Nutritional evaluation of children with phenylketonuria.
BACKGROUND
Dietary phenylalanine (PA) restriction is the most effective form for reducing its excess in the blood and is the only efficient method for treating phenylketonuria. The diet is complex and should be adapted to combine the patients' eating habits, growth and development. It depends basically on the use of industrialized products as substitutes free of PA for proteins that are not fully supplied.


OBJECTIVE
To evaluate the nutritional status of children with phenylketonuria (PKU) by anthropometric measurements and food intake.


METHODS
Cross-sectional study.


METHODS
Children with PKU attending the Association of Parents and Friends of Handicapped Children (Associação de Pais e Amigos dos Excepcionais - APAE) and normal children attending at municipal day care centers in São Paulo.


METHODS
42 children with PKU and 31 normal children aged 1 to 12 of both sexes were assessed in two groups, under and over 7 years of age.


METHODS
Weight and height measurements.


RESULTS
Children with PKU ingested calories, calcium, iron, zinc, and copper below the recommended values, whereas the protein intake was within the normal range. Food intake in the group of normal children was within normality rates. The height/weight Z-score means for children with PKU were 0.47 for those under 7 years and 1.86 for 7 year-olds and over; in normal children the means were 0.97 <7 years and 1.54 >/=7 years, with no statistically significant difference. The height/age Z-score means were significantly lower in the PKU children <7 years (-1.23) than in the normal controls (0.91).


CONCLUSIONS
The data presented demonstrate the importance of nutritional surveillance in patients with PKU so as to support adequacy of nutrient intake and to guarantee growth within the relevant standards.
Fisberg
R M
RM
Department of Nutrition, Public Health School, University of São Paulo, Brasil. rfisberg@usp.br
da Silva-Fernandes
M E
ME
Schmidt
B J
BJ
Fisberg
M
M
D002136:Calcium, Dietary / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004044:Dietary Proteins / Q000008:administration & dosage; D002149:Energy Intake; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009752:Nutritional Status; D010661:Phenylketonurias / Q000178:diet therapy*
D002136:Calcium, Dietary; D004044:Dietary Proteins