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

par De Vroede, Monique ;Mozin, Marie-Josee ;Cadranel, Samy ;Loeb, Helmut ;Heimann, Rudolf
Référence Pediatrie, 35, 4, page (353-358)
Publication Publié, 1980

Article révisé par les pairs

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Résumé :

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.