This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Legido, A. Articles by Grover, W. D. Search for Related Content PubMed PubMed Citation Articles by Legido, A. Articles by Grover, W. D. Pubmed/NCBI databases Genetics Home Reference Genetics Home Reference - phenylketonuria Medline Plus Health Information Phenylketonuria Social Bookmarking                         What's this?

Treatment Variables and Intellectual Outcome in Children With Classic Phenylketonuria

A Single-Center-Based Study

Section of Neurology, St. Christopher's Hospital for Children, Erie Ave. at Front St., Philadelphia, PA 19134

Linda Tonyes, R.D.

Section of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania

Donald Carter, M.S.

Section of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania

Ann Schoemaker, R.N.

Section of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania

Angelo Di George, M.D.

Section of Endocrinology, Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania

Warren D. Grover, MD.

Section of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania

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; group 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 of 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.

Clinical Pediatrics, Vol. 32, No. 7, 417-425 (1993)
DOI: 10.1177/000992289303200706


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?