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Reduced plasma C-20 and C-22 polyunsaturated fatty acids in children with phenylketonuria during dietary intervention
C Galli1, C Agostoni, C Mosconi
1Fifth Department of Pediatrics, School of Medicine, University of Milan, Italy.
Insights
Children with phenylketonuria on a special diet showed altered plasma fatty acid profiles. Their levels of essential arachidonic (n-6) and n-3 fatty acids were lower compared to healthy controls.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Metabolic Disorders
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring strict dietary management.
- Dietary interventions for PKU aim to control plasma phenylalanine levels.
- Nutritional status, including fatty acid profiles, can be affected by PKU diets.
Purpose of the Study:
- To analyze and compare plasma and erythrocyte fatty acid composition in children with phenylketonuria (PKU) undergoing dietary treatment versus healthy controls.
- To investigate the impact of a low-protein, modified-fat PKU diet on essential fatty acid levels.
Main Methods:
- Analysis of plasma and erythrocyte lipid fatty acid composition.
- Comparison between 15 children with PKU (aged 3-12 years) on dietary treatment and 12 matched control subjects.
- Dietary assessment of protein, fat, and fatty acid intake.
Main Results:
- Children with PKU exhibited higher plasma oleic acid levels.
- Lower plasma levels of arachidonic acid (n-6) and n-3 fatty acids were observed in PKU patients.
- Linoleic acid and eicosatrienoic acid (n-9) levels were similar between groups.
Conclusions:
- The PKU diet, characterized by low animal protein and modified fat, alters plasma fatty acid profiles.
- Observed changes in PKU patients resemble those in vegetarian diets, potentially due to reduced intake of preformed arachidonic and long-chain n-3 fatty acids.
- Dietary modifications in PKU management necessitate attention to essential fatty acid balance.
Abstract:
The fatty acid composition of plasma and erythrocyte lipids was analyzed in 15 children with phenylketonuria (aged 3 to 12 years) during dietary treatment aimed to maintain plasma phenylalanine levels at less than 8 mg/dl (485 mumol/L), and compared with those of 12 matched control subjects. The diet of children with phenylketonuria provided less protein, with a very low proportion of animal proteins, less fat, but a higher proportion of linoleic acid as a percentage of calories, and a higher carbohydrate content versus that in the diet of control subjects. The children with phenylketonuria had higher plasma levels of oleic acid but lower levels of arachidonic (n-6) and n-3 fatty acids. Linoleic and eicosatrienoic (n-9) acid levels were the same in both groups. These changes in patients with phenylketonuria resemble those observed in vegetarians and may be due to the absence of preformed arachidonic acid and long-chain n-3 fatty acids in the phenylketonuric diet.