From the Department of Radiology and Research Institute of Radiology (J.R.K., H.M.Y., A.Y.J., J.S.L., Y.A.C.), Department of Nuclear Medicine (J.S.K.), Department of Pediatric Surgery (J.M.N., D.Y.K.), and Department of Pediatrics (S.H.O., K.M.K.), Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, South Korea; and Department of Radiology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan-si, Gyeongsangnam-do, South Korea (J.Y.H.).
Radiology. 2018 Jul;288(1):262-269. doi: 10.1148/radiol.2018172390. Epub 2018 Apr 10.
Purpose To develop and validate a scoring system based on clinical and imaging features to predict the risk for biliary atresia in patients with neonatal cholestasis. Materials and Methods Patients with neonatal cholestasis who underwent both ultrasonography (US) and hepatobiliary scintigraphy (n = 480) were retrospectively identified from two tertiary referral hospitals from January 2000 to February 2017. Patients from one hospital were classified as the derivation cohort (n = 371), and those from the other hospital were classified as the validation cohort (n = 109). Clinical and imaging features associated with biliary atresia were assessed. Histopathologic or intraoperative cholangiographic findings served as the reference standard for biliary atresia. A prediction model was developed by using logistic regression and was then transformed into a scoring system. The scoring system was internally and externally validated. Results Among the 371 patients in the derivation cohort, 97 (26.15%) had biliary atresia. A scoring system was constructed with the following variables: full-term birth, presence of the triangular cord sign at US, abnormal gallbladder morphology at US, and failure of radioisotope excretion to the small bowel at hepatobiliary scintigraphy. The maximum possible score with this system is 7 points. This system enabled differentiation of biliary atresia in the derivation cohort (C statistic, 0.981; 95% confidence interval [CI]: 0.970, 0.992) and the validation cohort (C statistic, 0.995; 95% CI: 0.987, 1.000). The risk score also showed good calibration in both the derivation and the validation cohorts (P = .328 and P = .281, respectively). Conclusion A simple scoring system combining clinical and imaging features can help accurately estimate the risk for biliary atresia in patients with neonatal cholestasis.
基于临床和影像学特征开发并验证一种评分系统，以预测新生儿胆汁淤积患者发生胆道闭锁的风险。
本研究回顾性分析了 2000 年 1 月至 2017 年 2 月期间来自两家三级转诊医院的 480 例接受超声（US）和肝胆闪烁显像（HBS）检查的新生儿胆汁淤积患者的临床资料。其中，一家医院的患者（n = 371）被归入推导队列，另一家医院的患者（n = 109）被归入验证队列。评估了与胆道闭锁相关的临床和影像学特征。胆道闭锁的参考标准为组织病理学或术中胆管造影结果。采用逻辑回归法建立预测模型，并将其转化为评分系统。该评分系统分别在推导队列和验证队列中进行了内部和外部验证。
在推导队列的 371 例患者中，有 97 例（26.15%）患有胆道闭锁。该评分系统由以下变量构建而成：足月出生、US 上存在三角索带征、US 上胆囊形态异常、HBS 示放射性核素排泄至小肠失败。该系统的最高得分为 7 分。该系统能够区分推导队列（C 统计量为 0.981，95%置信区间 [CI]：0.9700.992）和验证队列（C 统计量为 0.995，95%CI：0.9871.000）中的胆道闭锁。在推导队列和验证队列中，该风险评分均具有良好的校准度（P =.328 和 P =.281）。
一种结合临床和影像学特征的简单评分系统可帮助准确评估新生儿胆汁淤积患者发生胆道闭锁的风险。
