Diagnostic Value of a Modified Version of Wilson's Diagnostic Score in Pediatrics.

IF 0.3 Q4 TRANSPLANTATION

International Journal of Organ Transplantation Medicine Pub Date : 2020-01-01

S Sajedianfard, M Ataollahi, S M Dehghani

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引用次数: 0

Abstract

Background: Wilson's disease (WD) is an autosomal-recessive hereditary liver disease affecting copper metabolism.

Objective: To test the diagnostic value of a questionnaire for the diagnosis of WD in pediatrics age group.

Methods: 70 children with biopsy-proven diagnosis of WD and 70 without WD were included in the study. A modified questionnaire with 4 items was used for the diagnosis of WD. The results were then compared to the definite diagnosis made by pathology (the gold standard test).

Results: The median (IQR) modified score in those with WD was 4 (4-5), significantly (p<0.001) higher than that calculated for the comparison group, which was 0 (0-1). The most appropriate cut-off value for the score was 2.5, corresponding to a sensitivity and specificity of 100%, and 98.6%, respectively. Using this cut-off value to classify 20 children with and without WD who underwent liver transplantation resulted in an accuracy of 100%.

Conclusion: The modified scoring system is a sensitive and specific diagnostic tool for the diagnosis of WD in children. This is especially important in regions with limited access to specific laboratory tests for the diagnosis of WD.

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本刊更多论文

改良版威尔逊诊断评分在儿科中的诊断价值。

背景:威尔逊氏病(WD)是一种常染色体隐性遗传性肝病，影响铜代谢。目的:探讨问卷对儿科年龄组WD的诊断价值。方法:70例经活检确诊为WD的儿童和70例未确诊为WD的儿童纳入研究。采用一份包含4个项目的改进问卷进行WD诊断。然后将结果与病理做出的明确诊断(金标准测试)进行比较。结果:WD患者的修正评分中位数(IQR)为4(4-5)分，差异有统计学意义(p)。结论:修正评分系统是诊断儿童WD的一种敏感、特异的诊断工具。这在获得诊断WD的特定实验室检测有限的地区尤为重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

International Journal of Organ Transplantation Medicine TRANSPLANTATION-

CiteScore

1.60

自引率

0.00%

发文量

审稿时长

12 weeks

期刊介绍： The International Journal of Organ Transplantation Medicine (IJOTM) is a quarterly peer-reviewed English-language journal that publishes high-quality basic sciences and clinical research on transplantation. The scope of the journal includes organ and tissue donation, procurement and preservation; surgical techniques, innovations, and novelties in all aspects of transplantation; genomics and immunobiology; immunosuppressive drugs and pharmacology relevant to transplantation; graft survival and prevention of graft dysfunction and failure; clinical trials and population analyses in the field of transplantation; transplant complications; cell and tissue transplantation; infection; post-transplant malignancies; sociological and ethical issues and xenotransplantation.