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[Aetiology, outcomes and prognostic indicators of paediatric acute liver failure]
Juan José Gilbert Pérez1, Belén Jordano Moreno1, Mónica Rodríguez Salas1
1Unidad de Gastroenterología, Hepatología, Nutrición y Trasplante Hepático Pediátrico, Unidad de Gestión Clínica, Críticos y Urgencias Pediátricas, Hospital Universitario Reina Sofía, Córdoba, España.
Insights
The Paediatric End-stage Liver Disease (PELD) score effectively predicts outcomes in children with acute liver failure (ALF). A high PELD score indicates a worse prognosis, aiding in transplant decisions.
Area of Science:
- Pediatric Hepatology
- Transplant Surgery
- Critical Care Medicine
Background:
- Acute liver failure (ALF) is a critical condition in children characterized by rapid liver dysfunction.
- The Paediatric End-stage Liver Disease (PELD) score is established for chronic liver disease but its utility in ALF is less understood.
Purpose of the Study:
- To analyze the causes and outcomes of pediatric ALF cases.
- To assess the PELD score's effectiveness as a prognostic tool in ALF.
Main Methods:
- Retrospective analysis of 49 pediatric ALF patients (2000-2013).
- Evaluation of etiological factors, treatment interventions (including liver transplantation), and survival rates.
- Assessment of PELD score accuracy using ROC curve analysis.
Main Results:
- Indeterminate and metabolic causes were most frequent in ALF etiology.
- 42.8% of patients required liver transplantation, with a 16.3% mortality rate.
- A PELD score cutoff of 27 demonstrated high sensitivity (86%) and specificity (85%) for predicting worse outcomes (AUC: 0.90).
- Higher bilirubin, INR, and encephalopathy correlated with increased need for liver transplant (OR for INR 1.93).
- Low PELD scores and absence of encephalopathy were associated with better survival without transplantation (RR 0.326).
Conclusions:
- High PELD scores and encephalopathy in pediatric ALF patients signify poorer outcomes.
- The PELD score shows promise as a valuable tool for optimizing liver transplant candidacy decisions in ALF.
- Further research is warranted to fully establish the role of PELD in ALF management.
Introduction:
Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF.
Objectives:
To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator.
Patients And Methods:
A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group.
Results:
The study included 49 patients with an age range 0-14years. The most frequent aetiologies were: indeterminate (36.7%) and metabolic (26.5%). Liver transplant (LT) was required by 42.8%, and there were 16.3% deaths. Patients with higher levels of bilirubin, INR, or encephalopathy were more likely to require a liver transplant, yielding an OR for INR 1.93. A cut-off of 27 in the PELD score according to the ROC curve showed a sensitivity of 86% and a specificity of 85%, predicting a worse outcome (AUC: 0.90; P<.001). The survival of patients with ALF without transplantation seems more likely in those who have low values of PELD and absence of encephalopathy, with a RR of 0.326.
Conclusions:
ALF patients with a high PELD score and the presence of encephalopathy had worse outcomes. The PELD score could be a useful tool to establish the optimum time for inclusion in the transplant list, however further studies are still needed.