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Published on: November 27, 2019
Pediatric Acute-on-Chronic Liver Failure
Seema Alam1, Bikrant Bihari Lal2
1Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India. seema_alam@hotmail.com.
Insights
Acute-on-chronic liver failure (ACLF) in children involves liver damage superimposed on cirrhosis, leading to inflammation and organ failure. Pediatric ACLF prognosis is often better than in adults due to treatable causes and better reserves.
Area of Science:
- Pediatric Hepatology
- Critical Care Medicine
- Gastroenterology
Background:
- Acute-on-chronic liver failure (ACLF) is a severe syndrome in patients with cirrhosis, characterized by systemic inflammation, sepsis, and organ failure.
- Definitions of pediatric ACLF lack consensus, with differing criteria between the Asia Pacific Association for Study of Liver Diseases (APASL) and European Association for Study of Liver Diseases (EASL).
- Common underlying causes in children include Wilson disease (WD) and autoimmune hepatitis (AIH), with acute viral hepatitis or flares of WD/AIH as precipitating events.
Purpose of the Study:
- To review the current understanding of pediatric ACLF, including definitions, etiologies, outcomes, and management strategies.
- To highlight the differences in ACLF definitions between major liver associations and the specific challenges in pediatric cases.
- To discuss prognostic factors and treatment approaches for pediatric ACLF.
Main Methods:
- Review of existing literature and guidelines on ACLF, with a focus on pediatric cases.
- Analysis of reported outcomes, mortality rates, and prognostic scores (e.g., APASL ACLF Research Consortium [AARC] score).
- Discussion of treatment modalities, including medical management, bridging therapies like plasma exchange, and liver transplantation (LT).
Main Results:
- Pediatric ACLF outcomes range from 19% to 59% for death or liver transplantation (LT).
- Prognosis in pediatric ACLF is generally better than in adults due to treatable etiologies, fewer organ failures, and better hepatic reserves.
- An APASL ACLF Research Consortium (AARC) score >= 11 predicts poor 28-90 day mortality.
Conclusions:
- Prompt diagnosis and management of underlying causes are crucial for pediatric ACLF treatment.
- Early initiation of bridging therapies, such as high-volume plasma exchange, can be vital.
- Liver transplantation should be considered promptly for non-improving cases to prevent sepsis or multi-organ failure.
Abstract:
Acute-on-chronic liver failure (ACLF) is characterized by an acute hepatic insult happening in a patient with underlying cirrhosis with compromised hepatic reserve leading to development of systemic inflammation, sepsis, and organ failure resulting in poor outcome in majority. While Asia Pacific Association for Study of Liver Diseases (APASL) emphasizes on early diagnosis before development of organ failure, European Association for Study of Liver Diseases (EASL) mandates the presence of organ failures to define ACLF. There is a lack of consensus definition of pediatric ACLF although recent APASL guidelines have tried to address the issue. While Wilson disease (WD) and autoimmune hepatitis (AIH) are the most common cause of underlying cirrhosis in children, acute viral hepatitis and flares of WD and AIH are the commonest acute precipitating events. Poor outcomes [death and liver transplantation (LT)] ranging from 19 to 59% have been reported. Prognosis in pediatric ACLF is usually better than that in adults due to greater proportion of treatable etiologies, lesser organ failures, comorbidities and better hepatic reserves. APASL ACLF Research Consortium (AARC) score more than or equal to 11 is predictive of poor 28-90 d mortality. Treatment of pediatric ACLF relies mainly on prompt diagnosis and medical management of a potentially treatable etiology of underlying cirrhosis. Bridging therapies, especially high volume plasma exchange can be initiated early as a bridge to LT or native liver recovery. Those with no improvement in 4-7 d should undergo LT before development of sepsis or multi-organ failure.
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