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Órgano Oficial del Instituto Nacional de Pediatría
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2012, Number 6

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Acta Pediatr Mex 2012; 33 (6)

Neuropsychological aspects in patients with phenylketonuria (PKU)

Sánchez C, Rodríguez- Ríos S
Full text How to cite this article

Language: Spanish
References: 7
Page: 293-295
PDF size: 68.72 Kb.


Key words:

Phenylketonuria, PKU, neurodevelopment, motor skills.

ABSTRACT

The evaluation of neuropsychological development in phenylketonuria patients must be considered in a comprehensive follow-up. This assessment should include all aspects of healthy development, including motor skills. This article outlines the results of a study whose objective was to show the relationship between age at onset of dietary treatment and adherence to the treatment, and the motor behavior of hyperphenylalaninemic children under nine years.


REFERENCES

  1. Christ SE, Huijbregts S, de Sonneville L, White D. Executive function in early-treated phenylketonuria: Profile and underlying mechanisms. Mol Genet Metab 2010;99:S22–32.

  2. Diamond A, Baddeley A. Evidence of Importance of Dopamine for prefrontal cortex functions early in life. Phil Trans R Soc Lond B 1996;351:1483–94.

  3. Diamond A, Prevor M, Callender G, Druin DP. Prefrontal cortex cognitive deficits in children treated early and continuously for PKU. Monogr Soc Res Child Dev 1997;62:1–208

  4. Diamond Adele. Close Interrelation of Motor Development and Cognitive Development and of the Cerebellum and Prefrontal Cortex. Child Develop 2000;71(1):44-56.

  5. Embury J, Charron C, Martynyuk A, Liu B, Ali S, Rowland N, Laipis P. PKU is a reversible neurodegenerative process within the nigrostriatum that begins as early as 4 weeks of age in PAHenu2 mice. Brain Res 2007;1127(1):136–50.

  6. Gesell A, Amatruda C. Diagnóstico del desarrollo normal y anormal del niño. Distrito Federal: Editorial Paidos; 2006. p. 42-4.

  7. Leuzzi V, Pansini M, Sechi E, Chiarotti F, Carducci C, Levi G, Antonozzi I. Executive function impairment in early-treated PKU subjects with normal mental development. J Inherit Metab Dis 2004;27:115–25.




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Acta Pediatr Mex. 2012;33