Abstract

This study aimed to analyse vitamin D-binding protein (Gc-globulin) serum levels in acute liver failure (ALF) in children in relation to disease outcomes and correlations with other known markers used to evaluate the severity of ALF. Our study included 34 children (mean age 4.87 ± 5.30 years) with ALF of different causes (metabolic, 26.47%; autoimmune, 23.53%; toxic, 20.59%; infection, 17.65%; unknown, 11.76%) and 30 children without any liver injury (mean age 6.11 ± 4.26 years). The outcome was poor in 14 patients (41.18%), including one child with liver transplantation (2.94%). Serum Gc-globulin levels were significantly lower in ALF patients compared to the control group (151.57 ± 171.8 mg/L vs. 498.63 ± 252.50 mg/L; p < 0.000001), with an optimum cut-off of 163.5 mg/L (Area Under the Curve, AUC, 0.8921; sensitivity, 76.50%; specificity, 100%). Levels were also lower in patients with poor outcomes compared to survivors (59.34 ± 33.73 mg/L vs. 216.12 ± 199.69 mg/L; p < 0.0001), with an optimum cut-off 115 mg/L (AUC, 0.7642; sensitivity, 100%; specificity, 50%). Gc-globulin serum levels were variable according to ALF aetiology, i.e., lower in metabolic, infectious, or unknown causes compared to autoimmune and toxic causes. Gc-globulin serum levels were decreased in children with ALF and lower in those with poor outcomes compared with survivors. Gc-globulin serum levels were correlated with other known parameters used to evaluate the severity of ALF and could help to identify patients at high risk for poor outcomes.

Highlights

  • Paediatric acute liver failure (ALF) is a severe condition defined according to the Paediatric AcuteLiver Failure study group (PALFSG) criteria as a moderate coagulopathy (International NormalizedRatio, INR 1.5–1.9) that cannot be corrected with parenteral vitamin K in the presence of encephalopathy, or severe coagulopathy (INR > 2.0) in the absence of encephalopathy, in a previously healthy child [1,2,3].Clinical and laboratory parameters are used to predict the evolution of ALF in children, such as encephalopathy, INR or coagulation factors V or VII [4,5,6,7,8]

  • We used the Paediatric AcuteLiver Failure study group (PALFSG) criteria to select the ALF patients included in this study, including those with jaundice, coagulopathy unable to be corrected by vitamin K infusion (INR > 1.5–1.9) and liver encephalopathy or INR > 2.0 without encephalopathy in a patient with no pre-existing liver condition [1,2,3]

  • The ALF patients were aged between 1 month and 17 years and 10 months, with an average age of 4.31 ± 4.89 years (32 out of the 34 subjects were younger than 12 years old)

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Summary

Introduction

Paediatric acute liver failure (ALF) is a severe condition defined according to the Paediatric AcuteLiver Failure study group (PALFSG) criteria as a moderate coagulopathy (International NormalizedRatio, INR 1.5–1.9) that cannot be corrected with parenteral vitamin K in the presence of encephalopathy, or severe coagulopathy (INR > 2.0) in the absence of encephalopathy, in a previously healthy child [1,2,3].Clinical and laboratory parameters are used to predict the evolution of ALF in children, such as encephalopathy, INR or coagulation factors V or VII [4,5,6,7,8]. Paediatric acute liver failure (ALF) is a severe condition defined according to the Paediatric Acute. Liver Failure study group (PALFSG) criteria as a moderate coagulopathy Previous studies presented the role of new biological markers in predicting the evolution of ALF in adults. Gc-globulin serum levels were previously used to identify adults with high risk of fatal evolution, with different studies revealing that Gc-globulin levels were significantly lower in patients who died compared to those who survived. In ALF, Gc-globulin levels drop rapidly as the liver synthesises it, with some authors reporting concentrations that were 75% lower compared with adults without ALF [16,17,18,19]

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