Essential polyunsaturated fatty acids in plasma and erythrocytes of children with inborn errors of amino acid metabolism
Copyright policy )Essential polyunsaturated fatty acids in plasma and erythrocytes of children with inborn errors of amino acid metabolism
Essential fatty acids (EFAs), and their longer-chain more-unsaturated derivatives (LCPUFAs) in particular, are essential for normal growth and cognitive development during childhood. Children with inborn errors of amino acid metabolism represent a risk population for a reduced LCPUFA status because their diet is low in EFAs and LCPUFAs. We have investigated the EFA and LCPUFA status of children with various amino acid metabolism disorders (not PKU) under treatment. Fatty acid profiles of plasma and erythrocyte phospholipids of 33 patients (aged 0-18 years) and 38 matched controls were determined by gas-liquid chromatography. Food-frequency questionnaires were used to assess the mean fatty acid intake. The dietary intake of the EFAs linoleic acid (LA) and alpha-linolenic acid (ALA) was comparable in both groups, while the LCPUFA intake was much lower in patients. This was associated with lower relative concentrations (% of total fatty acids) of n-3 docosahexaenoic acid (DHA) in plasma and erythrocyte phospholipids. Concentrations of arachidonic acid (AA) did not differ. The same was observed for the two EFAs LA and ALA. Thus, as compared to healthy controls, children with amino acid metabolism disorders have a lower intake of LCPUFAs and have lower concentrations of DHA but not of AA in plasma and erythrocyte phospholipids. This suggests that endogenous AA synthesis might guarantee an adequate AA status. The lower DHA status, however, warrants further investigations regarding the impact of DHA supplementation on growth and development of these children.
- Erasmus University Rotterdam Netherlands
- Radboud University Nijmegen Netherlands
- Boston Children's Hospital United States
- Maastricht University Netherlands
- Wilhelmina Children's Hospital Netherlands
Male, Erythrocytes, Adolescent, Fatty Acids, Essential, Infant, EMC MM-01-54-01, Nutrition Surveys, Dietary Fats, UMCN 5.3: Cellular energy metabolism, Child, Preschool, Diet, Protein-Restricted, Fatty Acids, Unsaturated, NCMLS 4: Energy and redox metabolism, Humans, Female, IGMD 8: Mitochondrial medicine, Child, Amino Acid Metabolism, Inborn Errors, Phospholipids
Male, Erythrocytes, Adolescent, Fatty Acids, Essential, Infant, EMC MM-01-54-01, Nutrition Surveys, Dietary Fats, UMCN 5.3: Cellular energy metabolism, Child, Preschool, Diet, Protein-Restricted, Fatty Acids, Unsaturated, NCMLS 4: Energy and redox metabolism, Humans, Female, IGMD 8: Mitochondrial medicine, Child, Amino Acid Metabolism, Inborn Errors, Phospholipids
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