Découverte d'une fructosémie à l'occasion d'une insuffisance hépatique aiguë chez un enfant de 16 mois

Abstract

The authors report the case of a 16 month old baby, who developed an acute hepatic failure with shock, secondary to an intravenous invert infusion. Liver biopsy showed lesions consistent with a metabolic disease. Fructose intolerance was suspected and confirmed by an oral fructose tolerance test and by the measurement of enzyme activity on liver biopsy. A fructose free diet improved liver function dramatically; but the neurological lesions, secondary to anoxia and/or haemorrhage, resulted in an important cortical atrophy. This observation stresses the danger of fructose infusion and argues in favour of neonatal fructosaemia screening.SCOPUS: NotDefined.jinfo:eu-repo/semantics/publishe