OBJECTIVES: To assess intelligence and its relationship with blood phenylalanine concentrations and socioeconomic status in patients with phenylketonuria after 6 to 12 years of treatment. METHODS: Sixty-three children were classified according to phenylalanine levels and socioeconomic status and assessed using the Wechsler Intelligence Scale for Children. The Statistical Package for the Social Sciences (SPSS) was used to analyze phenylalanine; ANOVA was used to analyze intelligence quotients (IQ) and phenylalanine levels; and ordinal logistic regression was used to analyze the likelihood of higher IQ. RESULTS: The overall IQ scores of 90.5% of the children were within a range from borderline intellectual deficiency to very high intelligence; for verbal IQ this proportion was 96.8% and 92.1% had performance IQ scores within this band. The categories from low to upper-medium socioeconomic status contained 98.4% of patients' families. The likelihood of having medium to high IQ was 4.29 times greater for children with good phenylalanine control and 4.03 greater for those from higher socioeconomic strata. CONCLUSIONS: Treatment prevented mental retardation in 90.5% of the patients. Control of phenylalanine levels and higher socioeconomic status were associated with higher IQ scores.
Phenylketonuria; intelligence; cognition; phenylalanine
