Abstract

Objectives: In children, acute liver failure (ALF) is a severe condition with high mortality. As some patients need liver transplantation (LT), it is essential to predict the fatal evolution and to refer them early for LT if needed. Our study aimed to evaluate the prognostic criteria and scores for assessing the outcome in children with ALF. Methods: Data of 161 children with ALF (54.66% female, mean age 7.66 ± 6.18 years) were analyzed based on final evolution (32.91% with fatal evolution or LT) and etiology. We calculated on the first day of hospitalization the PELD score (109 children), MELD, and MELD-Na score (52 children), and King’s College Criteria (KCC) for all patients. The Nazer prognostic index and Wilson index for predicting mortality were calculated for nine patients with ALF in Wilson’s disease (WD). Results: PELD, MELD, and MELD-Na scores were significantly higher in patients with fatal evolution (21.04 ± 13.28 vs. 13.99 ± 10.07, p = 0.0023; 36.20 ± 19.51 vs. 20.08 ± 8.57, p < 0.0001; and 33.07 ± 8.29 vs. 20.08 ± 8.47, p < 0.0001, respectively). Moreover, age, bilirubin, albumin, INR, and hemoglobin significantly differed in children with fatal evolution. Function to etiology, PELD, MELD, MELD-Na, and KCC accurately predicted fatal evolution in toxic ALF (25.33 vs. 9.90, p = 0.0032; 37.29 vs. 18.79, p < 0.0001; 34.29 vs. 19.24, p = 0.0002, respectively; with positive predicting value 100%, negative predicting value 88.52%, and accuracy 89.23% for King’s College criteria). The Wilson index for predicting mortality had an excellent predictive strength (100% sensibility and specificity), better than the Nazer prognostic index. Conclusions: Prognostic scores may be used to predict the fatal evolution of ALF in children in correlation with other parameters or criteria. Early estimation of the outcome of ALF is essential, mainly in countries where emergency LT is problematic, as the transfer to a specialized center could be delayed, affecting survival chances.

Highlights

  • This article is an open access articleIn children, acute liver failure (ALF) is a rare but severe disorder associated with high mortality [1,2]

  • The etiology of ALF was different by age group and included toxic causes (64 patients, 39.75%, drug-induced in 51 patients, and mushroom poisoning in 13 patients), infectious diseases (41 patients, 25.45%), metabolic causes (27 patients, 16.77%: IEM in 18 patients and Wilson’s disease (WD) in 9 patients), and autoimmune diseases (15 patients, 9.32%)

  • Emergency liver transplantation (LT) was performed in two girls with fulminant WD and one infant with metabolic disease that otherwise would not survive

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Summary

Introduction

Acute liver failure (ALF) is a rare but severe disorder associated with high mortality [1,2]. Pediatric ALF (PALF) differs from adults due to the type and diversity of causes and late appearance of hepatic encephalopathy (HE) [1]. The etiology of ALF in children varies according to age and worldwide location [3,4,5,6,7]. Children with autoimmune hepatitis (AIH), hepatitis A virus (HAV) infection, and acetaminophen overdose are more distributed under the terms and conditions of the Creative Commons. Neonates with Herpes simplex virus (HSV) infection and ALF have 10% recovery chances only with antiviral treatment [8,9]. In PALF, 20% of those who never developed HE died or underwent LT, and those with grade IV HE had a better outcome than those who progressed to grade IV [10]

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