[Evaluation of the program for prevention and health promotion in phenylketonuria patients in Brazil].
OBJECTIVE
To evaluate the results of systematizing preventive and health promotion actions among phenylketonuria (PKU) patients.


METHODS
Results of phenylketonuria patients attended in the Prenatal PKU Screening Program in the State of Paraná, Brazil, from 1996 to 2001, were evaluated. Socioeconomic data were investigated and the gross motor function measure was applied to determine the motor score among 32 PKU infants with early diagnosis and treatment. Pearson's correlation coefficient was adopted to investigate the relationship between the target variable (motor score) and other quantitative variables (mean post-treatment serum phenylalanine level, parents' educational level, infant's age at the start of treatment, and family income).


RESULTS
Among all the children evaluated, 93.7% showed development within normal limits as reported in the literature. Treatment was initiated in the first month of life in 71.9% of the PKU cases. Socioeconomic data showed 39.5% of parents having completed the fourth grade of primary school or less. There was a significant correlation between infant's motor score and parents' educational level (N=32), as well as between motor score and early treatment (N=27).


CONCLUSIONS
The results highlighted the program's effectiveness. The correlation between parents' low educational level and lower motor score emphasizes the importance of support for parents in their use of diet therapy. The association between motor score and early initiation of treatment confirms the need for immediate admission into the program. The paucity of other evaluation studies in the literature makes generalization of the results difficult.
Brandalize
Salete do Rocio Cavassin
Sdo R
Pontifícia Universidade Católica do Paraná, Curitiba, PR, Brasil. salete.brandalize@bol.com.br
Czeresnia
Dina
D
D001938:Brazil; D002648:Child; D002657:Child Development / Q000502:physiology; D002675:Child, Preschool; D006293:Health Promotion; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009048:Motor Skills / Q000502:physiology; D015997:Neonatal Screening; D010651:Phenylalanine Hydroxylase / Q000097:blood; D010661:Phenylketonurias / Q000175:diagnosis / Q000517:prevention & control*; D012959:Socioeconomic Factors; D016896:Treatment Outcome
D010651:Phenylalanine Hydroxylase