[对肝豆状核变性患儿24小时尿铜排泄诊断价值的重新评估]

[The reassessment of the diagnostic value of 24-hour urinary copper excretion in children with Wilson's disease].

作者信息

Lu Yi, Liu Xiao-Qing, Wang Xiao-Hong, Wang Jian-She

机构信息

Childrens Hospital of Fudan University, Shanghai 201102, China.

出版信息

Zhonghua Gan Zang Bing Za Zhi. 2010 Jan;18(1):49-53. doi: 10.3760/cma.j.issn.1007-3418.2010.01.012.

DOI:10.3760/cma.j.issn.1007-3418.2010.01.012

PMID:20128970

Abstract

OBJECTIVE

To reassess the diagnostic value of 24 hour urinary copper excretion in children with Wilson disease (WD).

METHODS

From July 2005 to June 2007, inpatients over three years old in a pediatric liver center were assigned into WD and non-WD group.

RESULTS

94 patients, including 26 cases in WD and 68 in non-WD group, were enrolled in this study. The median of 24 h urinary copper excretion was 98.5 microg in WD group and 25.8 microg in the non-WD group (Z = -6.111, P equal to 0.000). The area under receiver operator curve (ROC) was 0.909 (95% CI: 0.839-0.979, P equal to 0.000). The sensitivity, specificity, accuracy, positive predictive value and negative predictive value were 84.6%, 91.2%, 89.4%, 78.6% and 93.9% respectively using 52.0 ug as a cutoff value, and 50.0%, 97.1%, 84.0%, 86.7% and 83.5% using 100 microg as a cutoff value. The goodness of fitness of 52 microg criteria was significantly higher than 100 microg criteria (kappacoefficient 0.760, 0.541 respectively, P equal to 0.000).

CONCLUSION

Comparing to 100, 52 microg of 24 h urinary copper excretion as a cutoff value significantly improves the sensitivity and accuracy for diagnosing WD in children.

摘要

目的

重新评估24小时尿铜排泄量对肝豆状核变性（WD）患儿的诊断价值。

方法

2005年7月至2007年6月，将某儿科肝脏中心3岁以上的住院患儿分为WD组和非WD组。

结果

本研究共纳入94例患者，其中WD组26例，非WD组68例。WD组24小时尿铜排泄量中位数为98.5μg，非WD组为25.8μg（Z=-6.111，P=0.000）。受试者工作特征曲线（ROC）下面积为0.909（95%CI：0.839-0.979，P=0.000）。以52.0μg为临界值时，敏感度、特异度、准确度、阳性预测值和阴性预测值分别为84.6%、91.2%、89.4%、78.6%和93.9%；以100μg为临界值时，分别为50.0%、97.1%、84.0%、86.7%和83.5%。52μg标准的拟合优度显著高于100μg标准（卡帕系数分别为0.760、0.541，P=0.000）。