儿童肝豆状核变性急性肝衰竭患者天冬氨酸氨基转移酶与丙氨酸氨基转移酶比值及碱性磷酸酶与总胆红素比值。

Ratio of aspartate aminotransferase to alanine aminotransferase and alkaline phosphatase to total bilirubin in Wilsonian acute liver failure in children.

机构信息

Department of Pediatrics, Evercare Hospital Dhaka, Dhaka, Bangladesh.

Department of Gastroenterology, Evercare Hospital Dhaka, Dhaka, Bangladesh.

出版信息

Indian J Gastroenterol. 2022 Jun;41(3):224-230. doi: 10.1007/s12664-022-01244-5. Epub 2022 Jul 15.

DOI:10.1007/s12664-022-01244-5

PMID:35838867

Abstract

BACKGROUND

Acute liver failure (ALF) caused by Wilson disease (WD) is always fatal. Therefore, a quick diagnosis of WD is needed to start immediate management. This study aims to determine the ratio of aspartate aminotransferase to alanine aminotransferase (AST/ALT) and the ratio of alkaline phosphatase to total bilirubin (ALP/TB) in diagnosing Wilsonian acute liver failure (WALF) in children.

METHODS

Sixty children with acute liver failure were included in this study, of whom 40 had WALF and 20 had a non-Wilsonian acute liver failure (non-WALF). The serum ALT, AST, alkaline phosphatase, and total bilirubin of each blood sample were measured. We evaluated the sensitivity and specificity of AST/ALT ratio and ALP/TB ratio in WALF diagnosis.

RESULTS

Consanguinity and Kayser-Fleischer (K-F) rings were found in 32.5% and 72.5% of WALF cases, respectively. The mean hemoglobin, median ALT, median alkaline phosphatase, and mean ceruloplasmin of children with WALF were lower than those in the non-WALF group. In WALF cases, the median AST/ALT ratio was higher than in non-WALF cases. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy of the ratio of AST to ALT were 70%, 95%, 96.5%, 61.3% and 78.3%, respectively. However, when the cutoff value is ≥ 1.85, the maximum sensitivity produced by the AST/ALT ratio is 77.5% and the specificity is 95%. The sensitivity, specificity, PPV, NPV, and diagnostic accuracy of the ratio of ALP/TB < 4 were 32.5%, 100%, 100%, 42.5%, and 55%, respectively. The overall mortality rate was 50%, while the WALF mortality was 60%.

CONCLUSION

A positive AST/ALT and ALP/TB ratio strongly suggest WALF, but a negative result does not exclude WALF. We cannot use these ratios as a diagnostic tool for children with WALF. In WALF cases, the mortality rate is remarkably high, and the high score of the new Wilson index predicts the mortality rate without liver transplantation.

摘要

背景

由威尔逊病（WD）引起的急性肝衰竭（ALF）总是致命的。因此，需要快速诊断 WD，以便立即开始治疗。本研究旨在确定天门冬氨酸氨基转移酶与丙氨酸氨基转移酶（AST/ALT）比值和碱性磷酸酶与总胆红素（ALP/TB）比值在诊断儿童威尔逊急性肝衰竭（WALF）中的作用。

方法

本研究纳入 60 例急性肝衰竭患儿，其中 40 例为 WALF，20 例为非威尔逊急性肝衰竭（非 WALF）。检测每个血样的血清 ALT、AST、碱性磷酸酶和总胆红素。我们评估了 AST/ALT 比值和 ALP/TB 比值在 WALF 诊断中的敏感性和特异性。

结果

在 WALF 病例中，分别发现有血缘关系和凯氏环的占 32.5%和 72.5%。WALF 组患儿的平均血红蛋白、中位数 ALT、中位数碱性磷酸酶和平均铜蓝蛋白均低于非 WALF 组。在 WALF 病例中，AST/ALT 比值中位数高于非 WALF 病例。AST/ALT 比值的敏感性、特异性、阳性预测值（PPV）、阴性预测值（NPV）和诊断准确率分别为 70%、95%、96.5%、61.3%和 78.3%。然而，当截值≥1.85 时，AST/ALT 比值产生的最大敏感性为 77.5%，特异性为 95%。ALP/TB<4 的敏感性、特异性、PPV、NPV 和诊断准确率分别为 32.5%、100%、100%、42.5%和 55%。总死亡率为 50%，而 WALF 死亡率为 60%。