PEDIATRICS Vol. 79 No. 3 March 1987, pp. 351-355
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Predictors of Intelligence Quotient and Intelligence Quotient Change in Persons Treated for Phenylketonuria Early in Life

Susan E. Waisbren PhD1, Barbara E. Mahon BA1, Richard R. Schnell PhD1, and Harvey L. Levy MD1

1 From the IEM-PKU Program of the Developmental Evaluation Clinic, The Children’s Hospital; the Joseph P. Kennedy, Jr, Laboratories of the Neurology Service, Massachusetts General Hospital; the Departments of Psychiatry and Neurology, Harvard Medical School; and the State Laboratory Institute, Massachusetts Department of Public Health, Boston

Ninety-one individuals with phenylketonuria who were treated early in life were followed for as many as 22 years. Regression analyses were used to determine the best predictors of IQ and IQ change. Among treatment-related variables, good dietary control of the blood phenylalanine level stood out as the best predictor of IQ. Diet discontinuation and the natural (off diet) blood phenylalanine level best predicted IQ loss, suggesting that diet continuation may be important for children with natural blood phenylalanine levels greater than 18 mg/dL.

Key Words: intelligence quotient • phenylketonuria

Submitted on February 3, 1986
Accepted on June 2, 1986




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