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      Composición Lipídica de la Dieta de Niños Fenilquetonúricos Diagnósticados Precozmente


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          La Fenilquetonuria (PKU) se produce por un defecto total o parcial de la enzima fenilalanina hidroxilasa (FAH) acumulándose fenilalanina (FA) en sangre, lo que ocasiona retardo mental si no es diagnosticada en el período de recién nacido. El tratamiento consiste en una dieta restringida en FA. Diversos estudios han señalado que debido a la restricción de proteínas animales, la dieta es deficiente en ácidos grasos: alfalinolénico (ALL) y elevada en ácidos linoléico (AL). El objetivo de este estudio fue determinar la ingesta de lípidos en niños PKU diagnosticados precozmente, estudiándose la composición química de lípidos de la dieta de 29 niños con PKU, en dieta restringida en FA y en seguimiento en el INTA, Universidad de Chile. Fueron pareados por sexo y edad con un grupo control. Se aplicó encuesta de recordatorio de 24 horas por tres días consecutivos, calculándose la ingesta de ácidos grasos totales, saturados, monoinsaturados, poliinsaturados, AL y ALL. Los resultados muestran que la dieta de los PKU aporta el 31.8% como calorías grasas, de los cuales el 13% es AL y un 0.2% ALL, existiendo diferencias significativas con respecto al grupo control. La relación obtenida entre ácidos grasos saturados-monoinsaturados-poliinsaturados fue de 1:1.7:3.9 y la razón entre AL y ALL fue diez veces más de lo recomendado (115:1). Concluyéndose que la dieta de los niños PKU chilenos contiene mayor cantidad de AL y menor de ALL.

          Translated abstract

          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.

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          Most cited references22

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          Essential fatty acids as determinants of lipid requirements in infants, children and adults.

          Essential fatty acids (EFA) are the indispensable component of the lipid supply beyond the provision of energy as a fuel for oxidation. They serve as dietary precursors for the formation of prostanoids and other eicosanoids thus are of great significance in health and modulation of disease conditions. Eicosanoids are powerful autocrine and paracrine regulators of cell and tissue functions: thrombocyte aggregation, inflammatory reactions and leukocyte functions, vasoconstriction and vasodilatation, blood pressure, bronchial constriction, and uterine contraction. Recent attention has focused on the effect of n-3 and n-6 long chain EFAs in normal fetal development. Results from human infant studies suggest that n-3 fatty acids are needed for optimal development of visual and brain function. Human milk is the best and only time proven source of fat and dietary essential fatty acids for infant feeding. International recommendations for n-3 and n-6 EFA dietary intake are reviewed and suggested intakes for long chain EFAs are provided.
            • Record: found
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            Effect of diet on plasma total antioxidant status in phenylketonuric patients.

            Phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, is treated with a low Phe lifelong diet, which is a vegetarian and contains many antioxidants.
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              Long-chain polyunsaturated fatty acids in plasma and erythrocyte membrane lipids of children with phenylketonuria after controlled linoleic acid intake.

              It has been reported that children with classical phenylketonuria (PKU) have reduced levels of arachidonic acid (AA, 20:4 n-6) and docosahexaenoic acid (DHA, 22:6 n-3) in plasma and membrane phospholipids compared to controls and may therefore require supplementation. However, it is not established that these changes are specific for PKU. They may as well be attributed to the specific composition of a largely vegetarian diet used for dietary PKU treatment. We therefore investigated the fatty acid composition of plasma phospholipids (PL), plasma cholesterol esters (CE), red blood cell phosphatidylcholine (PC), and red blood cell phosphatidylethanolamine (PE) in two groups of PKU patients including 8 children between 1 and 6 years (group A), 9 adolescents between 11 and 18 years (group B), and 20 age-matched healthy controls. Group A had good dietary control (median plasma phenylalanine 272 mumol/L during the last 6 months before phospholipid analysis) while median phenylalanine in group B was 714 mumol/L (p < 0.001). When compared to age-matched controls, group A showed significantly lower DHA levels in PE (4.21 vs 5.85 weight% (wt%), p < 0.01), in PC (1.02 vs 1.25 wt%, p < 0.05) and in CE (0.25 vs 0.54 wt%, p < 0.05). There was no significant difference of DHA between group B and controls. AA levels were similar in phospholipids of all groups. We conclude that reduced levels of long-chain polyunsaturated fatty acids in PKU patients occur only in those patients with strict dietary therapy with respect to n-3 fatty acids, most probably caused by reduced intake of n-3 fatty acids.

                Author and article information

                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Archivos Latinoamericanos de Nutrición
                Sociedad Latinoamericana de Nutrición (Caracas )
                December 2005
                : 55
                : 4
                : 332-335
                [1 ] Universidad de Chile
                [2 ] Neuropediatra
                [3 ] Pediatra
                [4 ] Universidad de Chile



                SciELO Venezuela

                Self URI (journal page): http://www.scielo.org.ve/scielo.php?script=sci_serial&pid=0004-0622&lng=en
                NUTRITION & DIETETICS

                Nutrition & Dietetics
                children,phenylketonuria,linoleic acid,alfalinolenic acid,lipid,niño,Fenilquetonuria,ácido linoléico,ácido alfalinolénico,lípidos


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