OC50 Acute liver failure in children: aetiology and evolution

Abstract

Introduction Acute liver failure (ALF) is a syndrome defined by rapid decline of hepatic function, jaundice, coagulopathy (INR >1.5) and hepatic encephalopathy in patients with no evidence of previous liver disease. The aetiology of ALF in children varies on the age. Material and methods We have analysed retrospectively the aetiology of the ALF in children followed-up in our clinic during 7-year period (January 2012 - December 2018). We studied the main causes of ALF based on the age of children and their evolution after treatment. Results During this period, 97 patients (46 males, 47.42%) were admitted with ALF: 33 infants (0–12 months), 30 children (1–14 years) and 34 adolescents (14–18 years). In infants the most important causes of ALF were viral and bacterial infections: 24 patients (72.72%). Cytomegalovirus, Epstein-Barr virus, Herpesvirus, Parvovirus or Gram-negative bacteria were involved, with a high mortality: 10 patients (30.30%). Metabolic disorders were the cause of ALF in 9 patients (43.47%): 3 infants with mitochondrial disorder, 2 infants with galactosemia and tyrosinemia respectively, and one with neonatal haemochromatosis and hereditary fructose intolerance. Of those, 3 survived after supportive therapy and diet, one is alive after liver transplantation and 5 died. In children, toxic hepatitis was the most important cause of ALF (18 patients, 60%): albendazole in 11 children, mushrooms in 3 children, and one child with each acetaminophen, valproate or fluconazole. Viral and bacterial infections were the causes of ALF in 5 children (16.66%), 2 of them developed fatal aplastic anaemia. There were also 4 children with autoimmune hepatitis (AIH, 13.33%) and 3 children with Wilson disease (WD, 10%) presenting ALF at the time of diagnosis. The mortality in children with ALF was 20% (6 patients). Drugs and mushrooms intoxication was the most common cause of ALF among adolescents: 27 patients (79.41%), acetaminophen being the most often involved (17 patients). Fortunately, the mortality was low and only one patient died after colchicine overdose. There were 4 patients with AIH and 4 with WD. One patient died with Fulminant WD, other 2 were transplanted and the others are alive with supportive therapy. Conclusions In children, the aetiology of ALF is different depending on age and is associated with a high mortality despite optimal medical therapy, but without emergency liver transplantation. The management of this cases requires a multidisciplinary team involving paediatric hepatologist, critical care specialist, nephrologist or liver transplant surgeon.