CEDIE   05498
CENTRO DE INVESTIGACIONES ENDOCRINOLOGICAS "DR. CESAR BERGADA"
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Cognitive assessment of early detected and treated PKU children
Autor/es:
VALLE MG; PARDO CAMPOS ML,; CHIESA A; ENACAN R,
Lugar:
CABA
Reunión:
Congreso; XI Congreso Latinoamericano de errores congénitos del metabolismo y pesquisa neonatal; 2019
Institución organizadora:
Sociedad latinoamericana de errores congenitos y pesquisa neonatal (SLEIMPN)
Resumen:
Introduction: Early detected and treated PKU might present neurocognitive disfunction possibly related to the underlying disease. Objectives: Characterize their cognitive profiles, assessing specific deficits and their relationship with variables of the disease and its treatment .Patients: 30 moderate to severe PKU children (9-10years), detected and adequately treated at median age of 18 days were selected and compared with a control group (CG) of 30 healthy children of the same age. All had absence of other concurrent diseases, parents with complete high school educational level and similar socioeconomic status. Methods WISC IV, Rey-Osterrieth Complex Figure Test, Continuous Performance Test (CPT II), ITPA (Illinois test of psycholinguistic abilities), Verbal Fluency Test, Trail Making Test, Faces Test were administered. Cognitive profile was related to age at start of treatment, initial phenylalanine (Phe) levels, Phe tolerance mg/day, first year median Phe levels (MPHEL),MPHEL 2-5years of age, MPHEL 6 years to evaluation, MPHEL year previous to evaluation, number of controls the year previous to evaluation. Student´s T test for independent samples was used for statistical analysis and Pearson´s for correlations. (significance:p