Wilson’s disease (WD) is a rare cause of acute liver failure (ALF) and has a high fatality rate. Rapid and accurate diagnosis is important for ALF because of WD (ALF-WD). Our objective was to establish a simple, rapid, and accurate diagnostic test to distinguish ALF-WD from non-WD ALF (NWDALF) in children.

The data from all cases with pediatric ALF were retrospectively collected and analyzed. We performed receiver operator characteristics curve (ROC) analysis and confirmed the optimum cut-off points.

Fifty-eight patients with pediatric ALF (12 with WD, 46 with other etiologies) were included. Older age was observed in ALF-WD compared to NWDALF (11.16 ± 2.51 years vs. 3.34 ± 3.81 years, p < 0.001). An analysis based on routine biochemical testings revealed that total bilirubin (TBil), direct bilirubin, indirect bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), AST:ALT ratio, alkaline phosphatase (ALP), ALP:TBil ratio, serum albumin, gamma-glutamyl transferase, cholinesterase, hemoglobin, and platelet were statistically significant between the ALF-WD and NWDALF groups. The optimum cut-off points were obtained through ROC analysis. A scoring system was formed by assigning a score of 1 or 0 to patients who met the 13 cut-off points. Using ROC analysis, we determined a cut-off point of ≥ 6.5 for ALF-WD with 91.7% sensitivity and 97.8% specificity (p < 0.0001). In addition, a best cut-off point of ≥ 1.5 based on only five variables (ALT, AST, AST:ALT ratio, ALP, and ALP:TBil ratio), had 100% sensitivity and 91.3% specificity for ALF-WD (p < 0.0001). Based on this, when age was calculated as the sixth indicator, the best cut-off value of ≥ 2.5 had 100% sensitivity and 97.8% specificity (p < 00.0001).

Our study developed a new scoring system that consists of simple laboratory tests with good sensitivity and specificity and can be used by clinicians to quickly distinguish ALF-WD from NWDALF in children.

威尔逊氏病 (WD) 是急性肝功能衰竭 (ALF) 的罕见病因，死亡率很高。由于WD（ALF-WD），快速准确的诊断对于ALF很重要。我们的目标是建立一种简单、快速和准确的诊断测试，以区分儿童的 ALF-WD 和非 WD ALF (NWDALF)。

回顾性收集和分析所有小儿 ALF 病例的数据。我们进行了接受者操作员特征曲线 (ROC) 分析并确认了最佳截止点。

包括 58 名儿童 ALF 患者（12 名 WD，46 名其他病因）。与 NWDALF 相比，ALF-WD 的年龄更大（11.16 ± 2.51 岁）对比3.34 ± 3.81 岁，p< 0.001)。基于常规生化检测的分析显示，总胆红素 (TBil)、直接胆红素、间接胆红素、丙氨酸氨基转移酶 (ALT)、天冬氨酸氨基转移酶 (AST)、AST:ALT 比值、碱性磷酸酶 (ALP)、ALP:TBil 比值、血清白蛋白、γ-谷氨酰转移酶、胆碱酯酶、血红蛋白和血小板在 ALF-WD 和 NWDALF 组之间具有统计学意义。通过ROC分析获得最佳截止点。通过为满足 13 个分界点的患者分配 1 或 0 分来形成评分系统。使用 ROC 分析，我们确定 ALF-WD 的截止点为 ≥ 6.5，灵敏度为 91.7%，特异性为 97.8%（p< 0.0001)。此外，仅基于五个变量（ALT、AST、AST:ALT 比值、ALP 和 ALP:TBil 比值）的 ≥ 1.5 的最佳截止点对 ALF-WD 具有 100% 的敏感性和 91.3% 的特异性（p< 0.0001)。以此为基础，以年龄作为第六指标计算时，≥2.5的最佳截断值具有100%的敏感性和97.8%的特异性（p< 00.0001)。

我们的研究开发了一种新的评分系统，该系统由简单的实验室测试组成，具有良好的敏感性和特异性，可供临床医生用于快速区分儿童的 ALF-WD 和 NWDALF。