title: [Lipids composition diet in phenylketonuric children with early diagnosis].
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.
authors.affiliation: Laboratorio de Enfermedades Metabólicas, Instituto de Nutrición y Tecnología de los Alimentos, Universidad de Chile, Santiago.
authors.forename: Verónica
authors.initials: V
authors.lastname: Cornejo
authors.forename: Miluska
authors.initials: M
authors.lastname: Concha
authors.forename: Juan Francisco
authors.initials: JF
authors.lastname: Cabello
authors.forename: Erna
authors.initials: E
authors.lastname: Raimann
mesh_terms: D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004041:Dietary Fats / Q000008:administration & dosage / Q000097:blood*; D005260:Female; D006801:Humans; D007223:Infant; D008041:Linoleic Acids / Q000008:administration & dosage / Q000097:blood; D008297:Male; D009752:Nutritional Status; D010651:Phenylalanine Hydroxylase / Q000097:blood; D010661:Phenylketonurias / Q000097:blood* / Q000178:diet therapy; D011795:Surveys and Questionnaires; D017962:alpha-Linolenic Acid / Q000008:administration & dosage / Q000097:blood
chemical_list: D004041:Dietary Fats; D008041:Linoleic Acids; D017962:alpha-Linolenic Acid; D010651:Phenylalanine Hydroxylase