may be helpful for counseling BA patients about their clinical prognosis after LT.
胆管闭锁(BA)是儿童肝移植(LT)最常见的病因。然而,既往Kasai肝门肠吻合术(KP)及其时机是否会影响接受LT的BA患者的预后仍存在争议。招募了2013年6月至2022年11月期间在首都医科大学附属北京友谊医院接受LT的BA患儿。将患者分为非KP组、早期KP组(出生后90天内)和晚期KP组。比较各组的临床资料。基于多变量Cox模型构建并验证了用于预测1年、3年和5年移植物存活概率的列线图。在475例BA患者中,非KP组占31.8%,早期KP组占60.4%,晚期KP组占7.8%。各组LT并发症的发生率相当。多变量Cox分析显示,重症监护病房(ICU)住院时间和出血是术后患者存活的独立危险因素,而LT类型、移植物类型、血管并发症和胆道并发症是移植物存活的危险因素。构建了移植物存活列线图,C指数为0.82,1年、3年和5年存活列线图的曲线下面积(AUC)分别为0.829、0.824和0.824。校准和决策曲线分析(DCA)曲线显示出良好的区分能力和临床适用性。进一步建立了风险分类系统,Kaplan-Meier曲线显示高危组和低危组之间有高度区分度(<0.0001)。既往KP对BA患者LT后的患者或移植物存活没有影响。所建立的列线图可能有助于为BA患者提供LT后临床预后的咨询。