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

Yasmin, Afsana; Rukunuzzaman, M; Karim, Asm Bazlul; Alam, Rafiqul; Hossen, Kamal; Sonia, Zannatul Ferdous

doi:10.1007/s12664-022-01244-5

Cited by 5 publications

(2 citation statements)

References 18 publications

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“…In a cross-sectional study, 60 children aged 3–18 years with acute liver failure were divided into a WD acute liver failure (WALF) group and other causes of acute liver failure (non-WALF) group. The results showed that the mean HGB, mean ALT, mean AKP, and mean ceruloplasmin in WALF children were lower than those in the non-WALF group ( 12 ). No acute liver failure occurred in the patients included in this study, but the reduction of the above indicators can be used as a basis for further diagnosis of acute liver failure in WD.…”

Section: Discussionmentioning

confidence: 84%

Construction of diagnostic prediction model for Wilson's disease

Wang

Li²,

Xun³

et al. 2023

Front. Surg.

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BackgroundWilson's disease, also known as hepatolenticular degeneration, is a rare human autosomal recessive inherited disorder of copper metabolism. The clinical manifestations are diverse, and the diagnosis and treatment are often delayed. The purpose of this study is to establish a new predictive diagnostic model of Wilson's disease and evaluate its predictive efficacy by multivariate regression analysis of small trauma, good accuracy, low cost, and quantifiable serological indicators, in order to identify Wilson's disease early, improve the diagnosis rate, and clarify the treatment plan.MethodsA retrospective analysis was performed on 127 patients with Wilson's disease admitted to the First People's Hospital of Yunnan Province from January 2003 to May 2022 as the experimental group and 73 patients with normal serological indicators who were not diagnosed with Wilson's disease. SPSS version 26.0 software was used for single factor screening and a multivariate binary logistic regression analysis to screen out independent factors. R version 4.1.0 software was used to establish an intuitive nomogram prediction model for the independent influencing factors included. The accuracy of the nomogram prediction model was evaluated and quantified by calculating the concordance index (C-index) and drawing the calibration curve. At the same time, the area under the curve (AUC) of the nomogram prediction model and the receiver operating characteristic (ROC) curve of the Leipzig score was calculated to compare the predictive ability of the nomogram model and the current Leipzig score for Wilson's disease.ResultsAlanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (AKP), albumin (ALB), uric acid (UA), serum calcium (Ca), serum phosphorus (P), and hemoglobin (HGB) are closely related to the occurrence of Wilson's disease (p < 0.1). The prediction model of Wilson's disease contains seven independent predictors: ALT, AST, AKP, ALB, UA, Ca, and P. The AUC value of the prediction model was 0.971, and the C-index value was 0.972. The calibration curve was well fitted with the ideal curve. The nomogram prediction model had a good predictive effect on the occurrence of Wilson's disease; the ROC curve of Leipzig score was drawn, and the AUC value was calculated. The AUC of the Leipzig score was 0.969, indicating that the prediction model and the scoring system had predictive value, and the nomogram prediction model had a better predictive effect on the research objects of the center.ConclusionALT, AST, AKP, ALB, UA, Ca, and P are independent predictors of Wilson's disease, and can be used as early predictors. Based on the nomogram prediction model, the optimal threshold was determined to be 0.698, which was an important reference index for judging Wilson's disease. Compared with the Leipzig score, the nomogram prediction model has a relatively high sensitivity and specificity and has a good clinical application value.

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Section: Discussionmentioning

confidence: 84%

Construction of diagnostic prediction model for Wilson's disease

Wang

Li²,

Xun³

et al. 2023

Front. Surg.

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“…These tests are not as reliable as in chronic WD patients and are less sensitive and specific than other available tests such as hemoglobin, serum transaminases, alkaline phosphatase and total bilirubin [40]. In cases of acute liver failure, the presence of a nonimmune acute intravascular hemolysis with severe coagulopathy, relatively modest elevations of serum aminotransferases, normal or subnormal alkaline phosphatase and progression to renal failure is suggestive of the diagnosis of WD [4,41].…”

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confidence: 99%

Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests

Guillaud

Dumortier²,

Couchonnal-Bedoya³

et al. 2023

Diagnostics

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Wilson disease and alpha1-antitrypsin deficiency are two rare genetic diseases that may impact predominantly the liver and/or the brain, and the liver and/or the lung, respectively. The early diagnosis of these diseases is important in order to initiate a specific treatment, when available, ideally before irreversible organ damage, but also to initiate family screening. This review focuses on the non-invasive diagnostic tests available for clinicians in both diseases. These tests are crucial at diagnosis to reduce the potential diagnostic delay and assess organ involvement. They also play a pivotal role during follow-up to monitor disease progression and evaluate treatment efficacy of current or emerging therapies.

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Editorial commentary on the Indian Journal of Gastroenterology—May–June 2022

Limdi

2022

Indian J Gastroenterol

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Ratio of aspartate aminotransferase to alanine aminotransferase and alkaline phosphatase to total bilirubin in Wilsonian acute liver failure in children Acute liver failure (ALF) secondary to Wilson's disease (WD) is invariably fatal without emergency liver transplantation, and requires a prompt diagnosis [1]. Although reduced ceruloplasmin and elevated copper levels support a diagnosis of WD, they are less reliable in a fulminant setting [1]. Laboratory findings associated with fulminant WD include Coombs negative hemolytic anemia, low serum uric acid levels, low serum alkaline phosphatase (ALP) activity and increased aspartate amino transferase (AST):alanine amino transferase (ALT) ratios [2].Yasmin and colleagues from Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, report on a series of 60 children with ALF, of whom 40 had WDassociated ALF (WALF) [3]. The median AST/ALT ratio was higher in WALF than in non-WALF patients with sensitivity and specificity of 70% and 95% and a positive and negative predictive value of 96.5% and 61.3%, respectively. At a cut-off value of ≥1.85, AST/ALT ratio had a sensitivity of 77.5% and specificity of 95%. An ALP/total bilirubin ratio <4 had sensitivity and specificity of 32.5% and 100% and a positive and negative predictive value of 100% and 42.5%, respectively. The authors suggest that using these routinely available tests, patients with fulminant WD may be identified promptly, enabling emergency listing for liver transplantation for this rare but near uniformly fatal disorder.

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

Cited by 5 publications

References 18 publications

Construction of diagnostic prediction model for Wilson's disease

Construction of diagnostic prediction model for Wilson's disease

Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests

Editorial commentary on the Indian Journal of Gastroenterology—May–June 2022

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