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ABSTRACT Children with classical PKU had been maintained on a lowphenylalanine diet from the first few weeks of life until their 5th birthday when the phenylalanine-restricted diet was

stepwise changed to a regular diet. Physical and neurological examinations, nutritional evaluations, anthropometric measurements, and psychological assessments were done at 4, 4½, 5, and 6

years of age. In addition, neurophysiological investigations including somatosensory, auditory and visual evoked responses were obtained at 4 years 10 months, 5 years, 5 years 2 months, and

5 years 6 months. Analysis of relationships among the study variables revealed that children who had adequate dietary control and who were of average or above average intelligence displayed

normal somatosensory evoked potentials, whereas those children with less adequate dietary control and lower IQ scores had atypical somatosensory evoked potential waveforms. Yet, these

multiple assessments did not uncover any significant changes when pre-diet termination data were compared with post-diet termination results. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND

AFFILIATIONS * Department of Pediatrics, Department of Neurology, Brown University, Program in Medicine, Rhode Island Hospital, Providence, Seizure Unit, Clinical Neurophysiology,

Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts Siegfried M Pueschel, Laurie Foqelson-Dnyle, Betsy Kammerer, Ynichi Matsumiya & Leo Stern Authors *

Siegfried M Pueschel View author publications You can also search for this author inPubMed Google Scholar * Laurie Foqelson-Dnyle View author publications You can also search for this

author inPubMed Google Scholar * Betsy Kammerer View author publications You can also search for this author inPubMed Google Scholar * Ynichi Matsumiya View author publications You can also

search for this author inPubMed Google Scholar * Leo Stern View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and

permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Pueschel, S., Foqelson-Dnyle, L., Kammerer, B. _et al._ 1601 NEUROPHYSIOLOGICAL, PSYCHOLOGICAL, AND NUTRITIONAL INVESTIGATIONS DURING

DISCONTINUATION OF THE PHENYL-ALANINE-RESTRICTED DIET IN CHILDREN WITH CLASSICAL PHENYLKETONURIA (PKU). _Pediatr Res_ 15 (Suppl 4), 710 (1981).

https://doi.org/10.1203/00006450-198104001-01618 Download citation * Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-01618 SHARE THIS ARTICLE Anyone you share the

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