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• W2009074673 abstract "Introduction: the causes and natural history of acute liver failure (ALF) in infants differ from those in older children and liver transplant (LT) may not always be indicated. Metabolic aetiology could be responsible for 40% of the cases, so decisions regarding the timing of listing and the contraindications to LT are difficult. Aim: identification of markers of metabolic disease on admission in children under two years old with ALF. Methods: retrospective review of all children (< 2 years) with ALF which was defined as coagulopathy (INR > 2) unresponsive to vitamin K. Infants with evidence of chronic liver disease (CLD) and multiorgan dysfunction at presentation were excluded. Two Groups were defined according to the aetiology: Group A – Metabolic disease Group B – Other aetiologies Age, medical history, laboratory tests and echocardiography on admission were compared. Statistic analysis was done using Fisher exact and t Student tests. Results: there were 37 children with ALF of whom 21 were under two years old. Four were excluded because they had evidence of CLD. The aetiology was: Group A: 8 (1 hereditary fructose intolerance, 1 tyrosinaemia, 1 galactosaemia, 1 LCHAD deficiency, 1 CDG, 1 mitochondrial respiratory chain disorder, 1 neonatal haemochromatosis, 1 without defined aetiology); Group B: 9 (3 viral infections, 2 mushroom poisonings, 2 malformations, 1 autoimmune hepatitis, 1 without defined aetiology). The mean age of children from Group A was 2,4 months and from Group B was 9 months (p 0,033). The children from Group A had more frequent vomiting episodes and/ or failure to thrive (100%) when compared with Group B (33%) (p 0,028). Children with metabolic disease had lower bilirubin levels on admission (Group A - 136 umol/L; Group B - 328 umol/L; p 0,038), lower AST (Group A - 513; Group B – 3468UI/L; p 0,001), and higher base excess deficiency (Group A -6,9; Group B -0,8; p 0,024). There were no differences between the Groups in the other laboratory tests analysed on admission, including the presence of reducing substances in urine (positive in Group A - 57%; positive in Group B - 33%; p 0,592). Echocardiography revealed left ventricular hypertrophy in 75% of children with metabolic disease (Group A) and in 11% of other Group (p 0,015). Conclusion: children with metabolic disease had lower age on admission, more frequent episodes of vomiting/ failure to thrive, were less cholestatic and had lower AST levels, higher base excess deficiency and more frequent left ventricular hypertrophy. This data could be important to delineate a strategy and to establish decisions related to LT/ other therapeutic options but needs to be validated." @default.
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• W2009074673 date "2004-06-01" @default.
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• W2009074673 title "P0093 PP ACUTE LIVER FAILURE UNDER TWO YEARS OLD ???MARKERS OF METABOLIC DISEASE ON ADMISSION" @default.
• W2009074673 doi "https://doi.org/10.1097/00005176-200406001-00217" @default.
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