Summary
To determine whether then−6 andn−3 polyunsaturated fatty acid levels can be affected by the PKU diet, fatty acid composition was studied in PKU subjects and controls together with their lipid intake. The study population consisted of 40 PKU patients treated with a phenylalanine-restricted diet. Controls were 50 children on whom preoperative tests had been carried out for minor surgery. Methyl esters were obtained from plasma and red blood-cells and separated by gas chromatography. PKU patients showed lower docosahexaenoic acid levels (22:6n−3) (p<0.001) and highern−6 series levels in plasma (18:2n−6) (p<0.001) and in red blood-cells (20:4n−6) (p<0.001) than the control population. Since docosahexaenoic and arachidonic acids are fundamental structural components for normal brain development, it seems necessary to consider a dietary adjustment in these patients.
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References
Bickel H, Wachtel U (1987) Phenylketonuria in adolescence.Eur J Pediatr 146 (supplement 1): A1–A43.
Connor WE, Neuringer M, Lin DS (1990) Dietary effects on brain fatty acids composition: the reversibility ofn-3 acid deficiency and turnover of docosahexaenoic acid in the brain, erythrocytes and plasma of rhesus monkeys.J Lipid Res 31: 237–247.
Galli C, Agostoni C, Mosconi C, Riva E, Salari PC, Giovannini M (1991) Reduced plasma C-20 and C-22 polyunsaturated fatty acids in children with phenylketonuria during dietary intervention.J Pediatr 119: 562–567.
Koch R, Azen CG, Hurst N, Friedman EG, Fislher K (1987) The effects of diet discontinuation in children with phenylketonuria.Eur J Pediatr 146 (supplement 1): A12–A16.
Lepage G, Roy CC (1986) Direct transesterification of all classes of lipids in a one-step reaction.J Lipid Res 27: 114–120.
Olsen SF, Hansen HS, Sorensen TIAS et al (1986) Intake of marine fat, rich inn-3 polyunsaturated fatty acids, may increase birthweight by prolonging gestation.Lancet 2: 367–369.
Olson RE (1987) Influence of diet on fatty acid composition of red cell and neural membranes.Nutr Rev 45: 246–248.
Schulpis KH, Nounopoulos C, Scarpalezuo A, Bouloukos A, Missiou-Tsagarakis S (1990) Serum carnitine level in phenylketonuric children: dietary control in Greece.Acta Paediatr Scand 79: 930–934.
Villasana D, Butles IJ, Williams JC, Roongta SM (1989) Neurological deterioration in adult phenylketonuria.J Inher Metab Dis 12: 451–457.
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Sanjurjo, P., Perteagudo, L., Soriano, J.R. et al. Polyunsaturated fatty acid status in patients with phenylketonuria. J Inherit Metab Dis 17, 704–709 (1994). https://doi.org/10.1007/BF00712012
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DOI: https://doi.org/10.1007/BF00712012