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- Title
Treatment Variables and Intellectual Outcome in Children With Classic Phenylketonuria.
- Authors
Legido, Agustin; Tonyes, Linda; Carter, Donald; Schoemaker, Ann; Di George, Angelo; Grover, Warren D.
- Abstract
Records of 46 patients with classic phenylketonuria (PKU) were used to determine treatment variables associated with intellectual outcome. Patients comprised three groups: phenylalanine-restricted diet started 1) after 3 months and loss of diet control at a mean age of 7 years, 2) before age 3 months and loss of diet control at a mean age of 5 years, and 3) before age 3 months and through a mean age of 11 years. All underwent IQ testing during the diet: groups 1 and 2 were retested at a mean of six years off the diet. On the diet, groups 2 and 3 had higher IQs than group 1: groups 3 IQ was also higher than IQ off diet in groups 1 and 2. After discontinuing the diet, group 2 IQs decreased significantly. Predictors of IQ in group 1 were age at loss of diet control and percentage of phenylalanine concentrations > 15 mg/dL; in group 2, mean phenylalanine concentrations and age at loss diet control. Predictors of changes in group 1 IQs were global degree of dietary control and percentage of phenylalanine concentrations > 15 mg/dL, in group 2, phenylalanine concentrations of <3 mg/dL and age at start of diet. Group 1 patients with phenylalanine concentrations <3 mg/dL or >15 mg/dL achieved no IQ gain by continuing the diet after age 7 years. Thus, intellectual prognosis is best for PKU patients who start a phenylalanine-restricted diet early and continue through age 12 years.
- Subjects
PHENYLKETONURIA; AMINO acid metabolism disorders; PEDIATRICS; INTELLECTUAL disabilities; JUVENILE diseases
- Publication
Clinical Pediatrics, 1993, Vol 32, Issue 7, p417
- ISSN
0009-9228
- Publication type
Article
- DOI
10.1177/000992289303200706