Acute on chronic liver disease in children from the developing world: recognition and prognosis

Barath Jagadisan1, Anshu Srivastava, Surender Kumar Yachha

  • 1Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Insights

Children with chronic liver disease (CLD) experiencing acute hepatic insults face decompensation. Acute on chronic liver failure (ACLF) in children significantly increases mortality risk, but the Pediatric End-Stage Liver Disease (PELD) score can predict outcomes.

Area of Science:

  • Pediatric Hepatology
  • Gastroenterology
  • Virology

Background:

  • A subset of pediatric patients with chronic liver disease (CLD) experience decompensation after an acute insult.
  • Data on the clinical characteristics and outcomes of acute on chronic liver disease (ACLD) in children are limited.
  • Understanding the factors influencing short-term mortality in this population is crucial for timely intervention.

Purpose of the Study:

  • To characterize the clinical presentation, etiology, and outcomes of children with acute hepatic insults superimposed on CLD.
  • To identify determinants of short-term mortality in pediatric patients with ACLD.
  • To evaluate the utility of the Pediatric End-Stage Liver Disease (PELD) score in predicting mortality.

Main Methods:

  • Children with ACLD were classified into acute on chronic liver failure (ACLF) and non-ACLF groups based on established criteria.
  • Etiology of CLD, nature of the acute insult, and clinical/laboratory parameters at admission were assessed.
  • The 3-month outcome was evaluated, and Receiver Operating Characteristic (ROC) curve analysis was used to determine the predictive performance of the PELD score for 3-month mortality.

Main Results:

  • Of 36 children with ACLD, 17 met ACLF criteria. CLD was newly diagnosed in 86% of cases during the acute presentation.
  • Wilson disease and autoimmune liver disease were common underlying etiologies. Hepatitis E virus (HEV) was the most frequent cause of acute insult (75%).
  • The 3-month mortality was significantly higher in the ACLF group (59%) compared to the non-ACLF group (11%). A PELD score >25.5 accurately predicted death (100% sensitivity, 83.3% specificity).

Conclusions:

  • Superinfection with hepatotropic viruses on CLD in children leads to ACLD, presenting as ACLF or non-ACLF.
  • Hepatitis E virus is the predominant superinfecting agent in this pediatric cohort.
  • ACLF is associated with a fivefold increase in mortality compared to non-ACLF, and the PELD score is a valuable tool for differentiating survival and non-survival likelihoods.
Abstract

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