Submitted to: Journal of Pediatrics
Publication Type: Review article
Publication Acceptance Date: 3/12/2007
Publication Date: 5/4/2007
Citation: Heird, W.C. 2007. Omega-3 long chain polyunsaturated fatty acids in older children. Journal of Pediatrics. 150(5):457-459. Interpretive Summary:
Technical Abstract: Phenylketonuria (PKU), the most prevalent inborn error of metabolism, is usually secondary to low hepatic activity of phenylalanine hydroxylase, the enzyme that catalyzes conversion of phenylalanine to tyrosine. Growth and development of infants and children with PKU who are managed by mandatory neonatal screening programs, followed by a low protein diet supplemented with a low-phenylalanine or phenylalanine-free formula do not differ appreciably from population norms. Frequent monitoring and appropriate dietary changes in response to this monitoring are necessary to maintain plasma phenylalanine and tyrosine concentrations within the desired range. However, the IQ of well-managed children with PKU is somewhat lower than that of their unaffected siblings. These children also perform less well in school than their unaffected siblings; they tend to exhibit more behavioral problems; and they have problems concentrating. Nonetheless, current outcomes of infants and children with PKU are far superior to the severe psychomotor retardation that occurs without treatment.