Skip to main content
Log in

Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia

  • Original Article
  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

Abstract

Background

Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis.

Aims

To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia.

Methods

All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninety-day mortality was also evaluated. Prognostic factors related to both endpoints also were studied.

Results

One-year patient survival for NA was 97.1%, versus 80.8% for A1, versus 52% for A2, versus 13.6 for A3 (p < 0.001). The presence of ascites increased mortality by 17 times. In the multivariate analysis, clinically detectable ascites (HR 3.14, 95% CI 1.14–8.60, p = 0.026), lower sodium (HR 1.15, 95% CI 1.04–1.27, p = 0.006), higher bilirubin (HR 1.06, 95% CI 1.00–1.12, p = 0.023), and higher PELD score (HR 1.05, 95% CI 1.02–1.08, p = 0.001) were all associated with decreased survival. Lower serum sodium (HR 1.20, 95% CI 1.09–1.32, p < 0.001) and higher PELD score (HR 1.03, 95% CI 1.001–1.063, p = 0.043) were associated with increased 90-day mortality.

Conclusions

Clinically detectable ascites is associated with decreased 1-year survival of children with biliary atresia. These patients should be treated with caution and prioritized for liver transplantation.

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

Access this article

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Lakshminarayanan B, Davenport M. Biliary atresia: a comprehensive review. J Autoimmun. 2016;73:1–9.

    Article  Google Scholar 

  2. Muraji T, Ohtani H, Ieiri S. Unique manifestations of biliary atresia provide new immunological insight into its etiopathogenesis. Pediatr Surg Int. 2017;33:1249–1253.

    Article  Google Scholar 

  3. Petersen C. Biliary atresia: unity in diversity. Pediatr Surg Int. 2017;33:1255–1261.

    Article  Google Scholar 

  4. Nightingale S, Stormon MO, O’Loughlin EV, et al. Early posthepatoportoenterostomy predictors of native liver survival in biliary atresia. J Pediatr Gastroenterol Nutr. 2017;64:203–209.

    Article  Google Scholar 

  5. Lee WS, Ong SY, Foo HW, et al. Chronic liver disease is universal in children with biliary atresia living with native liver. World J Gastroenterol. 2017;23:7776–7784.

    Article  Google Scholar 

  6. van der Doef HPJ, van Rheenen PF, van Rosmalen M, Rogiers X, Verkade HJ, for pediatric liver transplantation centers of Eurotransplan. Wait-list mortality of young patients with biliary atresia: competing risk analysis of a Eurotransplant registry-based cohort. Liver Transpl. 2018;24:810–819.

    Article  Google Scholar 

  7. de Magnée C, Veyckemans F, Pirotte T, et al. Liver and systemic hemodynamics in children with cirrhosis: impact on the surgical management in pediatric living donor liver transplantation. Liver Transpl. 2017;23:1440–1450.

    Article  Google Scholar 

  8. Pugliese R, Fonseca EA, Porta G, et al. Ascites and serum sodium are markers of increased waiting list mortality in children with chronic liver failure. Hepatology. 2014;59:1964–1971.

    Article  CAS  Google Scholar 

  9. Bernardi M, Moreau R, Angeli P, Schnabl B, Arroyo V. Mechanisms of decompensation and organ failure in cirrhosis: from peripheral arterial vasodilation to systemic inflammation hypothesis. J Hepatol. 2015;63:1272–1284.

    Article  CAS  Google Scholar 

  10. D’Amico G, Morabito A, D’Amico M, et al. New concepts on the clinical course and stratification of compensated and decompensated cirrhosis. Hepatol Int. 2018;12:34–43.

    Article  Google Scholar 

  11. Fortune B, Cardenas A. Ascites, refractory ascites and hyponatremia in Cirrhosis. Gastroenterol Rep. 2017;5:104–112.

    Article  Google Scholar 

  12. Zipprich A, Seufferlein T, Dollinger MM. Subclinical ascites defines an intermediate stage between compensated and decompensated cirrhosis. Z Gastroenterol. 2012;50:996–1001.

    Article  CAS  Google Scholar 

  13. Child CG, Turcotte JG. Surgery and portal hypertension. Major Probl Clin Surg. 1964;1:1–85.

    CAS  PubMed  Google Scholar 

  14. Malatack JJ, Schaid DJ, Urbach AH, et al. Choosing a pediatric recipient for orthotopic liver transplantation. J Pediatr. 1987;111:479–489.

    Article  CAS  Google Scholar 

  15. Vieira SM, da Silveira TR, Matte U, et al. Amplification of bacterial DNA does not distinguish patients with ascitic fluid infection from those colonized by bacteria. J Pediatr Gastroenterol Nutr. 2007;44:603–607.

    Article  CAS  Google Scholar 

  16. Vieira SMG, Schwengber FP, Melere M, Ceza MR, Souza M, Kieling CO. The first episode of spontaneous bacterial peritonitis is a threat event in children with end-stage liver disease. Eur J Gastroenterol Hepatol. 2018;30:323–327.

    Article  Google Scholar 

  17. European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018;69:406–460.

    Article  Google Scholar 

  18. Barshes RN, Lee TC, Udell IW, et al. The pediatric end-stage liver disease (PELD) model as a predictor of survival benefit and posttransplant survival in pediatric liver transplant recipients. Liver Transpl. 2006;12:475–480.

    Article  Google Scholar 

  19. Moller S, Bernstein F. Cirrhotic multiorgan syndrome. Dig Dis Sci. 2015;60:3209–3225.

    Article  Google Scholar 

  20. Clària J, Stauber RE, Coenraad MJ, et al. Systemic inflammation in decompensated cirrhosis: characterization and role in acute-on-chronic liver failure. Hepatology. 2016;64:1249–1264.

    Article  Google Scholar 

  21. Wood JH, Patrick DA, Johnston RB Jr. The inflammatory response to injury in children. Curr Opin Pediatr. 2010;22:315–320.

    Article  Google Scholar 

  22. Jiang CB, Lee HC, Yeung CY, et al. A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. Eur J Pediatr. 2003;162:603–606.

    Article  Google Scholar 

  23. Gunadi, Gunawan TA, Widiyanto G, Yuanita A, Mulyani NS, Makhmudi A. Liver transplant score for prediction of biliary atresia patients’ survival following Kasai procedure. BMC Res Notes. 2018;11:381.

    Article  CAS  Google Scholar 

  24. Prohic D, Mesihovic R, Vanis N, Puhalovic A. Prognostic significance of ascites and serum sodium in patients with low meld scores. Med Arch. 2016;70:48–52.

    Article  Google Scholar 

Download references

Funding

Fundo de Incentivo à Pesquisa e Eventos do Hospital de Clínicas de Porto Alegre (FIPE), Porto Alegre, Brazil.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Renata R. Guedes.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

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

Guedes, R.R., Kieling, C.O., dos Santos, J.L. et al. Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia. Dig Dis Sci 65, 3369–3377 (2020). https://doi.org/10.1007/s10620-019-06029-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10620-019-06029-z

Keywords

Navigation