Nutritional evaluation of children with phenylketonuria
Keywords:
Phenylketonuria, Artificial diets, Anthropometric measurementsAbstract
CONTEXT: 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. DESIGN: Cross-sectional study. SETTING: 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. PARTICIPANTS: 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. MAIN MEASUREMENTS: 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 ³7 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.
Downloads
References
Acosta PB, Wenz E, Williamson M. Methods of dietary inception in infants with PKU. J Am Diet Assoc 1978;72:164-9.
Clark BJ. After a positive Guthrie - what next? Dietary management for the child with phenylketonuria. Eur J Clin Nutr 1992;46(suppl 1):S33-9.
Whitehead RG. Nutritional requirements of healthy adolescents and their significance during the management of PKU. Eur J Pediatr 1987;146(suppl 1):A25-A31.
McBurnie MA, Kronmal RA, Schuett VE, Koch R, Azeng CG. Physical growth of children treated for phenylketonuria. Am Hum Bio1991;18:357-68.
Fisberg RM, Silva-Fernandes ME, Martins AM, Nóbrega F, Schmidt BJ. Evaluación antropométrica de nińos brasileńos fenilcetonúricos. Actualidad Nutricional Espańa 1996;22:43-5.
Dwyer JT. Dietary assessment. In: Shils ME, Olson JA, Shike M. Modern nutrition in health and disease. 8th ed. Philadelphia: Lea & Febiger; 1994:842-60.
Flores M. Niveles dieteticos de familias y nińos según estado sócio-economico en el área rural de Panamá. Arch Latinoam Nutr 1975;25:135-62.
Ançăo M, Cuppari L, Tudisco ES, Draibe SA, Sigulem D. Programa de apoio a nutriçăo [programa de computador]. Versăo 2.0. Săo Paulo: CIS-EPM; 1984.
Fundação Instituto Brasileiro de Geográfia e Estatística. Estudo Nacional de Despesa Familiar: tabelas de composição química dos alimentos. Rio de Janeiro: FIBGE; 1977.
Souci SW, Fachmann W, Kraut H. Food composition and nutrition tables 86/87. Stuttgart: Wissenschaftliche Verlagsgesellschaft GmbH;1986.
National Research Council. Recommended dietary allowances. 10th ed. Washington: National Academy Press; 1989.
Lohman TG, Roche AF, Martorell R. Anthropometric standardization reference manual. Illinois: Human Kinetics Publishers; 1988.
Dean AG, Dean JA, Burton AH, Dicker RC. Epi Info [programa de computador]. Versăo 5. Atlanta: Center for Disease Control; 1990.
National Center for Health Statistics. Growth curves for children, birth to 18 years. Washington, DC: US NCHS; 1977. (Department of Health, Education and Welfare Publication No. (PHS) 78 - 1650).
Siegel S. Estatística năo paramétrica. Rio de Janeiro: McGraw-Hill; 1975.
Acosta PB, Gropper SS, Clarke-Sheehan N, et al. Trace element status of PKU children ingesting an elemental diet. J Parenter Enteral Nutr 1987;11:287-92.
Gropper SS, Acosta PB, Clarke-Sheehan N, Wenz E, Cheng M, Koch R. Trace element status of children with PKU and normal children. J Am Diet Assoc 1988;88:459-65.
Scaglioni S, Zuccotti G, Vedovello M, et al. Study of serum ferritin in 58 children with classic phenylketonuria and persistent hyperphenylalaninemia. J Inher Metab Dis 1985;8:160.
Longhi R, Rottoli A, Vittolrelli A, et al. Trace elements nutrition in hyperphenylalaninemic patients. Eur J Pediatr 1987;146:32-7.
Torres MAA, Sato K, Sousa Queiroz S. Anemia em crianças menores de dois anos atendidas nas Unidades Básicas de Saúde no Estado de Săo Paulo. Rev Saúde Pública 1994;28:290-4.
Acosta PB, Fernhoff PM, Warshaw HS, Hambidge KM, Ernest A, McCabe ERB. Zinc and copper status of treated children with phenylketonuria. J Parenter Enteral Nutr 1981;5:406-9.
Sibinga MS, Friedman CJ, Steisel IM, Baker EC. The depressing effect of diet on physical growth in phenylketonuria. Dev Med Child Neurol 1971;13:63-70.
