Skip to main content
SpringerLink
Log in

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

  • Original Article
  • Published:
Indian Journal of Gastroenterology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Wang JS, Lu Y, Wang XH, Zhu QR. Urinary copper/zinc ratio: a promising parameter for replacement of 24-hour urinary copper excretion for diagnosis of Wilson’s disease in children. World J Pediatr. 2010;6:148–53.

    Article  Google Scholar 

  2. Ala A, Schilsky ML. Wilson disease: pathophysiology, diagnosis, treatment, and screening. Clin Liver Dis. 2004;8:787–805.

    Article  Google Scholar 

  3. Ostapowicz G, Fontana RJ, Schiodt FV, et al. Acute Liver Failure Study Group. Results of a prospective study of acute liver failure at 17 tertiary care centers in the United States. Ann Intern Med. 2002;137:947–54.

    Article  Google Scholar 

  4. Roberts E, Schilsky ML. A practice guideline on Wilson disease. Hepatology. 2003;37:1475–92.

    Article  Google Scholar 

  5. Gitlin JD. Wilson disease.  Gastroenterology. 2003;125:1868–77.

    Article  Google Scholar 

  6. Berman DH, Leventhal RI, Gavaler JS, Cadoff EM, Van Thiel DH.  Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Gastroenterology. 1991;100:1129–34.

  7. Walshe JM, Dixon AK. Dangers of non-compliance in Wilson’s disease. Lancet. 1986;12:845–7.

    Article  Google Scholar 

  8. Eisenbach C, Sieg O, Stremmel W, Encke J, Merle U. Diagnostic criteria for acute liver failure due to Wilson disease. World J Gastroenterol. 2007;13:1711-4.

  9. Devarbhavi H, Singh R, Adarsh CK, Sheth K, Kiran R, Patil M. Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices. J Gastroenterol Hepatol. 2014;29:380-6.

  10. McCullough AJ, Fleming CR, Thistle JL, et al. Diagnosis of Wilson’s disease presenting as a fulminant hepatic failure. Gastroenterology. 1983;84:161–7.

    Article  CAS  Google Scholar 

  11. Korman JD, Volenberg I, Balko J, et al. Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic test. Hepatology. 2008;48:1167-74.

  12. Taly AB, Meenakshi-Sundaram S, Sinha S, Swamy HS, Arunodaya GR.  Wilson disease: description of 282 patients evaluated over 3 decades. Medicine (Baltimore). 2007;86:112–21.

    Article  Google Scholar 

  13. Sallie R, Katsiyiannakis L, Baldwin D, et al. Failure of simple biochemical indexes to reliably differentiate fulminant Wilson’s disease from other causes of fulminant liver failure. Hepatology. 1992;16:1206–11.

  14. Moores A, Fox SH, Lang AE, Hirschfield GM. Wilson disease: Canadian perspectives on presentation and outcomes from an adult ambulatory setting. Can J Gastroenterol. 2012;26:333–9.

    Article  CAS  Google Scholar 

  15. Shaver WA, Bhatt H, Combes B. Low serum alkaline phosphatase activity in Wilson’s disease. Hepatology. 1986;6:859–63.

    Article  CAS  Google Scholar 

  16. Devarbhavi H, Singh R, Adarsh CK. A simple prognostic model predicting outcome in children and adolescents with Wilson disease and acute liver failure and comparison with Wilson disease specific models. Hepatology. 2012;56 Suppl S1:962A [Abstract] [Google Scholar].

  17. Tian Y, Gong GZ, Yang x, Peng F. Diagnosis and management of fulminant Wilson’s disease: a single center’s experience. World J Pediatr. 2016;12:209–14.

    Article  CAS  Google Scholar 

  18. Socha P, Janczyk W, Dhawan A, et al. Wilson’s disease in children: a position paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66:334–44.

  19. Dhawan A, Taylor RM, Cheeseman P, De Silva P, Katsiyiannakis L, Mieli-Vergani G. Wilson’s disease in children: 37-year experience and revised King’s score for liver transplantation. Liver Transpl. 2005;11:441–8.

Download references

Author information

Authors and Affiliations

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

    Afsana Yasmin

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

    Afsana Yasmin

  3. Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

    Md. Rukunuzzaman, A S M Bazlul Karim, Rubaiyat Alam & Zannatul Ferdous Sonia

  4. Dhaka Medical College Hospital, Dhaka, Bangladesh

    Kamal Hossen

Authors
  1. Afsana Yasmin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Md. Rukunuzzaman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A S M Bazlul Karim
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rubaiyat Alam
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kamal Hossen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Zannatul Ferdous Sonia
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Afsana Yasmin and Md. Rukunuzzaman designed the research. Afsana Yasmin and Zannatul Ferdous Sonia collected the data. Afsana Yasmin, Rubaiyat Alam, and Kamal Hossen analyzed the data. Afsana Yasmin and Md. Rukunuzzaman wrote the manuscript. A S M Bazlul Karim closely supervised the whole study from the period of protocol establishment to end of study by repeated correction and gave final approval.

Corresponding author

Correspondence to Afsana Yasmin.

Ethics declarations

Conflict of Interest

AY, MR, ASMBK, RA, KH, and ZFS declare that they have no conflict of interest.

Ethics statement

The study was performed conforming to the Helsinki declaration of 1975, as revised in 2000 and 2008 concerning human and animal rights, and the authors followed the policy concerning informed consent as shown on Springer.com

Disclaimer

The authors are solely responsible for the data and the contents of the paper. In no way are the honorary editor in chief, editorial board members, the Indian Society of Gastroenterology or the printer/publishers responsible for the results/findings and content of this article.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yasmin, A., Rukunuzzaman, M., Karim, A. et al. Ratio of aspartate aminotransferase to alanine aminotransferase and alkaline phosphatase to total bilirubin in Wilsonian acute liver failure in children. Indian J Gastroenterol 41, 224–230 (2022). https://doi.org/10.1007/s12664-022-01244-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12664-022-01244-5

Keywords

Search

Navigation