Information processing in patients with early and continuously-treated phenylketonuria. 1995

B A Stemerdink, and J J van der Meere, and M W van der Molen, and A F Kalverboer, and M M Hendrikx, and J Huisman, and L W van der Schot, and F M Slijper, and F J van Spronsen, and P H Verkerk
Department of Developmental and Experimental Clinical Psychology, State University Groningen, The Netherlands.

A total of 33 patients with early and continuously-treated phenylketonuria (PKU) between 7 and 16 years of age and 33 matched controls participated in a study examining perceptual, central, and response-related mechanisms of information processing. The specific mechanisms studied were: perceptual filtering, memory search, response selection, response execution, and motor presetting. In addition, groups were compared on mean intelligence level and task oriented behaviour. The performance of the PKU patients practically matched that of the controls on all three tasks, suggesting that PKU patients who are continuously maintained on a well-controlled phenylalanine-restricted diet are not impaired in the elementary mechanisms of information processing. Furthermore, groups did not differ in mean IQ or task-oriented behaviour. CONCLUSIONS These results underline the importance of continued, well-controlled dietary treatment. Further studies are recommended to obtain a more complete evaluation of the potential of PKU patients under these stricter dietary treatment conditions.

UI MeSH Term Description Entries
D007361 Intelligence Tests Standardized tests that measure the present general ability or aptitude for intellectual performance. Mental Tests,Raven Test,Raven's Progressive Matrices,Intelligence Test,Mental Test,Raven Progressive Matrices,Ravens Progressive Matrices,Test, Intelligence,Test, Mental,Test, Raven
D008297 Male Males
D008606 Mental Processes Conceptual functions or thinking in all its forms. Information Processing, Human,Human Information Processing
D010661 Phenylketonurias A group of autosomal recessive disorders marked by a deficiency of the hepatic enzyme PHENYLALANINE HYDROXYLASE or less frequently by reduced activity of DIHYDROPTERIDINE REDUCTASE (i.e., atypical phenylketonuria). Classical phenylketonuria is caused by a severe deficiency of phenylalanine hydroxylase and presents in infancy with developmental delay; SEIZURES; skin HYPOPIGMENTATION; ECZEMA; and demyelination in the central nervous system. (From Adams et al., Principles of Neurology, 6th ed, p952). Biopterin Deficiency,Dihydropteridine Reductase Deficiency Disease,Hyperphenylalaninemia, Non-Phenylketonuric,Phenylalanine Hydroxylase Deficiency Disease,BH4 Deficiency,DHPR Deficiency,Deficiency Disease, Dihydropteridine Reductase,Deficiency Disease, Phenylalanine Hydroxylase,Deficiency Disease, Phenylalanine Hydroxylase, Severe,Dihydropteridine Reductase Deficiency,Folling Disease,Folling's Disease,HPABH4C,Hyperphenylalaninaemia,Hyperphenylalaninemia Caused by a Defect in Biopterin Metabolism,Hyperphenylalaninemia, BH4-Deficient, C,Hyperphenylalaninemia, Tetrahydrobiopterin-Deficient, Due To DHPR Deficiency,Non-Phenylketonuric Hyperphenylalaninemia,Oligophrenia Phenylpyruvica,PAH Deficiency,PKU, Atypical,Phenylalanine Hydroxylase Deficiency,Phenylalanine Hydroxylase Deficiency Disease, Severe,Phenylketonuria,Phenylketonuria I,Phenylketonuria II,Phenylketonuria Type 2,Phenylketonuria, Atypical,Phenylketonuria, Classical,QDPR Deficiency,Quinoid Dihydropteridine Reductase Deficiency,Tetrahydrobiopterin Deficiency,Atypical PKU,Atypical Phenylketonuria,Biopterin Deficiencies,Classical Phenylketonuria,Deficiency, BH4,Deficiency, Biopterin,Deficiency, DHPR,Deficiency, Dihydropteridine Reductase,Deficiency, PAH,Deficiency, Phenylalanine Hydroxylase,Deficiency, QDPR,Deficiency, Tetrahydrobiopterin,Disease, Folling,Disease, Folling's,Hyperphenylalaninemia, Non Phenylketonuric,Non Phenylketonuric Hyperphenylalaninemia,Non-Phenylketonuric Hyperphenylalaninemias
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D013647 Task Performance and Analysis The detailed examination of observable activity or behavior associated with the execution or completion of a required function or unit of work. Critical Incident Technique,Critical Incident Technic,Task Performance,Task Performance, Analysis,Critical Incident Technics,Critical Incident Techniques,Incident Technic, Critical,Incident Technics, Critical,Incident Technique, Critical,Incident Techniques, Critical,Performance, Analysis Task,Performance, Task,Performances, Analysis Task,Performances, Task,Task Performances,Task Performances, Analysis,Technic, Critical Incident,Technics, Critical Incident,Technique, Critical Incident,Techniques, Critical Incident
D016555 Matched-Pair Analysis A type of analysis in which subjects in a study group and a comparison group are made comparable with respect to extraneous factors by individually pairing study subjects with the comparison group subjects (e.g., age-matched controls). Analysis, Matched-Pair,Paired Comparisons,Analyses, Matched-Pair,Analysis, Matched Pair,Comparison, Paired,Comparisons, Paired,Matched Pair Analysis,Matched-Pair Analyses,Paired Comparison

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