HOME > EDICIONES > Año 2005, Volumen 55 - Número 4
Trabajos de Investigación
Composición lipidica de la dieta de niños fenilquetonúricos diagnosticados precozmente
Verónica Cornejo E1,5; Miluska Concha C.2; Juan Francisco Cabello3,5; Erna Raimann B4,5.
(1) Nutricionista, Magíster en Nutrición Humana, Profesor Asistente
(2) Nutricionista, Especialista en Enfermedades Crónicas no transmisibles de origen nutricional.
(3) Médico cirujano, Neuropediatra.
(4) Médico cirujano, Pediatra, Profesor Asistente
(5) Laboratorio de Enfermedades Metabólicas, Instituto de Nutrición y Tecnología de los Alimentos (INTA), Universidad de Chile. Av. El Líbano 5524, MACUL, Santiago, Chile
Correspondencia: Prof. Verónica Cornejo
INTA, Universidad de Chile
Casilla 13811, SANTIAGO
CHILE
e-mail: vcornejo@inta.cl
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SUMMARY
Lipids composition diet in phenylketonuric children with early diagnosis
Phenylketonuria (PKU) is a genetic disorder caused by a partial or complete mutation of the enzyme phenylalanine hydroxylase (PHA), fact that produces high levels of phenylalanine in blood resulting in mental retardation if not diagnosed during the neonatal period. Treatment consists of a phenylalanine (Phe) restricted diet. Several studies have shown that due to restriction of animal protein, this diet is deficient in fatty acids such as alfalinolenic acid (ALA) and provides high levels of linoleic acid (LA). The objective of this study was to determine the lipid composition of the diet consumed by children with early-diagnosed PKU. Lipid composition of the Phenylalanine restricted diet consumed by 29 children with PKU and in follow-up at INTA, University of Chile, were analyzed. Children were paired by sex and age with a control group. A twenty-four hour dietary recall was performed for 3 consecutive days and total fatty acid intake, including saturated, monounsaturated, polyunsaturated, LA and ALA, were calculated. In the restricted diet of children with PKU, 31.8% of total calories are from fat, 13% of which are LA and 0.2% ALA, showing significant differences as compared to the control group. The ratio of saturated: monounsaturated: polyunsaturated fatty acids was 1:1.7:3.9 and the ratio of LA: ALA was ten-fold higher than the recommended ratio of 115:1. It is concluded that the Phenyalanine restricted diet of Chilean children with PKU is high in LA and low in ALA.
Key words: children, phenylketonuria, linoleic acid, alfalinolenic acid, lipid
Recibido: 14/04/2005
Aceptado: 01/12/2005
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PRIVACIDAD | ACCESIBILIDAD
ALAN-VE ISSN 0004-0622 - Depósito Legal: pp 199602DF83
Sociedad Latinoamericana de Nutrición
Producción editorial en Venezuela: Capítulo Venezolano - RIF: J-30843129-0
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