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.

Fatty acid metabolism in phenylketonuria / Giovannini, M; Agostoni, C; Biasucci, G; Rottoli, A; Luotti, D; Trojan, S; Riva, E. - In: EUROPEAN JOURNAL OF PEDIATRICS. - ISSN 0340-6199. - 155:(1996), pp. 132-135.

Fatty acid metabolism in phenylketonuria

Biasucci G;
1996-01-01

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.
1996
Fatty acid metabolism in phenylketonuria / Giovannini, M; Agostoni, C; Biasucci, G; Rottoli, A; Luotti, D; Trojan, S; Riva, E. - In: EUROPEAN JOURNAL OF PEDIATRICS. - ISSN 0340-6199. - 155:(1996), pp. 132-135.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11381/2931005
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