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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.
Objectives:
A subset of children with chronic liver disease (CLD) decompensate following an acute insult; however, data for children are not readily available. The present study aims to characterize the clinical presentation, etiology, outcome, and determinants of short-term mortality in children with an acute hepatic insult superimposed over CLD.
Patients And Methods:
Children of acute on chronic liver disease (ACLD) were grouped as acute on chronic liver failure (ACLF) and non-ACLF. ACLF was defined as per the definition proposed by Asian Pacific Association for the Study of Liver. The acute insult, etiology of CLD, and clinical and laboratory parameters at admission along with 3-month outcome were assessed. Receiver operating curve (ROC) was plotted to measure the performance of pediatric end-stage liver disease (PELD) score in predicting the 3-month mortality.
Results:
Of the 36 children with ACLD (median age 9.5; range 3-15 years), 17 fulfilled ACLF criteria and 19 non-ACLF. CLD was diagnosed for the first time in 86% children during their presentation with a superimposed acute insult. Wilson disease and autoimmune liver disease were the most common underlying etiology. Acute insult was caused by hepatitis E virus (HEV) in 27 (75%) children and hepatitis A virus (HAV) in 10 (28%). The 3-month mortality of ACLF group was significantly higher than that of non-ACLF group (59% vs 11%, P = 0.001). PELD score of >25.5 predicted death, with a sensitivity of 100% and specificity of 83.3%.
Conclusions:
Superinfection with hepatotropic viruses on CLD in children manifests as ACLD: ACLF and non-ACLF. Hepatitis E virus is the most common superinfection in the population studied. The mortality in ACLF is 5 times higher than that in the non-ACLF group. PELD score is useful in differentiating likely survivors and nonsurvivors.
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