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Pediatric Acute-on-Chronic Liver Failure in a Specialized Liver Unit: Prevalence, Profile, Outcome, and Predictive
Seema Alam1, Bikrant B Lal, Vikrant Sood
1Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
Insights
Pediatric acute-on-chronic liver failure (ACLF) affects 11.2% of children with chronic liver disease, with a 61% survival rate. A high Chronic Liver Failure-Sequential Organ Failure Assessment score predicts mortality.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Critical Care Medicine
Background:
- Pediatric acute-on-chronic liver failure (ACLF) is a severe condition with high mortality.
- Understanding its prevalence, characteristics, and predictors is crucial for improving outcomes in children with chronic liver disease (CLD).
Purpose of the Study:
- To determine the prevalence, clinical profile, and outcomes of pediatric ACLF.
- To identify factors that predict mortality in children with ACLF.
Main Methods:
- A study included 499 children with CLD, identifying 56 (11.2%) with ACLF based on established criteria.
- Data were collected retrospectively and prospectively between January 2011 and October 2015.
- Outcomes were defined as survival with native liver at 90 days or death/liver transplantation.
Main Results:
- The mean age of children with ACLF was 9.35 years, with Wilson disease and autoimmune hepatitis being the most common underlying CLDs.
- Disease flare-ups and acute viral hepatitis were the primary triggers for ACLF.
- Poor outcomes (death or transplantation) occurred in 39.3% of cases, associated with severe hepatic encephalopathy, high bilirubin, elevated INR, and multiple organ failures.
Conclusions:
- Pediatric ACLF has an 11.2% prevalence among children with CLD, with a 61% 90-day survival rate.
- A Chronic Liver Failure-Sequential Organ Failure Assessment (CLIF-SOFA) score of ≥10 is a strong predictor of 90-day mortality.
Objectives:
The aim of the study was to assess the prevalence, profile, outcome, and predictive factors of pediatric acute-on-chronic liver failure (ACLF).
Methods:
All children 3 months to 18 years satisfying the Asia Pacific Association for the Study of Liver Diseases definition of ACLF were included. Data were both extracted from records (January 2011 to December 2014) and prospectively collected (January to October 2015). Successful outcome was defined as survival with native liver at 90 days, whereas poor outcome included those who died or received liver transplantation.
Results:
Of the 499 children with chronic liver disease (CLD), 56 (11.2%) presented as ACLF, with a mean age of 9.35 (±4.39) years. Wilson disease and autoimmune hepatitis were the commonest underlying CLDs accounting for 24 (42.8%) and 18 (32.1%) cases, respectively. The most frequent events precipitating ACLF were a flare up of the underlying disease in 27 (48.2%) and acute viral hepatitis in 17 (30%). Poor outcome occurred in 22 (39.3%) children: 17 (30.4%) died and 5 (8.9%) received liver transplantation. Poor outcome was associated with grades 3 to 4 hepatic encephalopathy, bilirubin ≥17.5, international normalized ratio ≥3.5, and presence of 2 or more organ failures. On multivariate analysis, a Chronic Liver Failure-Sequential Organ Failure Assessment score ≥10 best predicted mortality (odds ratio 20.45, 95% confidence interval 3.9-106.7).
Conclusions:
ACLF is present in 11.2% of childhood CLD, with a 90-day native liver survival of 61%. A Chronic Liver Failure-Sequential Organ Failure Assessment score of ≥10 best predicts mortality at day 90.
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