Behavior in Phenylketonuria

Bjornson, Jon

doi:10.1001/archpsyc.1964.01720190067009

Cited by 18 publications

(11 citation statements)

References 28 publications

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“…Since the initial description of this disorder by Foiling (1934), there have been a number of reports on behavioral deviations concomitant with phenylketonuria (PKU) (e.g., Bjornson, 1964;Jervis, 1954;Kaplan, 1962;Karrer & Cahilly, 1965;Lyman, 1963). Most prominent of these is mental deficiency (e.g., Hsia, Knox, & Paine, 1957;Kaplan, 1962).…”

mentioning

confidence: 99%

Interaction patterns in children with phenylketonuria.

Im¹,

Cj²,

Ac³

1967

Journal of Consulting Psychology

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To elucidate the role of emotional factors in PKU, 4 subject groups comprised of PKU, retarded and/or brain damaged, psychotic, and normal children were compared on a measure of interaction behavior. On total interaction scores, the PKU group was found to perform significantly poorer than the normals, but significantly better than the psychotics. Differences between the PKU group and retarded and/or brain-damaged group tended toward significance, although on separate comparisons for the 3 social stimulus conditions the differences between these 2 groups were not significant. The PKU group was found to be the most heterogeneous, and the clustering of scores suggested that phenylketonuria is behaviorally not a unitary disorder. Correlations of intelligence criteria and interaction scores for the PKU group further indicated that the interaction measure may tap functions not assessed by standardized IQ tests.

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mentioning

confidence: 99%

Interaction patterns in children with phenylketonuria.

Im¹,

Cj²,

Ac³

1967

Journal of Consulting Psychology

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“…One of these, a twelveyear-old girl, just under the age of three years at the time, had been hospitalized for a period of 30 days, most of which time was spent in the absence of parents of familiar persons. She remained in a private room and contentedly kept to herself, receiving attention 2 Test of significance between means of samples with heterogeneous variances [5].…”

Section: Resultsmentioning

confidence: 99%

“…There is no constant relation between biochemical findings and the degree of mental deficiency and behavioral symptoms in patients with PKU [11]. Institutionalization may lead to regression of intellectual capacity and behavioral adaptation of these patients because of long-term isolation and decreased stimulation [2]. However, more subtle factors in surroundings and in the handling of the affected infant or child may relate significantly to behavioral and intellectual function, especially when such influences occur at vulnerable stages of development and involve children with decreased resistance to such stresses.…”

Section: Discussionmentioning

confidence: 99%

The Effect of Immobilization and Sensory Restriction on Children with Phenylketonuria

et al. 1968

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The relation between the presence or absence of early immobilization and sensory restriction and current intellectual functioning, language comprehension, and emotional and behavioral patterns was investigated in 30 children with phenylketonuria. The 16 children who had experienced immobilization and sensory restriction, as defined, differed significantly from the other 14 children. In intelligence (WISC) and vocabulary (PPVT) tests, a 20-point difference in Intelligence Quotient was documented between the groups. The incidence of serious behavioral pathology was markedly increased in the children who were subjected to immobilization and sensory restriction during the first three years of life. SpeculationPractices of medical care commonly employed in pediatric hospitals can substantially affect the future intellectual capacity and behavioral pattern of children with phenylketonuria. Similarly, immobilization and sensory restriction might affect other young pediatric patients unfavorably in the presence of other deleterious influences during hospitalization. One out of four children in pediatric hospitals is subjected to a significant degree of sensory restriction and/or immobilization.

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“…autosomal, recessive disorder resulting from the inability of the liver to metabolize the essential amino acid, phenylalanine. Recent investigations have suggested that experiential factors (Friedman, Sibinga, Steisel, & Sinnamon, 1968;) may be implicated in the occurrence of reported behavioral abnormalities (Bjornson, 1964;Jervis, 1954;Koch, Fishier, Shild, & Ragsdale, 1964;Yaker & Goldberg, 1963). Characterization of the behavior of children with PKU by use of a structured interaction procedure indicated that children with this disorder were more variable in their interactional behavior than groups of normal children, retarded children with brain damage, and psychotic children.…”

mentioning

confidence: 99%

An objective approach to measurement of interpersonal behavior in phenylketonuria.

Cj¹,

Ms²,

Im³

et al. 1971

Journal of Consulting and Clinical Psychology

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Children with phenylketonuria (PKU), previously reported to show behavioral abnormalities and communicational deficits and to be extreme in activity patterns, were evaluated by a structured interaction procedure. PKU children, judged to be emotionally disturbed and inadequate in communication from independent impressions during psychological testing, performed more poorly than those PKU children judged nondisturbed and more adequate in communication under two of the three social stimulus interaction conditions. Children with PKU who manifested extreme patterns of activity were not significantly different in their interactional behavior from children with PKU who demonstrated normal activity levels. Differences in interaction behavior were obtained between children with PKU and normal control children similar on socioeconomic variables.

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Behavior in Phenylketonuria

Cited by 18 publications

References 28 publications

Interaction patterns in children with phenylketonuria.

Interaction patterns in children with phenylketonuria.

The Effect of Immobilization and Sensory Restriction on Children with Phenylketonuria

An objective approach to measurement of interpersonal behavior in phenylketonuria.

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