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Diagnostic Approach to Acute Liver Failure in Children: A Position Paper by the SIGENP Liver Disease Working Group
A Di Giorgio1, E Bartolini2, P L Calvo3
1Paediatric Liver, GI and Transplantation, ASST-Hospital Papa Giovanni XXIII, Piazza OMS1, Bergamo 24127, Italy.
Insights
Acute liver failure (ALF) is a serious condition in children. This review provides guidance on diagnosing and understanding the causes of pediatric ALF, crucial for effective management.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Clinical Medicine
Background:
- Acute liver failure (ALF) is a rapid decline in liver function.
- Pediatric ALF presents with coagulopathy and elevated transaminases, without prior chronic liver disease.
- ALF has a high mortality rate, often necessitating liver transplantation.
Purpose of the Study:
- To review current literature on pediatric ALF.
- To provide recommendations on the etiology, clinical features, and diagnostic work-up of ALF in children.
- To establish a foundation for future discussions on management and transplant candidacy.
Main Methods:
- Systematic review of relevant studies on pediatric ALF.
- Position statement developed by the Liver Disease Working Group of SIGENP.
- Focus on neonates, infants, and children presenting with ALF.
Main Results:
- Etiology of ALF varies significantly by age and region.
- Identifying the cause of ALF is critical for targeted treatment and transplant evaluation.
- Hepatic encephalopathy is not a prerequisite for diagnosing ALF in children.
Conclusions:
- Accurate diagnosis and etiological identification are paramount in pediatric ALF.
- This review offers essential guidance for clinicians managing pediatric ALF.
- Further consensus will address medical management and liver transplant considerations.
Abstract:
Acute liver failure (ALF) is a clinical condition characterized by the abrupt onset of coagulopathy and biochemical evidence of hepatocellular injury, leading to rapid deterioration of liver cell function. In children, ALF has been characterized by raised transaminases, coagulopathy, and no known evidence of pre-existing chronic liver disease; unlike in adults, the presence of hepatic encephalopathy is not required to establish the diagnosis. Although rare, ALF has a high mortality rate without liver transplantation (LT). Etiology of ALF varies with age and geographical location, although it may remain indeterminate in a significant proportion of cases. However, identifying its etiology is crucial to undertake disease-specific management and evaluate indication to LT. In this position statement, the Liver Disease Working Group of the Italian Society of Gastroenterology, Hepatology and Nutrition (SIGENP) reviewed the most relevant studies on pediatric ALF to provide recommendations on etiology, clinical features and diagnostic work-up of neonates, infants and children presenting with ALF. Recommendations on medical management and transplant candidacy will be discussed in a following consensus conference.
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