Factors affecting cognitive, motor, behavioral and executive functioning in children with phenylketonuria

Arnold, GL; Kramer, BM; Kirby, RS; Plumeau, PB; Blakely, Eileen; Cregan, LS Sanger; Pw, Davidson

doi:10.1080/08035259850158308

Cited by 41 publications

(41 citation statements)

References 19 publications

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“…Garrison and colleagues 25 employed the Dimensions of Temperament Scale 26 and reported that a child's activity, rhythmicity, and attention span were related to a subjective measure of the child's compliance with the diabetes regimen, while maternal factors predicted 90% of the variance in glycosylated hemoglobin. More recently Arnold et al 27 investigated the relationship of temperament and frontal lobe, motor, and cognitive function to phenylalanine levels in children with phenylketonuria. Using the Toddler Temperament Scale 28 and Behavioral Style Questionnaire, 22 they reported that difficult behavior is associated with higher current and average phenylalanine levels.…”

Section: E154mentioning

confidence: 99%

Temperament and Physical Performance in Children With Osteogenesis Imperfecta

et al. 2003

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ABSTRACT. Objective. Children with osteogenesis imperfecta (OI) must participate in therapy to achieve motor performance objectives. Their behavioral style may influence motor performance. For this reason, the temperament of children with types III or IV OI was assessed prospectively to 1) compare their temperament with that of nondisabled children, 2) investigate the relationship between temperament and gross motor performance, and 3) examine relationships among temperament, parental overprotection and coping, physical activity, muscle strength, and motor performance.Methods. Age-appropriate Carey Temperament Scales, Brief Assessment of Motor Function (BAMF), and the Vulnerable Child/Overprotecting Parents Scale were completed for 35 children 1 to 12 years old. Additional measures included the Childhood Health Assessment Questionnaire, Parent Daily Hassles Scale, manual muscle testing, Pediatric Activity Record, and a Summed Severity Score. Spearman correlations and multiple regression were used to identify and predict significant relationships.Results. Temperament of children with OI differed from age-based norms in only 1 domain: activity. Motor performance (BAMF) correlated significantly with 3 domains of temperament: persistence (r ‫؍‬ ؊.48), approach (r ‫؍‬ ؊.34), and activity (r ‫؍‬ .40). Activity was also related to the ratio of head circumference to body length (r ‫؍‬ ؊.45) and the number of fractures in the preceding year (r ‫؍‬ ؊.35). Parents' reports of their daily hassles significantly correlated with several domains of the child's temperament. No significant relationships were identified between parental overprotection and temperament or motor performance.Conclusions. The temperament of children with types III and IV OI does not differ from that of their nondisabled peers, with the exception of lower activity scores. Although it is considered a biological attribute, the expression of temperament, specifically activity, may be influenced by learned behaviors. Because gross motor performance is related to activity, persistence, and approach/avoidance, knowledge of an individual's temperament may enhance the child's ability to benefit from interventions to improve motor skill and activity levels. Pediatrics 2003;111:e153-e161. URL: http://www. pediatrics.org/cgi/content/full/111/2/e153; osteogenesis imperfecta, temperament, motor performance.

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Section: E154mentioning

confidence: 99%

Temperament and Physical Performance in Children With Osteogenesis Imperfecta

et al. 2003

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“…Reduced fine motor abilities and visuomotor integration have been regularly reported in early treated PKU patients (Arnold et al, 1998;Pietz et al, 1998;Seashore, Friedman, Novelly, & Bapat, 1985;Weglage, Pietsch, Fünders, Koch, & Ullrich, 1995). The fact that higher integrative functioning was usually required to perform these tasks is in agreement with the type of neuropsychological deficits most frequently found in treated PKU: executive function deficits, which have often been associated with concurrent or historical blood Phe levels (Diamond, 1994;Diamond, Prevor, Callender, & Druin, 1997;Ris, Williams, Hunt, Berry, & Leslie, 1994;Smith, Klim, Mallozzi, & Hanley, 1996;Weglage, Pietsch, Fünders, Koch, & Ull-rich, 1996;Welsh, Pennington, Ozonoff, Rouse, & McCabe, 1990).…”

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confidence: 99%

Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria.

et al. 2003

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This study examined motor control in 61 early and continuously treated patients with phenylketonuria (PKU) and 69 control participants, aged 7 to 14 years. The pursuit task demanded concurrent planning and execution of unpredictable movements, whereas the tracking task required a highly automated circular movement that could be planned in advance. PKU patients showed significantly poorer motor control in both tasks compared with control participants. Deficits were particularly observed for younger patients (age Ͻ 11 years). Differences between control participants and PKU patients were significantly greater in the pursuit task compared with the tracking task, indicating more serious deficits when a higher level of controlled processing is required. Correlations with historical phenylalanine levels indicated a later maturation of the level of control required by the pursuit task compared with the tracking task.

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“…Arnold y cols. [8] aplicaron las Escalas Motoras de Desarrollo de Peabody en un grupo de niños pequeños que iniciaban el tratamiento dietético en el curso de 3 semanas desde el nacimiento. Este grupo presentaba un cociente motor de 83, que es superior a 1 DE por debajo de la media normativa, lo que indica retraso motor.…”

Section: Función Motoraunclassified

“…Se cree que una peor actividad motora fina está relacionada con mayores niveles de PHE [8,19] . No en todos los estudios se comuni caron deficiencias motoras finas.…”

Section: Función Motoraunclassified

“…Entre estos procesos destacan los siguientes: (a) Inicio de la actividad; (b) control de impulsos y autorregulación; (c) memoria operativa; (d) flexibilidad mental y utilización de retroalimentación; (e) aptitud de planificación y organización [66] . Aunque se reportan corrientemente deficiencias en estos ámbitos en niños y adultos con FCU tratados precozmente [7,8,15,17,29,31,33,58,67] , existen algunos datos contradictorios [41,50,56,68] . Las diferencias en los resultados pueden atribuirse en parte, (a) a las diversas conceptualizaciones de la FE por parte de los investigadores; (b) al uso de diferentes herramientas para las evaluaciones de la FE; (c) a discrepancias en el muestreo [69] .…”

Section: Función Ejecutiva (Fe)unclassified

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Función neuropsicológica en la fenilcetonuria tratada precozmente: Una revisión

Crossley¹,

Anderson²

2010

Ann Nestlé [Esp]

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La fenilcetonuria (FCU) es un error metabólico congénito que implica una deficiencia de la enzima fenilalanina hidroxilasa. Este proceso da lugar a niveles elevados de fenilalanina y niveles bajos de tirosina. Si no se administra tratamiento aparecen graves secuelas neuropatológicas y neuroconductuales. La puesta en práctica de la detección sistemática de recién nacidos y el tratamiento dietético precoz ha reducido significativamente esta morbilidad. A pesar de una función intelectual general relativamente conservada, en los pacientes con FCU tratados precozmente se detectan todavía sutiles deficiencias cognitivas y conductuales. Este trabajo aporta una revisión de los datos del deterioro de la elaboración de información, la función ejecutiva, la memoria, el aprendizaje, el aprovechamiento escolar y la conducta. En este trabajo también revisamos las dos teorías principales propuestas hasta la fecha en relación con los mecanismos subyacentes de las deficiencias cognitivas y conductuales halladas en pacientes con FCU tratados, concretamente la disfunción prefrontal y las anomalías de la sustancia blanca. Aunque es necesario ampliar la investigación, la literatura disponible hasta la fecha da a entender que los pacientes afectados de FCU tratados precozmente presentan un riesgo de deficiencias neuroconductuales insidiosas en toda una gama de dominios neuropsicológicos. Por lo tanto, los pacientes con FCU tienen que ser monitorizados minuciosamente para detectar precozmente estos problemas y aplicar intervenciones apropiadas capaces de asegurar que estos pacientes alcancen su pleno potencial.

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Factors affecting cognitive, motor, behavioral and executive functioning in children with phenylketonuria

Cited by 41 publications

References 19 publications

Temperament and Physical Performance in Children With Osteogenesis Imperfecta

Temperament and Physical Performance in Children With Osteogenesis Imperfecta

Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria.

Función neuropsicológica en la fenilcetonuria tratada precozmente: Una revisión

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