Acta Paediatrica · 1999 · 31 citations · 4 references
Dietary AssessmentNutritionCaloric RestrictionVeneselli E. PhenylketonuriaBiochemical NutritionDietary IntakeNeurologyCerone RPublic HealthNeuropathologyChronic Kidney DiseaseMedical NutritionInherited Metabolic DiseaseClinical NutritionMedical Nutrition TherapyDietary TherapyRestricted Phenylalanine DietNutritional NeuroscienceNutritional SciencesHuman NutritionMetabolismMedicineNephrologyNutrition Assessment
Cerone R, Schiaffino MC, Di Stefano S, Veneselli E. Phenylketonuria: diet for life or not? Acta Pædiatr 1999; 88: 664‐6. Stockholm. ISSN 0803‐5253 In order to evaluate the argument whether or not a restricted phenylalanine diet should be maintained for life in patients with phenylketonuria (PKU), 16 patients with early treated PKU but off diet since their 11th birthday were investigated. The evaluation included a detailed neurological examination, IQ, neurophysiological testing and MRI of the brain. Even if IQ and electrophysiological studies were normal or unchanged if compared to results before diet discontinuation, all patients revealed abnormal neurological signs. We conclude that the diet should be continued during adult life, but somewhat higher phenylalanine levels (<10mg/dl;< 600 μmol/l) than at younger ages should be allowed. □ Adolescence, dietary treatment, phenylketonuria
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Brain MRI changes in phenylketonuria: Associations with dietary status
Alan J. Thompson, Sarah Tillotson, Isabel Smith et al. · Brain · 1993 · 163 citations
White Matter Abnormalities, Nutrition, Brain Mri Changes +14