Abstract
Children treated for phenylketonuria (PKU) have a low intake of whole animal foods. Consequently, the dietary intake of long-chain polyunsaturated fatty acids (PUFA) is just a few milligrams per day, mostly represented by arachidonic acid (AA). In a consecutive series of studies, we assessed in treated PKU children their long-chain PUFA status, the AA-related eicosanoid synthesis and the effects of specific PUFA supplementations. We found that the good compliance with the dietary regimen negatively influences the long-chain PUFA status and serum eicosanoid release from platelets. Supplementation with either marine or blackcurrant oils modifies the long-chain PUFA status of PKU children without approaching the fatty acid pattern of a healthy control population. Good-compliant PKU patients have diet-related, low levels of circulating long-chain PUFA, whose clinical and functional consequences deserve further investigation. The effects of dietary supplementations with long-chain PUFA of both the n-6 and n-3 series should be carefully evaluated.
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Abbreviations
- AA :
-
arachidonic acid (20:4 n-6)
- ALA :
-
alpha-linolenic acid (18:3 n-3)
- DHA :
-
docosahexaenoic acid (22:6 n-3)
- EPA :
-
eicosapentaenoic acid (20:5 n-3)
- LA :
-
linoleic acid (18:2 n-6)
- Phe :
-
phenylalanine
- PKU :
-
phenylketonuria
- PUFA :
-
polyunsaturated fatty acids
- Tx :
-
thromboxane
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Giovannini, M., Agostoni, C., Biasucci, G. et al. Fatty acid metabolism in phenylketonuria. Eur J Pediatr 155 (Suppl 1), S132–S135 (1996). https://doi.org/10.1007/PL00014230
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DOI: https://doi.org/10.1007/PL00014230