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Published on: November 27, 2019
Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure
Manuel Mendizabal1, Marcelo Dip2, Ezequiel Demirdjian3
1Hepatology and Liver Transplant Unit, Hospital Universitario Austral, Pilar, Argentina.
Insights
Pediatric acute liver failure (PALF) outcomes in Argentina show a 35% transplant-free survival. A new risk model using INR, bilirubin, and PALF type helps predict liver transplant or death risk.
Area of Science:
- Pediatric Hepatology
- Liver Transplantation
- Public Health
Background:
- The impact of universal hepatitis A vaccination on pediatric acute liver failure (PALF) outcomes in Argentina was previously unknown.
- Understanding PALF causes and short-term outcomes is crucial for patient management.
Purpose of the Study:
- To identify factors associated with liver transplantation (LT) or death in pediatric patients with acute liver failure.
- To determine the primary causes and short-term outcomes of PALF in Argentina.
- To develop a risk-stratification model for PALF patients.
Main Methods:
- Retrospective analysis of 135 pediatric patients with PALF listed for LT between 2007 and 2016.
- Classification of patients into "pure" PALF and PALF-chronic liver disease (CLD) based on etiology (autoimmune hepatitis, Wilson's disease, inborn errors of metabolism).
- Logistic regression used to identify independent risk factors for death or LT and to create a risk-staging model.
Main Results:
- The most frequent PALF etiologies were indeterminate (52%), autoimmune hepatitis (23%), Wilson's disease (6%), and inborn errors of metabolism (6%).
- Overall transplant-free survival was 35%, with 50% undergoing LT and 15% dying while awaiting transplant.
- Independent risk factors for worse outcomes included INR ≥3.5, bilirubin ≥17 mg/dL, and "pure" PALF. A risk-staging model showed 3-month risks of 17.6%, 36.6%, and 82% for patients with 0, 1, or ≥2 risk factors, respectively.
Conclusions:
- A significant proportion of pediatric acute liver failure patients require liver transplantation or succumb to the condition.
- A simple risk-staging model incorporating INR, bilirubin levels, and PALF classification can stratify patients by transplant-free survival.
- This model may aid in optimizing the timing of liver transplantation for pediatric patients with acute liver failure.
Abstract:
After the implementation of universal hepatitis A virus vaccination in Argentina, the outcome of pediatric acute liver failure (PALF) remains unknown. We aimed to identify variables associated with the risk of liver transplantation (LT) or death and to determine the causes and short-term outcomes of PALF in Argentina. We retrospectively included 135 patients with PALF listed for LT between 2007 and 2016. Patients with autoimmune hepatitis (AIH), Wilson's disease (WD), or inborn errors of metabolism (IEM) were classified as PALF-chronic liver disease (CLD), and others were classified as "pure" PALF. A logistic regression model was developed to identify factors independently associated with death or need of LT and risk stratification. The most common etiologies were indeterminate (52%), AIH (23%), WD (6%), and IEM (6%). Overall, transplant-free survival was 35%, whereas 50% of the patients underwent LT and 15% died on the waiting list. The 3-month risk of LT or death was significantly higher among patients with pure PALF compared with PALF-CLD (76.5% versus 42.5%; relative risk, 1.8 [1.3-2.5]; P < 0.001), and 3 risk factors were independently associated with worse outcome: international normalized ratio (INR) ≥3.5 (odds ratio [OR], 3.1; 95% confidence interval [CI], 1.3-7.2]), bilirubin ≥17 mg/dL (OR, 4.4; 95% CI, 1.9-10.3]), and pure PALF (OR, 3.8; 95% CI, 1.6-8.9). Patients were identified by the number of risk factors: Patients with 0, 1, or ≥2 risk factors presented a 3-month risk of worse outcome of 17.6%, 36.6%, and 82%, respectively. In conclusion, although lacking external validation, this simple risk-staging model might help stratify patients with different transplant-free survival rates and may contribute to establishing the optimal timing for LT.
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