title: Fatty acid metabolism in phenylketonuria.
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.
authors.affiliation: Department of Paediatrics, San Paolo Biomedical Institute, Milan, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Giovannini
authors.forename: C
authors.initials: C
authors.lastname: Agostoni
authors.forename: G
authors.initials: G
authors.lastname: Biasucci
authors.forename: A
authors.initials: A
authors.lastname: Rottoli
authors.forename: D
authors.initials: D
authors.lastname: Luotti
authors.forename: S
authors.initials: S
authors.lastname: Trojan
authors.forename: E
authors.initials: E
authors.lastname: Riva
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015777:Eicosanoids / Q000096:biosynthesis; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010661:Phenylketonurias / Q000097:blood* / Q000178:diet therapy
chemical_list: D015777:Eicosanoids; D005231:Fatty Acids, Unsaturated